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DANDY-WALKER SYNDROME:
PRESENTATION OF CONGENITAL FORMATION IN AN OLDER PATIENT
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JOURNAL
“ OF THE LOUISIANA STATE MEDICAL SOCIETY
BOARD OF TRUSTEES
Chairman, CONWAY S. MAGEE, MD K. BARTON FARRIS, MD W. CHARLES MILLER, MD EMILE K. VENTRE JR, MD Ex officio, C. Clinton Lewis, MD
EDITORIAL BOARD A. JOANNE GATES, MD RODNEY C. JUNG, MD PATRICK W. PEAVY, MD TRENTON L. JAMES II, MD JACK P. STRONG, MD CLAY A. WAGGENSPACK JR, MD WINSTON H. WEESE, MD
LSMS BOARD OF GOVERNORS C. CLINTON LEWIS, MD DUDLEY M. STEWART, MD KEITH DESONIER, MD LEO L. LOWENTRITT JR, MD K. BARTON FARRIS, MD RUSSELL C. KLEIN, MD WALLACE H. DUNLAP, MD VINCENT A. CULOTTA JR, MD RICHARD J. PADDOCK, MD BARRY G. LANDRY, MD WILLIAM T. HALL, MD JOSEPH BUSBY JR, MD LYNN Z. TUCKER, MD R. MARK WILLIAMS, MD MARTIN B. TANNER, MD MARTIN J. DUCOTE JR, MD MARCUS L. PITTMAN III, MD CHARLES D. BELLEAU, MD JOSHUA LOWENTRITT, MD LAURA BRESNAHAN
ESTABLISHED 1844. Owned and edited by The Journal of the Louisiana State Medical Society, Inc., 6767 Perkins Road, Baton Rouge, LA 70808; phone: (225) 763-2310; fax (225) 763-2332.
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ARTICLES'
Theodore F. Thurmon, MD 21
Praveen Reddy, MD 31
Prasad S.S.V. Vannemreddy, MD Laurie Grier, MD Anil Nanda, MD
Kim Edward LeBlanc, MD, PhD 35
Glenn N. Jones, PhD
Timothy J. Dozier, MD 41
John Kalmar, MD
DEPARTMENTS
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2 |
INFORMATION FOR AUTHORS |
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C. Clinton Lewis, MD |
5 |
PRESIDENT'S MESSAGE Our Access to Better Care Plan |
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Mrs. Karen Depp |
7 |
LSMS ALLIANCE The Value of Membership |
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Jorse 1. Martinez-Lopez, MD |
8 |
ECG OF THE MONTH Readins T Leaves |
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L. Nicole Murray, MD Ronald G. Amedee, MD |
10 |
OTOLARYNGOLOGY/HEAD & NECK SURGERY REPORT Recurrent Aphthous Stomatitis |
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Maria Calimano, MD Robert Perret, MD Harold Neitzschman, MD |
16 |
RADIOLOGY CASE OF THE MONTH Right Upper Quadrant Pain and Palpable Mass |
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Gustavo A. Colon, MD |
18 |
THE JOURNAL 150 & 100 YEARS AGO January 1850 and 1900 |
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43 |
CALENDAR |
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45 |
CLASSIFIED ADVERTISING |
HOW CAN GENETICS HELP IN THE MANAGEMENT OF OBESITY
DANDY-WALKER SYNDROME:
PRESENTATION OF THE CONGENITAL FORMATION IN AN OLDER PATIENT
LAFAYETTE'S FAMILY PRACTICE RESIDENCY PROGRAM: PRACTICE PATTERNS OF GRADUATES
QUADRICEPS SPARING MYOPATHY
Eusene New New Orleans
J La State Med Soc VOL 1 52 January 2000
Information for Authors (expanded)
THE JOURNAL IS PUBLISHED FOR THE BENEFIT of the members of the Louisiana State Medical Society. Manuscripts should be of interest to a broad spectrum of physicians and designed to provide practical information on the current status, progress, and changes in the field of clinical medicine. The articles published are primarily original scientific studies but may in- clude societal, socioeconomic, or medicolegal topics.
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Smith1 Brown et al2 Several authors3-4'5 '9
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The following six examples illustrate the reference style adopted by The Journal for (1) a reference to an article in a journal, (2) a reference to a book or monograph, (3) a reference to a part of a larger work, (4) an orga- nization as an author, (5) a reference to a government publication, (6) a reference to a presentation at a societal meeting, (7) a reference to an article
2 J La State Med Soc VOL 1 52 January 2000
Information for Authors (expanded)
in a newspaper, and (8) a reference to a book which has been accepted for publication but has not yet been published.
1 . Brush JE Jr, Cannon RO III, Schenke WH, et al. Angina due to coronary microvascular disease in hypertensive patients without left ventricular hypertrophy. N Engl J Med 1988;319:1302-1307.
2. Hajdu SI. Pathology of Soft Tissue Tumors. Philadelphia, Pa: Lea & Febiger; 1979:60-83.
3. Robinson BH. Lactic acidemia. In: Scriver CR, Beaudet AL, Sly WS, et al (editors). The Metabolic Basis of Inherited Disease, 6th edition. New York: McGraw-Hill; 1989:869-888.
4. American College of Physicians. Comprehensive functional assesment of elderly patients. Ann Intern Med 1988;109:70-72.
5. Office of Smoking and Health. The Health Consequences of Involuntary Smoking: A Report of the Surgeon General, 1986. Rockville, Md: US Department of Health and Human Resources; 1987:97-106 [CDC publication 87-8398],
6. Schacter RK, Arluk J. Flexural microflora in patients with psoriasis. Presented at the Annual Meeting of the American Academy of Dermatology, New Orleans, La, December 4-6, 1982.
7. Altman LK. Experts change guides for using drugs for HIV. New York Times June 27, 1993:1,23.
8. Levine S, Walsh D, Amic B, et al (editors). Society and Health Foundations for a Nation. London: Oxford University Press [in press].
Type each reference entry as a separate hanging paragraph; number the en- tries consecutively in the order cited; do not list alphabetically; double-space reference entries; and punctuate as shown in the examples above.
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an explanation of any notations on the figure, the techniques used, and an acknowledgment of the source if the figure has been previously published. Type all legends on a separate sheet; use block paragraphs.
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Permissions
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If human research was involved, a report from the Institutional Review Board should be attached.
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Editorial Assistance
An expanded version of Information for Authors is published in the January and July issues of The Journal. For further help in preparing your manu- script or for questions about the editorial process, you may write the Editor or the Managing Editor at the address below. Or, if you perfer, contact either the Editor (Dr Magee) at (337) 439-8450, Fax (337) 439-7576; E-mail: conwaystonemagee@compuserve.com; or the Managing Editor at (225) 763- 8500, Fax (225) 763-2332, E-mail: publicaffairs@lsms.org.
Final Check
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Submission of the Manuscript
Submit the manuscript (in triplicate), the illustrations (two copies each), the required permissions, and a cover letter to:
Editor
Journal of the Louisiana State Medical Society 6767 Perkins Road Baton Rouge, LA 70808
J La State Med Soc VOL 1 52 January 2000 3
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PRESIDENT’S MESSAGE
Our Access to Better Care Plan
C. Clinton Lewis, MD
The LSMS proposes a voucher approach for the Medicaid Program. In 1995, the legisla- ture passed Act 1242 (R.S. 46:160 et seq), the Access to Better Care Medical Insurance Demonstra- tion Project , an LSMS introduction, which estab- lished as a pilot project a voucher system for Med- icaid recipients, incorporating high deductible catastrophic health insurance and medical savings accounts for the medically indigent. However, to date, the Department of Health and Hospitals has not been able to implement the voucher pilot project.
In the 1999 Regular Session, SCR 22 passed, which authorized DHH to implement a Medicaid Region III modified CommunityCARE program, a Medicaid voucher program, a Medicaid center of excellence program, and a Medicaid regional managed care pilot program. Implementation of these programs would require DHH to secure the necessary waivers from Health Care Financing Administration (HCFA). SB 221 also passed in the 1999 Regular Session and became Act 642. Act 642 requires that any modification to the Medical As- sistance Program approved by a HCFA waiver, which includes a managed care or voucher sys- tem, requires approval of the House and Senate Committees on Health and Welfare and the Joint Committee on the Budget. In order to facilitate
implementation of a managed care or voucher sys- tem, Act 642 repealed the requirement for approval by both houses of the legislature during a regular or extraordinary session.
A voucher system would increase the patient's freedom to choose his or her physician, decrease public outlays, reduce overall health care costs by eliminating cost-shifting, increase access to qual- ity care, and empower the needy to make decisions about their health care needs. This is not a new concept. Governments at all levels have been do- ing this for years, tapping into the efficiencies and expertise available in the private sector.
The Access to Better Care plan would accom- plish these results by providing a publicly financed voucher to eligible Medicaid recipients. The voucher, limited to the categorically needy and AFDC-eligible recipients, would be used to pur- chase a private health insurance plan. The array of health coverage options available to Medicaid ben- eficiaries could include traditional indemnity- based insurance, managed care plans such as HMOs or PPOs, or a benefit-payment schedule. Coupled with these plans would be a medical sav- ings account (MSA) designed to reward recipients for prudent use of the health delivery system.
It is widely agreed by those in the private and public sectors that to ultimately control costs and
J La State Med Soc VOL 1 52 January 2000 5
the abuses of health care there must be behavioral change. The most lasting and rapid changes in be- havior come about when there is self-motivation to do so. The medical savings account is a concept that introduces motivation or incentives into the process of wisely utilizing health care resources.
Historically, both employees and employers have saved money using these types of accounts. The Rand Corporation found that people spend 30% less with no adverse effects on their health when they are spending their own money.
It should be emphasized that Access to Better Care breaks with recent tradition in regard to our Medicaid population. In recent years, this popula- tion has had their health care managed, directed, and in many cases, provided by the state of Loui- siana. This third party control of the process has functionally removed these individuals from con- trolling their own "health-care destiny". They be- came less familiar with the overall system since they were effectively removed from the entire de- cision making process. Such an approach fosters increasing dependence of these individuals on the state and does little to increase feelings of self-reli- ance and dignity. This third party control over the provision of health care was then "justified" by what the LSMS feels is a potentially detrimental assumption: that Medicaid recipients are unable to make the decisions necessary to secure their own health care services.
Access to Better Care achieves several impor- tant social functions in addition to improving the quality and affordability of health care. This con- cept helps the Medicaid recipient begin the pro- cess of more clearly understanding health care and the advantages and disadvantages of different health care systems. Equally important, this con- cept reorients the role of government from a self- perpetuating, ever growing, complex cradle-to-the- grave provider of assistance to a government that helps its citizens to be more intelligent consumers with meaningful input into the programs of which they are participants.
Access to Better Care would create savings by the state from the purchasing of private insurance for less than Louisiana currently spends per Med- icaid recipient and by streamlining the Medicaid bureaucracy. The state of Louisiana currently en- gages in this practice to some extent when it pur-
chases Medicare coverage for Medicare-Medicaid dually-eligible beneficiaries. In addition, a fixed annual amount for Louisiana in the form of a voucher would make it easier for Louisiana to ac- curately budget for Medicaid expenditures, while the elimination of claims processing, with a one- time payment, would significantly reduce the size of the state's Medicaid bureaucracy. Privatizing Medicaid would strengthen the private health in- surance market and, thereby, provide additional tax revenues.
In addition, providers are already used to dealing with private insurance entities and are fa- miliar with their administrative requirements. There would then be a lesser need for a fiscal in- termediary to handle claims and payment, which would reduce office administrative costs of pro- viders and increase the attractiveness of the Med- icaid patient by reducing the hassle factor associ- ated with government programs.
Access to Better Care would empower the needy to make decisions and create a system based on choice. Access to Better Care would empower re- cipients to make important decisions regarding their own health care needs, moving them away from dependency and closer to self-sufficiency. The voucher system would also encourage recipi- ents to make informed decisions regarding costs. The plan could tie these decisions to incentives, such as usable credits for unused benefits, which lead recipients to make cost-effective choices. Fi- nally, Access to Better Care would allow individu- als to choose the kind of health care coverage they desire and to choose their own physician.
The LSMS hopes that DHH will seek a HCFA waiver to implement the Access to Better Care plan, which would provide an innovative solution to controlling costs while improving the quality of care.
6 J La State Med Soc VOL 1 52 January 2000
LSMS ALLIANCE
THE VALUE OF MEMBERSHIP
MRS. KAREN DEPP
When we speak of membership, we often ask "what's in it for me?" to determine the value we receive. What we most need to recognize is that the value of membership in any association or group is determined by the individual members. The value may well be dif- ferent for each member, and it might not be what the association perceives as the value it delivers.
The Louisiana State Medical Society Alliance is this year asking the questions that will help us to determine, at the state level as well as the par- ish level, what our value might be. In the state al- liance the value should be different than what it is in the local alliances and auxiliaries. The purpose of the state organization is not what the mission or purpose of each individual component is. Try- ing to be everything for everyone often results in being nothing for some and too much for others.
Meeting and working with the members of the parish alliances has given the Board of LSMSA a better idea of what the value of LSMSA member- ship is for individuals as well as their component alliances. The same holds true for membership in American Medical Association Alliance. While we
might not perceive the immediate benefit of per- sonal membership in either the LSMSA or the AMAA, it becomes apparent through the leader- ship of the parishes that value is being delivered.
While we attempt to rebuild our alliance for the future, we are recognizing that strong compo- nent medical societies and a strong LSMS are criti- cal for our success. In our efforts to strengthen our membership we are including efforts to help build membership in our societies. The one recurring message that we are receiving is that our alliances see their role as supporting our medical societies as well as providing programs and services to our communities that improve the health and well- being of our citizens. There are many organizations to which we might belong, but none have the one thing that our alliances and societies do — and that is the practice of medicine and the health of our communities is what brings us to membership. To- gether the LSMS and the LSMSA can move into the future with a strong commitment to our pro- fession and our members. That is both perceived and delivered value!
Mrs Depp is President of the Louisiana State Medical Society Alliance.
J La State Med Soc VOL 152 January 2000 7
ECG OF THE MONTH
Reading T Leaves
Jorse I. Martinez-Lopez, MD
A 64-year-old man was admitted to the CCU complaining of sudden onset of chest tightness and shortness of breath 5 hours earlier. The 12-lead ECG shown below was recorded on his third hospital day.
What is your diagnosis? Elucidation is on page 9.
8 J La State Med Soc VOL 1 52 January 2000
ECG of the Month Presentation is on page 8.
DIAGNOSIS — Acute ischemic cardiac syndrome
The tracing shows sinus bradycardia, at 56 times a minute. The PR interval is normal and the QRS complexes narrow, findings which indicate normal AV and intraventricular conduction.
Major abnormalities in the tracing involve the T waves and the QT interval. T waves are sharply inverted in all leads, except in leads AVR and AVL, are moderately deep — especially from precordial leads V2 through V6, and reach a maximal depth of 20 mm from the baseline in lead V3 — and are wider than normal. T waves in lead AVR, which normally are inverted, are distinctly upright, peaked, and wide. In AVL, T waves are also up- right, even though they are inverted in lead I. Last, there is minimal downsloping depression of the ST segments in association with the inverted T waves.
Second, the QT interval is prolonged, with a QT dispersion that ranges from 0.52 sec to 0.64 sec. The lengthened repolarization time is caused solely by prolongation of the duration of the T waves.
In the distant past, inverted T waves with the characteristic morphology, amplitude, and width found in this tracing were referred to as "Pardee- type" of T waves, and their presence as "Pardee sign". In recent years, this eponymic designation, which honored Dr Pardee, has been replaced by descriptive terms: giant T-wave inversion, global T-wave inversion, and canyon T waves.
At present, the leading causes of giant T-wave inversions (ie, > 5 mm) are myocardial ischemia and myocardial infarction. Nevertheless, attempt- ing to arrive at a specific clinical diagnosis on the basis of the ECG abnormalities alone is akin to "reading T leaves". These abnormalities are not pathognomonic for any single clinical disorder. Similar T-wave changes, with or without associ- ated lengthening of the QT interval, can be found in a wide variety of cardiac and non-cardiac dis- orders.
In view of the presenting symptoms reported by the patient (chest tightness and shortness of breath) and the abnormal ECG changes, primary consideration in the differential diagnosis should be given to ischemic heart disease, in one of its
acute forms: unstable angina pectoris or myocar- dial infarction, with or without ST-segment eleva- tion.
Chest discomfort and giant T-wave inversions may also occur in non-coronary cardiac disorders and may, in this context, mimic ischemic heart dis- ease. Among these are the following conditions: the Japanese form of apical hypertrophy and some other types of hypertrophic cardiomyopathy; stage 3 pericarditis; myocarditis; mitral valve prolapse; and cardiac metastases. Less often, this abnormal T-wave pattern is recorded in some patients with complete AV heart block and Stokes-Adams syn- cope, and in long-term ventricular pacing.
Whether giant T-wave inversions are associ- ated with or without prolongation of the QT inter- val, the pattern itself is not restricted to cardiac pathology. For this reason, the differential diag- nosis should include non-cardiac causes of giant T-wave inversions. For example, the combination of giant T-wave inversions and prolonged QT in- tervals is a relatively common finding in some patients with severe brain damage due to intra- cerebral hemorrhage, subarachnoid hemorrhage with intracerebral extension, and traumatic brain injury. In this setting, however, T waves are not as sharply inverted and peaked as in acute ischemic heart disease; instead, T waves are broad and in- verted and have a rounded nadir. Other non-car- diac conditions in which giant T-wave inversions may be found include the following: pheochro- mocytoma; bilateral carotid endarterectomy; after vagotomy; cocaine abuse; flecainide intoxication; and acute gastrointestinal disorders, such as acute gall bladder disease, acute pancreatic disease, and perforated duodenal ulcer.
A thorough work-up of the patient whose trac- ing is discussed here confirmed a non-Q-wave myocardial infarction and combined aortic valve stenosis and regurgitation. He underwent aortocoronary bypass to the left anterior descend- ing coronary artery and aortic valve replacement.
Dr. Martinez-Lopez is a specialist in cardiovascular diseases affiliated with the Cardiology Service, Department of Medicine, Texas Tech University Health Sciences Center arid Thomason General Hospital
in El Paso, Texas.
J La State Med Soc VOL 1 52 January 2000 9
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RECURRENT APHTHOUS STOMATITIS
L. Nicole Murray, MD; Ronald G. Amedee, MD
Recurrent aphthous stomatitis is the most common oral mucosal disease in North America but it is com- monly misdiagnosed and poorly understood. Pediatricians, internists, otolaryngologists, oral surgeons, and dentists may all be expected to treat this illness but little formal training in oral medicine may be offered to many of these health care professionals. This article reviews current evidence regarding etiol- ogy, pathogenesis, natural history, and treatment of this disorder.
The word aphtha has been translated as "to inflame", "thrush", or simply "ulcer". None of these translations helps to define the clini- cal entity that has come to be known as recurrent aphthous stomatitis (RAS). These lesions are com- monly called mouth ulcers or canker sores, and they have been reported to affect anywhere from 5% to 66% of the North American population.1 In- terestingly, 60% of those affected are members of the professional class.2 It is important for physi- cians to be able to recognize RAS and to distin- guish it from other ulcerative lesions of the oral cavity, as prognosis and treatment of these diseases may vary greatly.
CLINICAL FEATURES
Patients with recurrent aphthous stomatitis will complain of recurrence of one or more pain-
ful oral ulcers at intervals ranging from days to months. The disease usually begins in childhood or adolescence and may diminish in frequency and severity with age. Ulcers due to RAS are confined to the "soft mucosa" of the mouth, or areas of nonkeratinized mucosa that are not immediately adherent to bone.3 These areas include the buccal and labial mucosa, lateral and ventral tongue, floor of mouth, soft palate, and oropharyngeal mucosa. The only areas in the mouth that are not affected by RAS ulcers are the hard palate and the attached gingiva.
RAS is subdivided into three categories based on the size of the ulcers and on disease severity. Most patients have "minor aphthae", which are less than 1 cm in diameter and heal completely in 7-10 days. These usually involve a prodromal stage of prickling and burning for 1-2 days and may oc-
1 0 J La State Med Soc VOL 1 52 January 2000
cur in crops of 1-5 ulcers. Ulcers are shallow and round to oval in shape with a gray- to yellow-col- ored membrane. These ulcers are very painful for about 4 days, then, after several more days, are healed completely without scarring. "Major aph- thae" are uncommon and involve irregular deep ulcers of 1-3 cm in size. They may have a raised border and will require up to 4 weeks to heal. These ulcers leave extensive scarring and distortion upon healing, and these patients are rarely lesion free. This disorder is also known as Sutton's disease or periadenitis mucosa necrotica recurrens. "Herpe- tiform aphthae" are also uncommon and consist of crops of up to 150 very small (1-3 mm) ulcers that heal completely in 7-10 days. This category of RAS is unfortunately named because these ulcers, like all RAS ulcers, are completely unrelated to the herpes virus.
ETIOLOGY
Although many theories for the etiology of RAS have been proposed and investigated, none has been proven. A viral association has been sug- gested, but this is not supported by the majority of the literature. Occasionally, viruses such as her- pes, varicella-zoster, or adenovirus have been cul- tured in patients with RAS. These viruses are ubiq- uitous, however, and there are no reports of suc- cessful treatment of RAS with antiviral therapy.4 A bacterial association has also been proposed. An L-form streptococcus (probably S sanguinous or S mitis) has been isolated from RAS patients, and RAS outbreaks have been associated with in- creased antibody titres, but this has not been well corroborated, and it is clear that antibacterial drugs do not cure RAS.4
A great deal of literature has focused on a possible association with estrogen and progester- one levels in women. A recent metanalysis con- cluded, however, that no associations have been clearly established between RAS and the premen- strual period, pregnancy, or menopause.5 Atten- tion has also been focused on anxiety, stress, and the "type A" personality. It is clear that there is a higher incidence of RAS among college, medical, and dental students, and there is also a higher in- cidence among elementary students of higher so- cioeconomic status.2 However, studies that have attempted to link periods of life stress to the onset of RAS outbreaks have failed.6 There may be a pre-
dilection towards RAS among certain HLA types, and a positive family history may increase one's risk for developing RAS by 20%. These data are confounded, however, by the role that environ- ment and psychologic stress may play.
The role of nutrition is somewhat controver- sial. Deficiencies of B vitamins, iron, and zinc have all been implicated in small studies.7 In patients with documented vitamin deficiencies, replace- ment therapy may be of benefit. A small subset of patients with gluten-sensitive enteropathies may experience outbreaks of RAS that resolve with a gluten-free diet, but ulcers in the majority of these patients will not respond to dietary measures.8 Sensitivities to foods such as nuts, chocolate, cere- als, tomatoes, dairy products, and citrus fruits have also been implicated in the etiology of RAS. Avoid- ance in these patients may decrease the frequency of outbreaks.8
The role of noxious stimuli has also been in- vestigated. Minor trauma, such as lip biting, oral burns, or dental procedures, may precipitate an outbreak in susceptible persons.6 Nicotine, inter- estingly, seems to have a protective effect. Older studies have shown that resumption of smoking after cessation caused preexisting ulcers to heal within a few days.9 One recent small study showed that nicotine gum caused ulcer healing and pre- vention when taken for 1 month, and patients re- lapsed upon discontinuation of the gum.10 It has been postulated that this protective effect is due to the keratinizing action of nicotine on the oral mucosa.
One of the most interesting areas of recent study involves the investigation of a possible im- mune mechanism. Immunopathologic studies have shown abnormal expression of major histo- compatibility complex antigens on epithelial cells and nonspecific deposition of immune complexes in patients with RAS. Also, the presence of abnor- mal lymphocyte subpopulations and increased activity of antibody-dependent cytotoxic cells have been documented in patients during remission and activation of disease. In genetically predisposed people, unidentified antigens may trigger changes in local lymphocyte subpopulations. This, in turn, may result in an autoimmune reaction against tar- geted epithelial cells. The exact antigen triggers are not yet clear. There are those who find fault with this theory, however, given that the disease is in-
J La State Med Soc VOL 1 52 January 2000 1 1
termittent, is generally mild and self limiting, and does not reliably respond to immunomodulating drugs.11
DIAGNOSIS
History taking and physical examination are usually all that is required to make the diagnosis. The typical presentation and appearance are as discussed above. Key points that will help to elimi- nate other disorders are that the lesions will never have gone through a vesicular stage and will never have any crusting. Patients may report triggering factors, such as stress, trauma, or certain foods. The examination will show typical appearing shallow ulcers anywhere in the mouth except for the hard mucosa (ie, hard palate and attached gingiva). With these findings, it is often appropriate to ini- tiate treatment without any further workup.
Diseases that can be easily confused with RAS include herpetic gingivostomatitis and herpangina. Herpetic gingivostomatitis may occur primarily or secondarily. Primary infection usually occurs in young patients and is associated with systemic symptoms. Vesicles will appear anywhere in the mouth, including the hard mucosa, and will progress to ulcers that crust. Secondary herpetic infection is characterized by lesions that occur only on the hard mucosa and tend to recur in the same spot. In most patients these are benign and self- limiting, but antiviral drugs may shorten the course. Herpangina is caused by the Coxsackie A virus and generally occurs in children less than 10 years old. Affected children develop systemic symptoms 48 hours prior to developing papulove- sicular lesions of the tonsils and uvula. Generally, supportive therapy is all that is required for her- pangina.
Patients with oral lesions whose appearance is not consistent with RAS or with lesions that ap- pear to be RAS major may require further investi- gation for diagnosis. Often a biopsy is helpful. Dis- eases such as pemphigus vulgaris, benign mucosal pemphigoid, lichen planus, and of course squa- mous cell carcinoma can all present with oral le- sions but these lesions will not have the typical small, round, shallow appearance of ulcers due to RAS.
There are several systemic diseases that may involve oral ulcers that are clinically similar to or identical to those due to RAS. When evaluating
RAS patients, these should be kept in mind. Bechet's disease is a multisystemic disorder that tends to affect males of Mediteranean, Middle East- ern, or Japanese descent. These patients present with the classic triad of aphthous ulcers, genital ulcers, and uveitis or conjunctivitis. The oral ul- cers may be treated in the same manner as those not associated for Bechet's disease, but these pa- tients need referral for systemic treatment as well.4 Patients with HIV or AIDS may present with a myriad of oral lesions. Aphthous ulcers have been reported with increased frequency in patients with CD4+ counts below 100.
RAS may require a biopsy in these patients and treatment may be less successful. Several stud- ies have shown an association between RAS and gastrointestinal diseases.4 A subset of patients with celiac sprue will have RAS and their disease will get better with treatment for their sprue. Likewise, a subset of patients with Crohn's disease will have RAS that responds to treatment of the bowel dis- ease. Some patients with vitamin deficiencies such as iron and B vitamins will have RAS that responds to vitamin replacement therapy.7 Some children will get aphthous ulcers in conjunction with the PFAPA syndrome (Periodic Fever, Aphthous ul- cer, Pharyngitis, and cervical Adenitis).12 Most RAS patients, however, will be healthy with no sign of associated disease.
MANAGEMENT
Goals in management of this disease reflect that the disease is generally mild and self-limit- ing, and that, currently, there is no treatment that is widely believed to be curative. Therefore, treat- ments that reduce pain and maintain function dur- ing attacks, or that reduce the severity and fre- quency of recurrent attacks, are considered suc- cessful. Identification and avoidance of precipitat- ing factors may be more helpful for some patients than others, and to this end an "ulcer diary" may be helpful. Medical treatments used for this gen- erally benign disease should, of course, not be as- sociated with more morbidity than the disease it- self.
Treatment options can generally be broken down into palliative treatments and those that may truly alter the course of the disease. Palliative medi- cations are generally applied topically. Topical medicines that relieve pain temporarily will allow
1 2 J La State Med Soc VOL 1 52 January 2000
patients to comfortably eat, and many of these are available over the counter. Preparations which contain benzocaine, diclonine HC1, or benzydamine HC1 are very effective. Patients with multiple lesions, or lesions that are not easily within reach, may be treated with a solution con- taining 2% viscous lidocaine, Kaopectate, and benadryl. This solution can be mixed by pharma- cists and combines an anesthetic with a protective coating agent. Patients should be advised that swallowing this solution is usually unnecessary and will likely cause drowsiness.13
A large number of therapeutic modalities have been described for this disorder. Topical caustic agents, such as hydrogen peroxide, phenol, and silver nitrate, have been used for some time but data regarding success using these agents is lim- ited to anecdotal reports. Topical antimicrobials have been used for some time. Antiseptic mouth- washes, such as chlorhexidine gluconate and Listerine, do seem to have a beneficial effect on both the duration and the frequency of outbreaks if used regularly in susceptible patients.14 Topical application of tetracycline 250 mg/ 30 cc 4-6 times daily for 4-5 days also has been shown to possibly reduce the duration and severity of an outbreak.15 This tetracycline elixir is no longer available from pharmacies, however, and would have to be mixed up by patients using capsules and water.
Many practitioners currently feel that the mainstay of treatment of this disorder is topical steroid application. Several different formulations are available. Triamcinolone 0.1% in dental paste can be applied directly to lesions 4 times daily, with the last application at bedtime.16 Patients must be instructed not to eat or drink for 1 hour after each application and the medication should be used for 4-5 days or until the lesion begins to heal. This medication can be applied at the prodromal stage and may prevent or abort an outbreak. If patients have multiple lesions or lesions that cannot be reached, triamcinolone 0.1% in an aqueous base can be swished around the mouth 4 times daily.17
Beclomethasone spray has also been shown to be successful in treating multiple ulcers or those that are hard to reach.18 As oral candidiasis has been reported in patients using sprays and solu- tions, prophylaxis with antifungal agents should be considered in these patients. If patients have an especially large number of lesions or long dura-
tion of outbreaks, a "burst regimen" of systemic steroid treatment may be prescribed in addition to topical treatment.1 Patients may be given pred- nisone 40 mg qd x 5 days and then 20 mg qod x 5 days. The success of steroid therapy lends support to the theory that the disorder has an autoimmune component.
Other immunotherapeutic regimens are being investigated but are not widely accepted yet. Levamisole is an immunomodulator which seems to reduce healing time and reduce the number of ulcers.19 It does take several months of treatment to achieve this effect however, and side effects may include nausea, hyperosmia, dysgeusia, and agranulocytosis.8
Azathioprine and colchicine have shown vari- able success in recalcitrant cases, but the lesions usually recur upon discontinuation of the drug.4 Thalidomide has been shown to produce healing of major aphthae and to improve eating ability in affected HIV patients. Due to side effects such as peripheral neuropathy, the use of this drug is gen- erally limited to the HIV population whose lesions produce severe pain, impair oral intake, and are refractory to other treatments.20
CONCLUSIONS
Although most patients who present with a complaint of recurrent mouth ulcerations will have RAS minor, it is important to rule out other disor- ders such as Bechet's disease, herpes simplex, li- chen planus, etc. This is generally accomplished with an adequate history and physical. Patients with lesions not typical of RAS may require a bi- opsy for diagnosis. Patients with systemic symp- toms may need further laboratory work-up or re- ferral to the appropriate specialist. Although RAS is generally mild and self-limiting, it can be highly uncomfortable and frustrating for affected patients.
Many patients may have been told that there are no effective treatments for this disorder, but this is a misconception. Although there is no cure, there are a number of regimens that may signifi- cantly improve the patient's symptoms during an outbreak. Patients may often even be taught to treat themselves at the prodromal stage and therefore abort an outbreak. The etiology of this disorder remains elusive but hopefully will become clear with continued research into immunopathologic mechanisms.
J La State Med Soc VOL 1 52 January 2000 1 3
REFERENCES
1. Embil JA, Stephens RG, Manuel RK. Prevalence of recurrent herpes labialis and aphthous ulcers among young adults on six continents. Can Med Assoc J 1975; 113:627-630.
2. Ship II, Morris AL, Durocher RT, et al. Recurrent aphthous ulcerations and recurrent herpes labialis in a professional school student population. Oral Surg Oral Med Oral Pathol 1960;13:1191-1202.
3. Burns RA, Davis WJ. Recurrent aphthous stomatitis. AFP 1985;32:99-104.
4. Woo SB, Sonis ST. Recurrent aphthous ulcers: a review of diagnosis and treatment. JADA 1996;127:1202-1213.
5. McCartan BE, Sullivan A. The association of menstrual cycle, pregnancy, and menopause with recurrent oral aphthous stomatitis: a review and critique. Obstet Gynecol 1992;80:455-458.
6. Rees TD, Binnie WH. Recurrent aphthous stomatitis. Derm Clinics 1996;14:243-256.
7. Wray D, Ferguson MM, Hutcheon AW. Nutritional deficiencies in recurrent aphthae. J Oral Pathol 1978;7:418- 423.
8. Porter SR, Scully C, Pedersen A. Recurrent aphthous stomatitis. Grit Rev Oral Biol Med 1998;9:306-321.
9. Bookman R. Relief of canker sores on resumption of cigarette smoking. Calif Med 1960;93:235-236.
10. Bittoun R. Recurrent aphthous ulcers and nicotine. Med J Austr 1991;154:471-472.
11. Porter SC, Porter SR. Recurrent aphthous stomatitis: current concepts of etiology, pathogenesis and management. J Oral Pathol Med 1989;18:21-27.
12. Thomas KT, Feder HM, Lawton AR, et al. Periodic fever syndrome in children. } Pediatr 1999;135:15-21.
13. Carpenter WM, Silverman S Jr. Over-the-counter products for oral ulcerations. J Calif Dent Assoc 1998;26:199-201
14. Meiller TF, Kutcher MJ, Overholser CD, et al. Effect of
an antimicrobial mouth rinse on recurrent aphthous ulcerations. Oral Surg Oral Med Oral Pathol 1991;72:425- 429.
15. Graykowski EA, Kingman AK. Double-blind trial of tetracycline in recurrent aphthous ulceration. J Oral Pathol 1978;7:376-382.
16. Graykowski EA, Hooks JJ. Treatment of recurrent aphthous ulcerations. J Oral Pathol 1978;7:439-440.
17. Vincent SD, Lilly GE. Clinical, historic, and therapeutic features of aphthous stomatitis: literature review and open clinical trial employing steroids. Oral Surg Oral Med Oral Pathol 1992;74:79-86.
18. Thomas AC, Nolan A, Lamey P-J. Aphthous oral ulceration: a double blind crossover study of beclomethasone diproprionate aerosol spray. Scott Med J 1990;34:531-532.
19. Sun A, Chiang CP, Chiou PS, et al. Immunomodulation by levamisole in patients with recurrent aphthous ulcers or oral lichen planus. J Oral Pathol Med 1994;23:172-177.
20. Jacobson JM, Spritzler J, Fox L, et al and the National Institute of Allergy and Infectious Diseases AIDS Clinical Trials Group. Thalidomide for the treatment of esophageal aphthous ulcers in patients with human immunodeficiency virus infection. J InfDis 1999;180:61- 67.
Dr Murray is a resident physician in the Department of Otolaryngology at Tulane University Medical Center in New Orleans, Louisiana.
Dr Amedee is a Professor and Chair of the Department of Otolaryngology at Tulane University Medical Center in New Orleans, Louisiana.
Can You Help?
The Louisiana State Medical Society has discovered that bound copies of The Journal of the LSMS for the years 1988-1991 are missing,
and we need your help.
We would like to maintain our library archives by rebinding issues for the missing
years.
If you have copies of any issue of
The Journal published during 1988-1991 and would like to contribute to this effort, please
send them to the Managing Editor, LSMS,
6767 Perkins Road, Baton Rouge, LA 70808 or call (225) 763-8500.
1 4 J La State Med Soc VOL 1 52 January 2000
Save the date!
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Plenary sessions will feature nationally acclaimed speakers and panel participants.
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Interactive break-out and optional sessions will include opportunities such as the AMA/ Intel Internet Health Road Show and:
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RADIOLOGY CASE OF THE MONTH
RIGHT UPPER QUADRANT PAIN AND PALPABLE MASS
Maria Calimano, MD; Robert Perret, MD; Harold Neitzschman, MD
This is a 52-year-old woman who presented to the E.R. with right upper quadrant pain and a palpable mass on physical examination.
Figure 1. Sagittal ultrasound the right lobe of the liver.
image
through
Figure 2. Contrast enhanced, early phase CT scan image through the upper abdomen.
What is your diagnosis? Elucidation is on page 17.
1 6 J La State Med Soc VOL 1 52 January 2000
Figure 3. Contrast enhanced, delayed phase CT scan image through the upper abdomen.
Radiology Case of the Month Presentation is on page 16.
RADIOLOGIC DIAGNOSIS— Hemangioma of the liver PATHOLOGIC DIAGNOSIS— Same
INTERPRETATION OF IMAGING
Figure 1 demonstrates an approximately 11x10 cm, well demarcated, heterogeneously echogenic mass in the right lobe of the liver. Fig- ure 2 demonstrates the same mass, which shows nodular-globular peripheral enhancement on early contrast phase. In Figure 3 we see how the areas of contrast enhancement become confluent as the tumor fills centripetally.
DISCUSSION
Hemangiomas are considered the most com- mon benign neoplasm of the liver, occurring in 15% to 20% of autopsy studies. They are typically asymptomatic, can be single or multiple, 3 cm to 4 cm in diameter, most frequently occurring in the right hepatic lobe, and often subcapsular. There is a significant female predominance of 5:1 over male occurrence. Though usually detected incidentally, patients may present for evaluation of acute or chronic abdominal complaints secondary to rup- ture or mass effect caused by large tumors. These large tumors, greater than 8 cm to 10 cm are re- ferred to as giant cavernous hemangiomas, as in the case being presented.
Histologically, hemangiomas are composed of large capillary channels filled with blood or lymph. In the liver these channels are lined by a layer of endothelial cells without bile ducts and are sepa- rated by fibrous septae.1 Hepatic hemangiomas can demonstrate fibrosis, calcifications, or hemor- rhage.
Diagnosis of hepatic hemangiomas on imag- ing studies is usually not difficult as they have typi- cal appearances on ultrasound, CT, and MRI. On ultrasound, hemangiomas most commonly appear as well delineated, uniformly hyperechoic masses, often with some acoustic enhancement. Larger tu- mors are often heterogeneous due to necrosis, thrombosis, and fibrosis (Figure 1).
CT scan evaluation should include pre-con- trast as well as early phase post-contrast and de-
layed imaging. On pre-contrast images, hemangio- mas appear as well-defined hypodense masses. Following IV contrast injection, early contrast phase images should demonstrate nodular-globu- lar peripheral enhancement.2 This finding is the most reliable sign that helps distinguish heman- giomas from hepatic metastases (Figure 2). This peripheral nodule-like enhancement has been found to be 88% sensitive and 84% to 100% spe- cific for hemangiomas.3 In the delayed phase of CT imaging, areas of enhancement become confluent as the tumor fills centripetally with con- trast enhancement persisting for 20-30 minutes (Figure 3). If the lesion has fibrosis or necrosis these areas will remain hypodense with time.
Both Tc-99 RBC scintigraphy with SPECT im- aging and MRI with and without IV contrast have also shown to have very high positive predictive value.
REFERENCES
1. Kumar V, Cotran RS, Robbins SL. Basic Pathology, 5th edition. Philadelphia, Pa: WB Saunders; 1992:301.
2. Mergo PJ, Ros PR. Benign lesions of the liver. Radiol Clin North Am 1998;36:319-331.
3. Leslie DF, Johnson CD, Johnson CM, et al. Distinction between cavernous hemangiomas of the liver and hepatic metastases on CT: value of contrast enhancement patterns. AJR 1995;164:625-629.
Dr Calimano is Junior Radiology Resident at Louisiana State University Health Services Center in New Orleans, Louisiana.
Dr Ferret is Associate Professor of Radiology at Louisiana State University Health Services Center in New Orleans, Louisiana.
Dr Neitzschman is Associate Professor of Radiology, Orthopedics, and Nuclear Medicine at Louisiana State University Health Services Center
in New Orleans, Louisiana.
J La State Med Soc VOL 1 52 January 2000 1 1
IE JOURNAL 150 & 100 YEARS AGO
JANUARY 1850 AND 1900
Gustavo Colon, MD
There is an article on Apoplexy of the South, its pathology and treatment by Dr Samuel Cartwright. He states that there is a differ- ence in apoplexy, which we define today as a stroke, but which was defined at that time as a brain seizure which could either be a stroke or a seizure (fit). He defines the disease as being a complete or partial suspension of "life of relation", which had two functions: one by which impressions from without reached the brain, that is, external stimuli, and the other which the brain exercises on the body, the internal functions of the brain on our complete organic system.
The brain can neither act nor be acted upon except in a very imperfect manner when an apo- plexy occurs. Both of these orders of function in a healthy state are equivalent to each other and both require a proper degree of activity of the circulat- ing system for performance. However, apoplexy occurs when the two orders of function cease to be equivalent; in other words, a "super abundant simulation or a defect of excretory function". He states that the first problem in constituting apo- plexy is the depletion of red and the latter deple- tion of black blood, and that the one connected with
red blood is arterial reaction and the other one is secondary to venous congestion in the brain and that these arise not from the brain itself but as a consequence of a defect in the secretory and ex- cretory functions of the body itself and generally in the South or in warmer weather. The best cure is to awaken all the absorbing and secretory and excretory glands from their dormant state into in- creased activity particularly those glands and fol- licles in the immediate vicinity of the congested brain. The congestion itself occurs because of the increased action in the excretory system and this is evidenced in the symptoms that occur in apo- plexy or a seizure in which copious amounts of excretions of viscid mucus occurred during the apoplexy fit with abundant secretions of the lar- ynx, trachea, and esophagus that require a great deal of suction to remove and can occasionally cause death by obstructing the respiratory organs. As a matter of fact, the Dominican Friars of the middle ages had an elixir "antapoplexia" which was nothing more than a combination of power- ful antiphlogistic ingredients calculated to de- crease the inactivity of the trachea, larynx, esopha- gus, and naris and to increase the secretion of their
1 8 J La State Med Soc VOL 1 52 January 2000
mucous membranes. In order to excite further se- cretions, it was felt the remedy had total success in curing the disease in all of southern Europe. They advocated the following treatment for apo- plexy: "2 teaspoons of table salt, 2 teaspoons of mustard flour, 1 teaspoon of ipecac, and 1 teaspoon of tincture of a gum resin in a tumbler of warm or cold water". The more disgusting the medicine the better it is because it loosens the tenacious phlegm adhering to the throat and air patches and creates further secretions. The pungency of the mustard is all important for the same purpose. The throat is so choked up with mucus and phlegm that swal- lowing anything is impossible but the mixture does create good without being swallowed, its mere presence in the mouth loosens the tenacious phlegm adhering to the back of the throat, caus- ing it to pour out of the mouth, and arrests the stertorous breathing caused by the phlegm in the throat which enables the patient to breathe easier by creating further secretions and thinning out the thick phlegm that they have secondary to the sei- zure or apoplexy.
However, it is better for the patient to swal- low the mixture until he vomits or it acts on the bowels. It should be forced into the throat by hold- ing the mouth open with a spoon and into this the mixture should be poured with another spoon. When it falls down the root of the tongue, it causes a heating, strangling kind of motion made by the patient; then he should be turned a little on the side to enable the loosened phlegm and the in- creased secretions to run out the mouth. But soon the patient should be placed on his back again with the head a little elevated to get more medicine.
While this is going on, hot water with mus- tard should be poured time after time on the patient's feet and hands, and a flannel shirt, rung out of very hot water, doubled up in a large ball and wrapped in a dry flannel, should be applied over the stomach and bowels and frequently re- newed as hot as the hands can bear it. A great deal of phlegm and a ropy, white, egg-looking sub- stance will be thrown up and the patient will get relief. A chamomile may be given to encourage the vomit. If the head is hot and the face red, then the head and face should frequently be wet with cold water. When the skin gets hot, the pulses rise and the face is flushed, bleeding from the arm should be resorted to at that time but it is a very danger-
ous expedient at this point and should only be re- sorted to as a last measure. After the vomiting, a 20-grain dose of chamomile floating on a spoon of water should be given along with a stimulating enema to move the bowels. Subsequent treatment consists of little more than a gruel diet, a little salt, and very small doses of sweet alcoholic spirits to act on the kidneys. If the patient can be made to vomit, he almost invariably regains his facilities. Some physicians have theoretical fears of vomit- ing but it never does mischief to the head in any case except where there is great heat on the whole surface and a flushed face. In that case, bleeding and vomiting at the same time may be well used together. The means that have been recommended should be well fixed in the mind beforehand as to what needs to be done. Everything should be done in conjunction in order to ameliorate the apoplec- tic event. He states finally that the greatest num- ber of cases of apoplexy in the South occur in the summer months. In a cold climate, apoplexy oc- curs because of a surplus of arterial blood in the cerebral vessels whereas in a hot, damp climate the reverse is the case. There is an accumulation of blood in the venous system and the treatment which is recommended is to increase the secretion and minimize the congestion within the venous system particularly around the head and neck area.
The January 1900 issue of The Journal has some points of interest about different diseases and how they should be treated. Heart disease from an obstetric point of view is briefly summarized as follows. (1) A woman having a heart lesion which is compensated should not be prevented from marrying. (2) Abortion should not be induced on a woman with disease unless her symptoms are present. (3) Premature labor should seldom or never be induced on account of heart disease. (4) Mitral stenosis is the most serious heart lesion dur- ing pregnancy and labor, aortic stenosis comes next and then probably aortic incompetency. Mitral in- sufficiency is the least serious lesion. (5) Treatment during pregnancy — administer strychnine, digi- talis, cathartics, and nitroglycerin, and regulate the diet. (6) During labor, keep up the action of digi- talis especially during the first stage; give strych- nine and stimulants if required and chloroform as indicated; and as soon as the first stage is com- plete deliver with forceps. (7) Watch the patient carefully during the third stage, which is the most
J La State Med Soc VOL 1 52 January 2000 1 9
dangerous time, and for some days after.
There is another list of persons who should not take alcohol: (1) those who have a family his- tory of drunkenness, insanity, or nervous diseases; (2) those who have used alcohol in excess or in childhood; (3) those who are nervous, irritable, or badly nourished; (4) those who suffer from inju- ries to the head, diseases of the brain, and sun stroke; (5) those who suffer from great bodily weakness particularly during convalescence from exhausting diseases; (6) anyone who engages in exciting or exhausting climates in bad air and in the surroundings of workshops and mines; (7) those that are solitary and lonely and require amusement; (8) those who have little self control either hereditary or acquired; and (9) those who suffer from brain weakness as a result of senile degeneration.
This is how to avoid consumption: (1) a gen- erous diet of nutritious food; (2) free ventilation of a dwelling and sleeping by open windows; (3) adequate house heating in the winter; (4) boil all milk and cream prior to using; (5) obtain 8 hours of sleep, if not sound sleep contract hours to 7 and rest during the day; (6) if debilitated with weak digestion, rest in a recumbent position shortly be- fore and after meals; (7) wear loose clothing espe- cially around the waist and lower ribs to afford freedom of respiration; (8) take systematic daily exercises in the open air or on foot; (9) if means and station in life permit, take a long holiday from time to time and live during fine weather in a tent in the open air or in a summer house for most of the day; and (10) if unemployed, pursue a hobby to occupy the mind.
From the Department of Ear, Nose and Throat is the following case of vicarious menstruation from the ear which was reported from Paris. It states its subject was a 17-year-old girl who for 3 years had been having regular monthly discharges of blood from the right ear lasting 3 or 4 days and accompanied by all of the phenomena associated with menstrual periods. The patient suffered with headaches, malaise, and slight tenderness about the ear with each recurrence of hemorrhage. These symptoms disappeared upon its cessation. Exami- nation of the ear revealed a perfectly normal tym- panic membrane but a number of small varicose vessels occupied the walls of the osseous portion of the meatus which was the site of the hemor-
rhage. The genital menstruation appeared at age 17, but the discharge from the ear, instead of ceas- ing, accompanied the vaginal flow, in addition at times associated with epistaxis and hemorrhage from the mouth. The patient was in good general health and presented no evidence of being hemo- philic or hysteric. The hearing was not materially affected. The conclusion is that menstruation from the ear is an extremely rare condition, but a few similar cases have been reported in the past.
Dr Colon has a plastic surgery practice in Metairie, Louisiana and has lectured on the history of medicine at Lousisiana State University School Health Services Center, in New Orleans, Louisiana and Tulane University School of Medicine in New Orleans, Louisiana.
The author and The Journal welcome comments on the history of
medicine.
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20 J La State Med Soc VOL 1 52 January 2000
HOW CAN GENETICS HELP IN THE MANAGEMENT OF OBESITY?
Theodore F. Thurmon, MD
Obesity usually results from unwanted variations in metabolism. Inadequate neurotransmission, ther- mogenesis, or acylation underlie about 90% of cases. These are complex, weakly heritable, polygenic traits. Mutations in major gene loci cause another 5% of cases, and still another 5% of cases are due to gluttony. Careful observation can help define the type of obesity. All forms are associated with excess mortality and require lifelong episodic or continuous management. Management centers around diet, exercise, behavior therapy, and life-style counseling. Serotonin agonists and serotonin uptake inhibitors, as well as alternative therapies like phototherapy and 5-hydroxytryptophan are worthwhile for neurotrans- mitter inadequacy, except in children and pregnant women. When thermogenesis is inadequate, intake may be normal and weight reduction may require subnormal intake. Some degree of obesity may be required for optimal health in patients with inadequate acylation. In some Mendelian syndromes, obe- sity may balance a metabolic error, and weight reduction may restore metabolic imbalance.
The body mass index or BMI (weight/ height) is a working standard for defin- ing obesity. A working standard for expres- sion of the adverse effects of obesity is the all-cause excess mortality. Older definitions assumed a sort of constancy in BMI and in its relationship to mor- tality. More recent data clearly show changes in both with age. The same BMI is associated with greater excess mortality at younger ages. Percen- tile distributions of BMI and age are available,1 so there remains little reason to use ill-fitting assump- tions of constancy. The 90th percentile of the
NHANES II BMI for age2 closely matches the dis- tribution of excess mortality. For purposes of this discussion, it will be used as the definition of obe- sity.
Careful studies have not revealed major meta- bolic defects as causes of the majority of cases of obesity. Instead, there is a group of relatively subtle metabolic variations that are easiest to recognize after obesity has become established. These varia- tions can produce obesity rapidly if intake is pro- digiously increased. More typically, they cause a mild, long term, positive imbalance between food
J La State Med Soc VOL 152 January 2000 21
intake and energy expenditure that results in obe- sity.
Prior to the Genome Project, variations in neu- rotransmission, thermogenesis, and acylation were found to be important to the pathogenesis of obe- sity. The Genome Project diverted research efforts into searching for the culprit genes. Numbers of those genes have been found but none that ac- counts for a significant proportion of cases of obe- sity. Instead, genetic aspects of most cases of obe- sity are similar to those that underlie other body characteristics: important, but not due to identifi- able individual genes.
Attention has now returned to the pathogen- esis of obesity. In the seminal work in the 1980s, the Wurtman team observed obese subjects in a closed environment.3 That work provided impor- tant leads to the etiology of obesity. By following one of the leads, that team substantiated the neu- rotransmitter mechanism. Recent work has been summarized by Jeanrenaud et al.4 Liebel et al docu- mented the acylation mechanism.5 A description of a current project and a comprehensive bibliog- raphy of recent work in acylation are provided by Guo et al.6 Jequier7 promoted work on the thermo- genesis mechanism in humans. Recent work in thermogenesis is described by Schrauwen et al.8 Rare cases of variations in these mechanisms may be due to major genes. Major genes have also been found to cause other problems that lead to rare cases of obesity.9
Metabolic variations that underlie obesity may predispose to ill health when famine renders food in short supply. Persons with a neurotransmitter problem may suffer insomnia and chronic fatigue. Persons with a thermogenesis problem may tend toward hypothermia. Those with an acylation problem may have hypotension and immunode- ficiency. During famine, the proportion of persons who are asthenic, sickly, and short-lived due to those problems may approximate the proportion of obese persons when food is plentiful. Effects of rare major genes for obesity may also result in poor health during deprivation.
POLYGENIC OBESITY
Investigators of obesity often work under the assumption of homogeneity. Heterogeneity among obese patients was documented by Jequier. He found that about one third of obese patients had
inadequate thermogenesis. Inclusion of other pa- tients in previous studies had hampered discern- ment of the importance of inadequate thermogen- esis. The Wurtman team found about the same fre- quency of inadequate neurotransmission. Studies of heterogeneity of inadequate acylation allow a similar estimate of its frequency. Even within each category, there is heterogeneity, as the degree of inadequacy may vary from mild to severe.
Not all persons with these problems are obese. Frugal eating habits and an active life style arrived at for reasons other than weight control may re- sult in normal weight. If that equilibrium is dis- turbed, obesity may then occur in a person who seemingly had no problem previously. The fre- quency of these metabolic problems suggests that ready availability of highly nutritious foods com- bined with societal promotion of poorly-controlled eating habits and a sedentary life style could even- tually push the frequency of obesity as high as 50%.
Combined defects are evident in numbers of reports but there has been no study to determine their frequency. These are such common problems that many obese patients may have combinations of one or more of them. Other metabolic problems that may be exaggerated in Mendelian disorders, like fat storage, insulin effects, hormone effects, and energy expenditure, are not clearly separate from these. Some characteristics of obesity, such as body fat distributions, have more to do with the consequences of obesity than with its acquisi- tion.
Prescriptive feeding of children will usually delay manifestation of these problems until eman- cipation, as significant, long-term excessive intake is required. De facto emancipation may occur quite early for latch-key children. When obesity occurs in childhood, it has the same characteristics as in adulthood. However, except in the case of ther- mogenesis defects, the food must be provided by a caretaker or a group of caretakers. Successful management involves identifying the source of the food.
Neurotransmission
The central theme is that metabolites of food may act as signal molecules or may stimulate pro- duction of signal molecules that ordinarily reach the brain via the blood stream and staunch further eating. If that process is blunted, obesity is likely.
22 J La State Med Soc VOL 1 52 January 2000
Intake is excessive. Quests for just the right food to satisfy the appetite are typical of this form of obesity. Nocturnal hyperphagia may be a prereq- uisite for sleep, and lack of it may produce sleep disorder and chronic fatigue. The plasma tryp- tophan/large neutral amino acids ratio, which normally rises after a carbohydrate meal, does not change in these patients. That test is not available for clinical use. Serotonin and similar neurotrans- mitters are at the end of the signaling pathway. Maneuvers that promote serotonergic activity in the brain normalize food intake.
Acylation
Most of the energy derived from food is stored in fat cells as triglyceride. The main way in which this stored energy can be utilized is through lipoly- sis into fatty acids that are released into the blood. An acylation mechanism within the fat cell nor- mally recycles about half of the fatty acids back into triglycerides before they reach the blood. De- pression of that mechanism leads to excessive re- lease of fatty acids. Depletion of intracellular trig- lyceride stimulates hunger and the resultant ad- ditional intake causes obesity. Intake is excessive. Obsession with food may be a marker of this type of obesity. Affected persons may have gourmet tendencies. Documentation of postprandial fatty acid release is difficult because the process is un- evenly distributed throughout the body. Signifi- cant weight reduction may lead to low thyroid function, leukopenia, hypotension, bradycardia, amenorrhea, and malaise.
Thermogenesis
About 15% of bodily energy expenditure nor- mally produces heat, mainly through oxidation of fat during cellular respiration. If this process is inadequate, the energy is stored as fat instead, and obesity results. Typically, food preferences are not unusual, and intake is normal. This type of obe- sity may be typical of isolated populations in hot climates where the food supply is marginal.
In animal models, thermogenesis is a function of brown adipose tissue and can be measured over body parts where brown adipose tissue is local- ized. Humans have additional steps in adipose tis- sue development that disperse the brown adipose tissue. The distribution is uneven throughout the
body and different in different people so thermo- genesis cannot be easily evaluated. The respira- tory chamber is used for research but is impracti- cal for clinical purposes. Significant weight reduc- tion may lead to lowered basal metabolic rate (BMR), cold intolerance, poor stress responses, and, eventually, malnutrition.
GLUTTONY
In colonies maintained for research purposes, rats are ordinarily fed a "chow" that is totally nu- tritionally adequate but lacks attractive excipients. It is common practice to keep a hopper filled with chow to ensure an adequate food supply. Despite this excess of available food, the rats do not gain weight excessively. In cafeteria diet experiments, new and palatable foods similar to those in a caf- eteria line were provided to the rats daily. That quickly and uniformly caused obesity. Return to the diet of chow resulted in return to normal body weight. Repetitive experiments with the same rats resulted in return to normal weight each time, with no lasting effects on metabolism.
The "cafeteria diet" rat is a model for the prob- lem of gluttony in humans. Rats have little self- control or judgment, both of which are required for weight control in a milieu of readily available, palatable foods. Patients affected by gluttony may be deficient in these personality characteristics and may exhibit impulsiveness and denial. They may represent themselves as lovers of good, ordinary food. Intake is excessive but there is no metabolic abnormality. A genetic tendency has not been iden- tified but the known heritability of personality traits suggests that there may be one. Unlike in other forms of obesity, weight reduction is rela- tively simple and safe. Like in other forms, relapse is frequent. Gluttony probably accounts for about 5% of cases of obesity at present but could be ex- pected to rise in frequency if palatable food be- comes even more available.
This type of obesity may also be found in pa- tients with more serious problems such as psychi- atric disorders and the anorexia-bulemia spectrum. In these disorders, standard obesity treatment ap- proaches may have paradoxical or untoward re- sults so efforts should be made to identify them before embarking on a course of therapy. Third party observers are invaluable in this regard. The
J La State Med Soc VOL 1 52 January 2000 23
|
Table 1 Typical Major Genes for Obesity (DNA testing is currently unavailable for any of them) |
|||
|
OMIM |
Locus |
Gene, abbreviation, inheritance |
Process causing obesity |
|
109690 |
5q32 |
Beta-2-adrenersic receptor, ADRB2, Autosomal dominant. Decreased energy expenditure. |
Decreased lipolysis |
|
109691 |
8p12 |
Beta-3-adrenergic receptor, ADRB3, Autosomal dominant. Decreased lipolysis. |
Decreased thermogenesis |
|
118444 |
4p15.2 |
Cholecystikin A receptor, CCKAR, Autosomal dominant. Intestinal motility disorder. |
Central hyperphagia |
|
164160 |
7q31 .3 |
Leptin, LEP, Autosomal dominant. |
Central hyperphagia |
|
155541 |
18q22 |
Melanocortin 4 receptor, MC4R, Autosomal dominant. |
Central hyperphagia |
|
601487 |
3p25 |
Perioxisome proliferator activated receptor, gamma, PPARG, Autosomal dominant. Insulin resistance. |
Hyperlipidemia |
|
176830 |
2p23.3 |
Proopiomelanocortin, POMC, Autosomal dominant. Decreased energy expenditure. |
Central hyperphagia |
|
162150 |
15q15 |
Proprotein convertase subtilisin/kexin type 1 , PCSK 1 , Autosomal dominant. |
Impaired prohormone processing |
|
602044 |
1 1 ql 3 |
Uncoupling protein 3, UCP3, Autosomal dominant. |
Decreased thermogenesis |
|
601007 |
1p31 |
Leptin receptor, LEPR, Autosomal recessive. |
Hypothalamic hyperphagia |
binge eating that is a hallmark of the anorexia- bulemia spectrum may be difficult to differentiate from the satisfaction-seeking activities of neu- rotransmitter inadequacy. When it is possible to differentiate the two, it is usually on the basis of the more generalized disturbance of personal and occupational relationships that may typify the an- orexia-bulemia spectrum.
MAJOR GENES
Major genes for obesity have been located on every chromosome except Y. Nearly half have been identified through homology with genes of other species. Those genes are not usually independent causes of actual cases of obesity in humans but they provide vital clues to the genetic background of obesity. Some of their effects include decreased energy utilization, excessive fat storage, hyper- phagia, impaired prohormone processing, insulin resistance, and decreased thermogenesis. Some of the variations are similar to those of polygenic obesity but none of these genes has been associ-
ated with it. McKusick10 provides extensive docu- mentation. A free online version is more current (OMIM).11 Also documented in OMIM are several reports of single cases or single families of curious syndromes in which obesity may be primary. OMIM contains discussions and literature citations about the remainder of the disorders discussed in this section.
Most of the major genes associated with obe- sity have been discovered through rare families in which obesity follows a Mendelian inheritance pattern: autosomal dominant (inherited from an affected parent), autosomal recessive (1/4 of sibs affected, parents consanguineous), or X-linked (brothers, maternal uncles, and sons of maternal aunts affected). All are quite rare. Actual counts of numbers of cases that have been identified al- low an estimate that these genes, in toto, account for no more than 5% of cases of obesity.
The family genealogy pattern is usually the only clinical clue to the presence of one of these genes. Clinical use of DNA analysis for diagnosis
24 J La State Med Soc VOL 1 52 January 2000
|
Table 2 Typical Syndromes with Features that Lead to Obesity |
|||
|
OMIM |
Locus |
Gene, abbreviation, inheritance |
Process causing obesity |
|
103581 |
15q1 1 |
Albright hereditary osteodystrophy 2, AH02, Autosomal dominant. |
Hormone resistance |
|
103580 |
20q13.2 |
Albright hereditary osteodystrophy, AHO, Autosomal dominant |
Hormone resistance |
|
107730 |
2p24 |
Apolipoprotein B, APOB, Autosomal dominant |
Hyperlipidemia |
|
139250 |
1 7q22 |
Growth hormone 1*, GH1, Autosomal dominant |
Insulin resistance Hyperlipidemia |
|
144800 |
? |
Hyperostosis frontalis interna, Autosomal dominant |
Hyperprolactinemia |
|
184700 |
? |
Polycystic ovary syndrome 1, PCOI, Autosomal dominant. |
Insulin resistance |
|
190160 |
3p24.3 |
Thyroid hormone receptor, beta*, THRB, Autosomal dominant |
Thyroid hormone resistance |
|
190430 |
? |
Triglyceride storage disease - type II, Autosomal dominant |
Hyperlipidemia |
|
*DNA test available |
is impractical at present. The genes control diverse metabolic processes (Table 1). There are no gener- alities such as the most common gene, chromo- some, or metabolic process. In these syndromes, obesity may be a compensatory mechanism to bal- ance a metabolic error. Weight reduction may re- turn the patient to metabolic imbalance.
Obesity is non-randomly associated with sev- eral syndromes as a secondary effect. In achondro- plasia (OMIM 100800), there may be decreased energy expenditure due to unusual body confor- mations. Soft tissue growth, though normal, may be out of proportion to bone growth, producing a high body mass index. Decreased energy expen- diture may result from the deformities of acrocephalosyndactyly III (Carpenter syndrome. OMIM 201000). Pain from subcutaneous lipomas of adiposis dolorosa (OMIM 103200) may result in decreased activity and consequent decreased energy expenditure. Choroideremia-deafness-obe- sity (OMIM 303110) is an example of a contiguous gene syndrome. A long deletion inactivates all of
the genes in the neighborhood. A gene for obesity may exist in that area of the X chromosome. It is curious that obesity is also non-randomly associ- ated with another eye disorder, corneal dystrophy- obesity (OMIM 122000). Decreased vision may lead to decreased activity. Obesity in Cushing syn- drome (OMIM 219080, 219890) is well known but a pathogenic mechanism is unclear.
A number of other syndromes that may not be considered " obesity syndromes" have aspects that lead to obesity (Table 2). Clinical features would suggest the presence of one of these syn- dromes and standard diagnostic testing would document it. DNA testing is also available for some of them. A Mendelian family history would usu- ally be found.
The classical recognizable obesity syndromes are outlined in Table 3. One of the strongest indi- cations of the presence of one of these syndromes is mental defect, which occurs in all but two of them. A very careful diagnostic evaluation for these syndromes is indicated for any obese patient with
J La State Med Soc VOL 1 52 January 2000 25
|
Table 3 Classical Recognizable Obesity Syndromes |
||
|
OMIM |
Locus Syndrome, inheritance |
Recognizable features |
|
203800 |
2p1 4 Alstrom syndrome*, Autosomal recessive |
Retinitis pigmentosa, progressive deafness, nephropathy, diabetes |
|
209901 |
1 1 ql 3, Bardet-Biedl syndrome*, 16q21, Autosomal recessive 15q22.3, 2q3 |
Mental defect, pigmentary retinopathy, renal malformation, hypogenitalism, polydactyly |
|
210350 |
? Biemond syndrome II, Autosomal recessive |
Mental defect, coloboma, short stature, hypogenitalism, polydactyly, |
|
301900 |
Xq26.3 Borjeson-Forssman-Lehmann syndrome, X-linked recessive |
Mental defect, epilepsy, hypogonadism, hypometabolism, swelling of subcutaneous tissue of face, narrow palpebral fissure, large ears |
|
309490 |
? Chudley syndrome, X-linked recessive |
Mental defect, short stature, hypogonadism, bitemporal narrowness, depressed nasal bridge, short and inverted-V-shaped upper lip, macrostomia |
|
216550 |
8q22 Cohen syndrome, Autosomal recessive |
Mental defect, high nasal bridge, strabismus, large ears, prominent incisors, narrow hand, tapering fingers |
|
601794 |
? Coloboma-obesity-hypogenitalism-mental retardation, Autosomal dominant |
Mental defect, microphthalmia, coloboma, cataract, hypogenitalism |
|
300148 |
Xp22.13 MEHMO syndrome, X-linked recessive |
Mental defect, epilepsy, hypogonadism and hypogenitalism, microcephaly |
|
157980 |
? MOMO syndrome, Autosomal dominant |
Mental defect, macrocephaly, coloboma, nystagmus, down eye slant, delayed bone maturation |
|
176270 |
1 5q1 1 Prader-Willi syndrome*, Autosomal dominant |
Mental defect, neonatal hypotonia, bitemporal narrowness, small hands & feet, hypogonadism, short stature, diabetes |
|
181450 |
1 2q24.1 Ulnar-mammary syndrome*, Autosomal dominant |
Breast hypoplasia, axillary apocrine gland hypoplasia, ulnar hypoplasia, malformation or absence of fingers 4 and 5 including metacar- pals, small penis, delayed puberty, anal atresia, pyloric stenosis, congenital subglottic cartilaginous web |
|
309585 |
Xp21 .1 Vasquez syndrome, X-linked recessive |
Mental defect, gynecomastia, hypogonadism, short stature, tapering fingers, small feet |
|
*DNA test available |
26 J La State Med Soc VOL 152 January 2000
a mental defect. The syndromes often entail a spec- trum of abnormalities about which it is important to know for management and prognosis. Most of them also entail a considerable genetic risk to prog- eny of the patient or relatives. Pathogenesis of the obesity in these syndromes is indefinite but com- pulsive eating seems to be a final common path- way. Some work in the Prader-Willi syndrome has shown decreased energy utilization and decreased lipolysis. Weight reduction may be safely accom- plished through metered intake in most cases.
GENETIC COUNSELING
Ponderosity has long been known to be famil- ial but studies of actual inheritance of obesity have been confounded by non-genetic familial and cul- tural factors. Cases that are clearly due to a syn- drome are rare, but standard Mendelian risk fig- ures adequately characterize the likelihood of obe- sity in relatives in those cases: autosomal domi- nant (50% risk to progeny of affected persons), autosomal recessive (25% risk to sibs of affected persons), or X-linked (50% risk to brothers of af- fected males). In some cases, testing for the pres- ence of the gene or the metabolic error makes risk estimation more accurate for individuals. Sources of tests are available online at http: / / www.genetests.org/ .
For the more common polygenic obesity, the most informative data are from studies that com- pare adoptees to their biological and adoptive par- ents, and twins reared together and apart. Actual risk figures derived from those studies indicate a genetic risk of about 40% for obesity in the monozygotic twin of an obese person and about 3% for obesity in other first degree relatives of an obese person. The figures are probably accurate for all variants of polygenic obesity. Mendelian inheritance is associated with higher figures. A high frequency of obesity in a family, particularly if it is limited to one side of the family, indicates a need for careful diagnostic investigation for fea- tures of a Mendelian syndrome
Early feeding influence is a time-honored as- pect of the maternal effects that heighten the simi- larity among sibs above that expected due to ge- netic factors. It could lead to a concentration of obesity due to entrained gluttony among children of women whose obesity is due to any number of other causes. However, studies place that likeli-
hood at about 4% or about the same as the fre- quency of gluttony in general.
There is decided assortative mating for quan- titative traits. The best-documented effect of as- sortative mating on a quantitative trait is that of intelligence, in which the average intelligence of progeny is above two standard deviations if both parents are above two standard deviations. Assor- tative mating for obesity has not been documented to that degree but it is quite likely that progeny could be obese on average if both parents had the same variety of polygenic obesity. That would pro- duce a bilateral family history of obesity that would not be typical of a Mendelian pattern.
CODING AND TREATMENT
ICD9-CM codes that include "obesity" in ter- minology are inaccurate and misleading. Payment plans may disallow services based on those codes. Obesity is an end result. To code for obesity per se is no more meaningful than to code for the depig- mentation that is an end result of phenylketonuria. To include "obesity" in the description of the prob- lem is no more necessary than it is to include "de- pigmentation" in the description of phenylketo- nuria. Current codes in the ICD9-CM are not opti- mal for polygenic diseases so the closest similar code should be used. Accurate terms and codes for patients with polygenic obesity are as follows: Predominant neurotransmitter inadequacy, 270.2; Predominant acylation inadequacy, 272.9; Pre- dominant thermogenesis inadequacy, 271.9; Com- bined metabolic inadequacies, 277.9. The most optimal code for gluttony is 307.5. There are spe- cific codes for many of the Mendelian disorders and syndromes. Close attention to the actual de- fect allows for accurate coding of the others.
The key to treatment of obesity is the realiza- tion that it results from constitutional variations that are lifelong and cannot be cured. Management and control require either continual or episodic treatment. Treatment is difficult in all cases and dangerous in some, but the margin of success can be improved by tailoring the treatment to the un- derlying cause. One process may be the pre- dominant one. In most studies, obese patients sig- nificantly underreport intake; however, if normal intake can be unequivocally established, thermo- genesis is likely to be the major process at fault. Other polygenic processes may be more difficult
J La State Med Soc VOL 152 January 2000 27
to recognize, but careful interview, including ob- servations of relatives and acquaintances, may elicit recognizable features. Recognizable features may also arise during therapeutic trials.
A Mendelian distribution of obesity in the family is an indication of the presence of one of the major genes for obesity. Currently, there is no other practical diagnostic approach. Treatment for Mendelian obesity is the same as for polygenic obesity except for added caution in regard to pos- sible metabolic decompensation. Syndromes with features that lead to obesity may have to be treated in the same manner; however, treatment of the syndrome itself may resolve the obesity. For ex- ample, hormonal therapy of the polycystic ovary syndrome counters insulin resistance and reduces abdominal fat deposition.
One of the problems in obesity treatment is defining an endpoint. If there is a clear time of onset of obesity, photographs, weight records, and clothes sizes allow an endpoint to be defined as a return to the pre-obese state. Decrements of body mass index toward the 50th percentile represent significant progress in control of obesity. If that process plateaus at a point where diet, eating be- havior, and life style seem optimal, one has reached a working endpoint. Patients who become over- zealous about obesity treatment and who were formerly below the 50th percentile for body mass index, may drop past the 50th percentile during treatment. If the body mass index in those patients drops to the 10th percentile, the physician should call a halt to the process and try to stabilize weight at that point. Psychiatry consultation is indicated if weight drops lower and the process seems to be out of control.
The multiple processes that lead to obesity make it unlikely that currently available drug treat- ment will benefit the majority of cases. It is most applicable in patients with inadequate neurotrans- mitter mechanism. Some types of depressive dis- orders share the same biochemical problem as the neurotransmitter defect and may co-exist with obesity, requiring more specific management. Anti-obesity drugs may have unfortunate effects on patients with other psychiatric disorders or may have untoward interactions with drugs used to treat them.
Serotonin agonists and serotonin uptake in- hibitors are the most popular anti-obesity drugs.
Most of them effectively staunch eating in patients with neurotransmitter inadequacy but are subject to addiction and tachyphylaxis as well as untoward side effects. Online services like http:// www.mdconsult.com/ can provide current au- thoritative information. Search it for " obesity treat- ment benefits". None of the drugs has a long-term advantage over diet and exercise. Alternative therapies like phototherapy or 5-hydroxytry- ptophan may be equally effective but require close observation for currently unknown side effects.
Phototherapy involves sitting under a bank of fluorescent lights providing at least 1500 lux to face and shoulders from 6 a.m.-8 a.m. each morn- ing, initially for 10 days, then on Monday and Thursday as maintenance. The dose of 5- hydroxytryptophan is 300 mg 3 times daily, 30 minutes before main meals. If there is a place for this drug, it is in young adult obese patients with no co-morbidity. It may be hazardous in hypertensives, in patients with sympatho amine- secreting tumors, and in other disease states. Drug interactions have not been investigated.
Currently approved drugs have little efficacy in problems of acylation, thermogenesis, or glut- tony other than an evanescent non-specific anorec- tic effect. Cachectin (tumor necrosis factor alpha) is under study as a more general anti-obesity drug. It causes weight loss due to anorexia in experimen- tal models; however, its very nature makes dan- gerous side effects likely. The current therapeutic approach to obesity due to problems other than neurotransmission is the combination of diet, ex- ercise, behavior therapy, and life-style counseling. These are the only approved approaches to any type of obesity in children and pregnant women. Actual weight loss during pregnancy may be haz- ardous to the fetus.
Diet, exercise, behavior therapy, and life-style counseling should also be given concurrently with drug therapy or alternative therapies. Behavior therapy and life-style counseling are based on as- sumptions that the patient does not know about the importance of eating behaviors and life style in weight control, does not appreciate their sig- nificance, or is distracted. There are well-docu- mented assessment instruments that therapists can use to identify behavior and life-style problems and recommend resolutions. The patient is encour- aged toward a frugal diet and an active life style.
28 J La State Med Soc VOL 1 52 January 2000
Periodic reviews and reinforcement techniques are used. Excellent benefit has been shown from on- going or episodic rounds of behavior therapy and life style counseling. Older physicians may think of this as "brain-washing". It is outlined on http: / / www.mdconsult.com/ . Due to the time commit- ment, many physicians will find it best to refer the patient to a counseling service for these purposes. Professional dietitians may offer counseling and follow-through. Many hospitals now provide ex- ercise and counseling programs.
Some self-selection is necessary because the patient must provide the impetus for follow- through of treatment. Due to the chronicity of the problem, even the most highly motivated patients require encouragement, monitoring and recall by the physician. Excellent weight reduction diet plans are widely available, eg, http: / / www.mdconsult.com/. The error of undue con- centration on any one modality should be empha- sized. Dieting is seldom efficacious without behav- ioral therapy and life-style counseling. Exercise alone can be particularly disappointing, as the pro- digious amounts required to burn only a few calo- ries can lead to exhaustion. Emphasis should be on the place of an exercise program in an active life style and cardiovascular health rather than on any direct relationship between exercise and weight loss.
The mental defect that is typical of many clas- sical recognizable obesity syndromes requires en- listment of the caretaker in treatment. Management programs in these cases may already have opti- mized aspects other than diet; however, it is im- portant to review meal and exercise practices. It often will be found that the most advantageous changes are to arrange meals with correct calorie and nutrition content and to curtail access to any other foods.
The caretaker or caretakers must also be en- listed in the treatment of obesity of childhood, the difference being that there may be no pre-existing program to manage exercise and counseling. While caretakers may pose a significant barrier to man- agement of childhood obesity, the continual growth is a significant advantage. Minor successes in diet, exercise, behavior, and life style can lead to appreciable improvement. Because of the life- long nature of obesity, it is quite important to in- clude the child in all aspects of counseling, with
age-appropriate vocabulary. Plans should always be made for transition to adult management.
For patients who have the means, reputable retreats and spas have a real place in obesity man- agement as a respite and an opportunity to refresh the management regimen. However, some retreats and spas are useless or harmful. Before endorsing any adjunct like that, the physician should care- fully investigate and document the program. Like any other form of obesity management, these are without long-lasting effects and resort to them may be required recurrently, so they are of little value to patients of limited means.
Even a seemingly harmless weight reduction diet could have untoward results. Most are clearly inadequate for patients undertaking moderate to severe exertion and for patients with intercurrent medical illnesses. Patients whose obesity is a com- pensation for a rare Mendelian inborn error may revert to an uncompensated state, so contact with each patient must be maintained while on the diet and the diet should be discontinued if there are signs of decompensation. A weekly checkup is prudent. The marginal nutritional content of most weight reduction diets will lead to weight loss within 2 weeks. They are highly effective for glut- tony and serve as a demonstration of the source of the problem to the patient.
Weight gain on a standard weight reduction diet is usually a sign of cheating, which is likely in cases of neurotransmitter or acylation inadequacy. A more closely controlled diet such as the protein- sparing modified fast may be required for patients with those problems.12 That severe approach, if successful, may test the limits of therapeusis. Weight loss may occur at the expense of health in some cases. Patients with predominant neurotrans- mitter defect may experience sleep disorders and chronic fatigue. Patients with predominant acyla- tion defect may develop malaise. Some degree of obesity may be required to prevent them from laps- ing into malnutrition. After the diet period, pa- tients with neurotransmitter inadequacy will still be driven to eat and may benefit from recycling through drug therapy.
Patients with inadequate thermogenesis usu- ally do not require severe diets. Standard weight reduction diets are effective but these patients may have a "knife-edge" nutritional balance that re- quires careful tuning of intake. Intake below nor-
J La State Med Soc VOL 1 52 January 2000 29
mal is required for weight maintenance but not so far below normal that it compromises health. A BMR is a practical, objective assessment. A steady decline which passes -15% indicates that maximum dietary benefit has been achieved, and additional nutrients should be added. A practical approach is to cycle the patient between a normal diet and a weight-reduction diet.
In any form of obesity, weight loss should be considered a remission. There will be a relapse af- ter variable periods of time in almost all cases, usu- ally after behavioral and life-style adjustments fal- ter. Periodic evaluations will allow the treating physician to gauge this process and decide when to re-institute treatment.
REFERENCES
1 . Thurmon TF. A Comprehensive Primer on Medical Genetics. New York; Parthenon Publishing; 1999:321-322.
2. Simopoulos AP. Characteristics of obesity: an overview. Ann NY Acad Sci 1987;499:4-13.
3. Wurtman RJ, Wurtman JJ. Carbohydrates and depression.
Scientific Am 1989;Jan:68-75.
4. Jeanrenaud B, Cusin I, Rohner-Jeanrenaud F. From Claude
Bernard to the regulatory system between the hypothalamus and the periphery: implications for homeostasis of body weight and obesity. C R Seances Biol Fil 1998;192:829-841.
5. Leibel RL, Hirsch J, Berry EM, et al. Alterations in
adipocyte free fatty acid re-esterification associated with obesity and weight reduction in man. Am J Clin Nutr 1985;42:198-206.
6. Guo Z, Hensrud DD, Johnson CM, et al. Regional postprandial fatty acid metabolism in different obesity phenotypes. Diabetes 1999;48:586-592.
7. Jequier E. Energy expenditure in obesity. Clin Endocrinol
Metab 1984;13:563-580.
8. Schrauwen P, Walder K, Ravussin E. Fluman uncoupling
proteins and obesity. Obes Res 1999;7:97-105.
9. Echwald SM. Genetics of human obesity: lessons from mouse models and candidate genes. / Intern Med 1999;245:653-666.
10. McKusick VA. Mendelian Inheritance in Man: A Catalogue of Human Genes and Genetic Disorders. Baltimore: Johns Hopkins Press; 1998.
11. Online Mendelian Inheritance in Man, OMIM. Center for Medical Genetics, Johns Hopkins University (Baltimore, Md) and National Center for Biotechnology Information, National Library of Medicine (Bethesda, Md), 1998. World Wide Web URL: http: / / www.ncbi.nlm.nih.gov/ omim/
12. Seim HC, Rigden SR. Approaching the protein-sparing
modified fast. Am Earn Physician 1990;42(suppl 5):51S- 56S.
Dr Thurmon is a professor of Pediatrics in the Louisiana State University School of Medicine in Shreveport, Louisiana.
30 J La State Med Soc VOL 1 52 January 2000
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DANDY-WALKER SYNDROME:
PRESENTATION OF THE CONGENITAL MALFORMATION IN AN OLDER PATIENT
Praveen Reddy, MD; Prasad S.S.V. Vannemreddy, MD; Laurie Grier, MD; Anil Nanda, MD
Dandy- Walker syndrome, a congenital malformation of the hindbrain involving the cerebellum and the fourth ventricle, is a rare cranial abnormality that commonly occurs before the sixth or seventh week of development. It is usually diagnosed at birth or in early childhood; however, an occasional patient may first become symptomatic in adult life. A case of Dandy-Walker syndrome in a 58-year-old woman is reported because of the older age at presentation and relatively long asymptomatic period after birth.
The term Dandy- Walker syndrome (DWS) was coined by Benda in 1954.1 It is a con- genital anomaly of the central nervous sys- tem characterized by the triad of (a) a posterior fossa cyst due to abnormal dilatation of the fourth ventricle, (b) agenesis or hypoplasia of the cerebel- lar vermis, and (c) hydrocephalus due to atresia or absence of the foramina of Luschka and Magendie.2
It may be commonly associated with other neural (gyral anomalies ranging from agyria to polymicrogyria, agenesis of corpus collosum, in- fundibular hamartomas, posterior fossa lympho- mas and lipomas, aqueductal stenosis, syringomy- elia cerebellar hypoplasia, and occipital meningoceles) and non-neural (cleft palate, poly-
cystic kidneys, polydactyly, syndactyly, vertebral anomalies, cataracts, and retinal dysgenesis) ab- normalities.3
DWS usually manifests at birth or in early in- fancy with hydrocephalus, slow motor develop- ment, and mental retardation or seizures. In older children, symptoms of increased intracranial pres- sure such as irritability, vomiting and convulsions, or signs of cerebellar dysfunction such as ataxia and nystagmus may occur. It is unusual for DWS to be asymptomatic throughout infancy and child- hood and manifest late in adulthood. The oldest patient reported was 72 years of age4 with symp- toms of raised intracranial pressure and cerebel- lar and brainstem dysfunction. Pre-natal sonography can be used to detect DWS in utero or
J La State Med Soc VOL 152 January 2000 31
a diagnosis can be made antenatally by plain roent- genogram, ultrasound, cerebral angiography, CT, or MRI. 5 A case of Dandy-Walker cyst presenting in an older patient is reported.
CASE REPORT
A 58-year-old, African-American woman was transferred from a local nursing home with com- plaints of altered mental status of 6-8 hours dura- tion. Closer questioning of nursing home person- nel and relatives revealed a past medical history significant for hypertension, diabetes, and a left cerebrovascular accident in 1995. Unfortunately, no clinical or radiological reports of those hospi- talizations were available. Two years previously, the patient was placed in a nursing home because of residual weakness on her right side. The patient was able to perform routine daily activities by her- self and never complained of headaches, visual difficulties, or persistent nausea and vomiting.
At the time of admission, the patient was le- thargic, not oriented, and not responding to ver- bal commands. Pupils were 3 mm on both sides, round, and reactive to light. The patient was hyporeflexic on the right side and Babinski's was equivocal. Her laboratory workup was within nor- mal limits. A CT scan was done (Figure), which
Figure. Axial CT (non-contrast) demonstrates the posterior fossa cyst, communicating with the fourth ventricle. There is panventricular dilatation.
revealed hydrocephalus, and a large posterior fossa cyst communicating with the enlarged fourth ventricle. She required endotracheal intubation, as the sensorium gradually deteriorated. After dis- cussing the neurological condition of the patient, the family decided not to undergo any surgical intervention and consented to a DNR (do not re- suscitate) status. No further MRI, EEG, or cerebral angiogram could be obtained subsequently. After 5 days of ventilatory support, the patient was ex- tubated and she expired. Family members refused an autopsy study.
DISCUSSION
Dandy- Walker malformations, though the exact etiology is not known, are believed to result from insults to the developing nervous system by genetic or environmental factors. An increased in- cidence of DWS was associated with warfarin or isotretinoin6 use during pregnancy. Although DWS is almost always a pediatric anomaly, cases of adults with DWS have been reported in the litera- ture in recent years. These may be asymptomatic or symptomatic with manifestations of raised in- tracranial pressure and cerebellar or brain stem dysfunction. In the present case, the patient was asymptomatic until 53 years of age, when she suf- fered a CVA, which may or may not have been related to DWS. The cause for altered mental sta- tus change at the time of this event could have been due to raised intracranial pressure secondary to DWS.
Unsgaard et al reviewed the cases reported in the literature.7 Only three cases were reported in the literature between 1987 and 1996. The Table shows some features of the patients with DWS presenting in adolescent and adult life. The mean age of these 20 patients is 34 years. Fifty percent of these cases were older than 31 years at presenta- tion. Increased intracranial pressure and gait ataxia were the most common clinical findings, account- ing for 62% and 38%, respectively.
The explanation for delayed presentation in certain cases is unclear. In these cases, the small openings present in the cyst membrane are effec- tive or the foramina of Lushka may be patent. In some cases, contrast cisternography or ventricu- lography2' 7 can demonstrate this communication between the cyst and the subarachnoid space. These communications between the Dandy-
32 J la State Med Soc VOL 1 52 January 2000
|
Table Patients Presentins with Dandy-Walker Syndrome Later in Life |
||||
|
S No. |
AUTHOR |
AGE |
SEX |
PRESENTING COMPLAINT |
|
1. |
Sato et al 1 996 |
35 |
F |
headache, gait disturb |
|
2. |
Herbert et al 1995 |
53 |
F |
headache, gait disturb |
|
3. |
Herbert et al 1995 |
50 |
F |
visual loss, diplopia, |
|
4. |
Unsgaard et al 1987 |
69 |
F |
gait disturbance |
|
5. |
Unsgaard et al 1987 |
44 |
M |
brain stem dysfunction |
|
6. |
Cox et al 1979 |
27 |
rebound nystagmus |
|
|
7. |
Upton et al 1979 |
34 |
raised ICP, diplopia |
|
|
8. |
Epstein et al 1975 |
14 |
raised ICP |
|
|
9. |
Epstein et al 1975 |
34 |
raised ICP |
|
|
10. |
Gardner et al 1975 |
72 |
ataxia |
|
|
11. |
Engelhardt et al 1975 |
40 |
ICP |
|
|
12. |
Hubert et al 1974 |
26 |
ICP, ataxia |
|
|
13. |
Agostino et al 1 963 |
23 |
hemiparisis |
|
|
14. |
Whitten et al 1976 |
21 |
ICP, subdural hematoma |
|
|
15. |
Maloney et al 1954 |
13 |
ICP, palsy |
|
|
16. |
Coleman et al 1948 |
17 |
ICP, ataxia |
|
|
17. |
Walker 1 944 |
20 |
ICP |
|
|
18. |
Cohen 1942 |
13 |
ICP, ataxia, diplopia |
|
|
19. |
Sahs 1941 |
16 |
ICP, ataxia |
|
|
20. |
Castrillon 1933 |
59 |
ICP |
Walker cyst and other CSF compartments may ef- fectively mitigate pressure effects of the accumu- lating fluid until late in life. However, in the ma- jority, this communication is valvular.
Diagnosis of DWS is established promptly by present day neuro-imaging. Plain x-ray films of the skull show a high inion and a deep posterior fossa with superior displacement of the torcular herophili. Cerebral angiography reveals an avas- cular area corresponding to the posteriorly-located cyst and hypoplastic posterior inferior cerebellar arteries. Contrast cisternography and ventriculo- graphy are useful in demonstrating the communi- cations of the cyst. Currently, CT and MRI are the diagnostic modalities of choice, and they show a grossly deformed fourth ventricle occupying most of the posterior fossa along with other associated neurological anomalies.
With the advent of newer radiological tech- niques and treatment modalities, the mortality from DWS, especially in children, has decreased considerably from 100% in 1942 to less than 10% in recent years.8
Fluid-diversion procedures and cyst excision
have both been reported to be effective. It is inter- esting to note the effective absorption of the fluid that follows marsupialization of the cyst. This in- directly demonstrates that CSF absorptive path- ways and mechanisms remain intact in a DW anomaly. With effective absorption and periodic efflux of cyst fluid through the pores in the mem- brane, it is theoretically possible to have a latent DW cyst until late in life. The term asymptomatic may not be true in these cases, since some form of neurological presentation might have possibly gone unrecorded, as is the case in our patient. In some instances, DW cyst was diagnosed by CT incidentally or after minor head trauma.9'12 In a few of these cases, the symptoms were subtle, prob- ably because of effective drainage of the cyst which kept the pressure below the critical level.
REFERENCES
1. Benda CE. The Dandv-Walker Syndrome or the so called atresia of the foramen Magendie. J Neurcrpathol Exp Neurol 1954;13:14-29.
2. Sato K, Kubota T, Nakamura Y. Adult onset of the Dandy- Walker syndrome. Br J Neurosurg 1996;10:109-112.
3. Hubbert CH, Faris AA, Martinez AJ. Dandy-Walker
J La State Med Soc VOL 152 January 2000 33
syndrome: spectrum of congenital anomalies. South Med J 1974;67:274-277.
4. Gardner E, O'Rahilly R, Prolo D. The Dandy- Walker and Arnold-Chiari malformations: clinical, developmental and teratological considerations. Arch Neurol 1975;32:393-407.
5. Cornford E, Twining P. The Dandy-Walker syndrome: the value of antenatal diagnosis. Clin Radiol 1992;45:172- 174.
6. Kaplan LC, Anderson GG, Ring BA. Congenital hydrocephalus and Dandy-Walker malformations associated with warfarin use during pregnancy. Birth Defects: Original Article Series 1982;18:79-83.
7. Unsgaard G, Sand T, Stovring J, et al. Adult manifestation of the Dandy-Walker syndrome. Report of two cases with review of the literature. Neuro-Chirurgie 1987;30:21-24.
8. Asai A, Hoffman HJ, Hendrick EB, et al. Dandy- Walker syndrome: experience at the Hospital for Sick Children, Toronto. Pediatr Neurosurg 1989;15:66-73.
9. Dandy WE. The diagnosis and treatment of hydrocephalus due to occlusions of the foramina of Magendie and Luschka. Surg Gynecol Obstet 1921;32:112- 124.
10. Lipton HL, Preziosi TJ, Moses H. Adult onset of the
Dandy-Walker syndrome. Arch Neurol 1978;35:672-674.
11. Masdeu JC, Dobben GD, Azar-Kia B. Dandy- Walker syndrome studied by computed tomography and pneumoencephalography. Radiology 1983;147:109-114.
12. Stovall JM, Venkatesh R. Magnetic resonance imaging of an adult with the Dandy-Walker syndrome. J Natl Med Ass 1988;80:1241-3, 1246-1247.
Dr Reddy is a resident in the Department of Internal Medicine at Louisiana State University Health Services Center in Shreveport, Louisiana.
Dr Vannemreddy is Research Fellow in the Department of Neurosurgery at Louisiana State University Health Services Center
in Shreveport, Louisiana.
Dr Grier is Assistant Professor in the Department of Internal Medicine at Louisiana State University Health Services Center
in Shreveport, Louisiana,
Dr Nanda is Associate Professor and Chairman of the Department of Neurosurgery at Louisiana State Health Services Center
in Shreveport, Louisiana.
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34 J La State Med Soc VOL 152 January 2000
LAFAYETTE’S FAMILY PRACTICE RESIDENCY PROGRAM:
PRACTICE PATTERNS OF GRADUATES
Kim Edward LeBlanc, MD, PhD; Glenn N. Jones, PhD
The Lafayette Family Practice Residency Pro- gram has graduated 51 physicians since 1989. This review of the residency program was undertaken as a follow-up to our previous survey which was done 5 years ago. The initial survey was performed to support our assumption that graduates establish practices in communities near their residency programs.
In addition, this survey attempted to deter- mine graduate satisfaction and practice character- istics. The vast majority (92%) of the respondents are practicing in Louisiana at the time of this sur- vey. Sixty percent (60%) are practicing in commu- nities with populations of 25, 000 or less. This rep- resents a substantial improvement from the 1994 survey. In addition, the results suggest that these physicians are satisfied in their careers as family
physicians which concurs with the previous sur- vey.
The main purpose of this investigation was to determine if our residency is continuing to meet its primary goal, ie, to train family physicians pre- pared to treat families in communities of all sizes (with particular emphasis for the smaller, more needy communities). In addition, we could learn if we are also continuing to train family physicians who would stay and practice in south central and southwestern Louisiana in the region known as Acadiana. Lastly, we could determine the satisfac- tion of our graduates in their chosen field.
This most recent data collection, when com- pared to our previous survey which was per- formed in 1994, would serve as an indicator of the adequacy of the residency experience.1 It was
J La State Med Soc VOL 152 January 2000 3^
hoped that our residency has continued to provide adequately trained family physicians for the sur- rounding communities. Moreover, it is anticipated that a majority of these physicians would practice in rural communities in the Acadiana region of the state.
PHASE 1: PRACTICE LOCATION Methods
A total of 51 physicians have completed the Lafayette Family Practice Residency Program from 1989 to July 1998. All of these 51 graduates were mailed the Phase 2 survey (described below). It requested that each physician confirm their ad- dress and indicate the population of the town or city where they practiced. Fifty-one percent (51%) of the graduates responded to the survey. Twenty- five completed the survey, while two surveys were returned as undeliverable.
Results
Similar to our 1994 survey, the vast majority of the respondents practice in Louisiana (92%), while 8% practice out of state. The 1994 survey indicated that 88% practiced in Louisiana, with the remainder practicing in southern states. The gradu- ates of the Lafayette Family Practice Residency Program are serving communities of various sizes. Sixty percent are practicing in communities of 25,000 or less. This is an improvement over the 1994 survey which revealed that only 39.5% of the
|
Table 1 Which Best Describes the Size of the Community Where You Practice? |
||
|
COMMUNITY POPULATION |
1994 |
1999 |
|
< 2500 |
5.5% |
8.0% |
|
2500 - 10,000 |
17% |
52% |
|
25,000 - 50,000 |
11% |
16% |
|
50,000 - 100,000 |
17% |
16% |
|
> 500,000 |
17% |
16% |
|
City > 500,000 |
11% |
4% |
|
Suburban, metropolitan area of > 500,000 |
5.5% |
4% |
|
Does not equal 100% as not all respondents responded to this question. |
graduates were practicing in communities of simi- lar population. Ninety-two percent of the respon- dents are practicing in communities with popula- tions of less than 100,000. This, likewise, is an im- provement since the previous survey which had 67.5% practicing in this size community. Almost all of these communities are in the Acadiana re- gion.
Discussion
As indicated from the above results, the pro- gram is accomplishing its goal of providing fam- ily physicians for Louisiana communities. Further- more, it would appear that this direction has con- tinued from 1994 and has shown improvement as we would hope.
PHASE 2: SURVEY
Methods
In early 1999, the graduates were surveyed to determine the characteristics of their practice. This survey was very similar to the questionnaire that was administered in 1994. The survey consisted of a series of multiple choice questions, with blanks for written-in responses used liberally. Questions were asked about practice location, practice pat- terns, and satisfaction with practice.
Results
Of the 51 surveys sent out, 25 (51%) were re- turned. As stated above, a significant majority (60%) are practicing in communities with popula- tions of 25,000 or less. Table 1 provides compari- sons from the previous 1994 survey to the present 1999 survey.
The overwhelming majority of those who re- sponded are board certified in family practice (24/ 25, 96%). This represents an improvement as pre- vious respondents indicated a 89% certification rate. Eighty-four percent (21/25) indicated that they planned to maintain their certification while four did not respond to this question. In 1994, 89% indicated that they would maintain their certifica- tion. All of the respondents (100%) indicated that they were practicing as family physicians. This represented an increase from 89% reported in 1994.
Practice Arrangements
Eight percent characterized their practice as fee-for-service which is an increase from 61% in
36 J La State Med Soc VOL 1 52 January 2000
the previous survey. Two (8%) indicated that their practices were essentially in health maintenance organizations compared to 11% in 1994. One indi- cated employment with a public community clinic while one other was employed primarily in an emergency room setting. Eighty-four percent in- dicated that they had a contract with a preferred provider organization or an independent practice association. This was a substantial increase from 67% noted 5 years ago.
Similar to the study survey of 1994, there are a wide variety of practice arrangements as reflected in the particular sources of reimbursement. The breakdown and comparison of the two surveys are listed in Table 2. The largest group was represented by private insurance which accounted for 33% and 45% of reimbursement in 1994 and 1999, respec- tively. Medicaid counted for 24% in 1994, yet had declined to 12% in the latest analysis. Medicare reimbursement remained steady at 19% in both surveys. Prepaid contracts, represented by capitated at-risk contracts or health maintenance organizations, declined from 16% in 1994 to nearly 10% in 1999. Those making up the indigent popu- lation or those with partial payment increased slightly between the two survey years from 10% to 12%. Similar to the previous survey, these aver- ages fail to capture the wide range of payment ar- rangements. Virtually all of the physicians re- ported that their income came from three or more sources.
A high percentage of the respondents (84%) indicated that they had a contract with a preferred provider organization or an independent practice association. The remaining 16% did not have such an affiliation. This information was not indicated in the 1994 study.
The patient volume from 1994 to 1999 has seen few changes as noted from a comparison of the data. In both years of the surveys, the vast major- ity of patient encounters were in the physician's office (eg, 115 and 113 per week, respectively). Hospital visits declined somewhat with the phy- sician encounters numbering 15 per week in 1994 and 10 per week in 1999. While 39% of the gradu- ates reported following patients in nursing homes in 1994, 24 of 25 (96%) reported following this type of patients in 1999. The median number of nurs- ing home encounters was 2 per week in 1994 with a mean of 3 per week in 1999. Additionally, 12%
Table 2
Approximately What Percentage of Your Patients Pay for Your Service in the Following Ways?
|
Payment Method |
1994 |
1999 |
|
Medicare |
19.2±15.4 |
19.2±9.6 |
|
Medicaid |
23.9±25.2 |
12.6±14.9 |
|
Indigent or Private Pay |
10.3±16.6 |
1 2.6±1 3.6 |
|
Private Insurance (including PPO, IPA) |
32.5±23.3 |
44.8±24.3 |
|
Prepaid |
15.6±29.2 |
9.8±16.3 |
(capitated at-risk contract, HMO)
The numbers indicated represent percentase means plus/ minus standard deviation.
(3/25) in 1999 indicated that they are serving as medical director of a nursing home facility. House calls have remained a rare event from these two surveys. However, a few physicians did report an occasional house call (1994 average 0.3 per week, 1999 average 0.5 per week). There was consider- able parallel concerning the pattern of managing after-hours calls which ranged widely in both sur- veys. In 1994, 24% reported having no call respon- sibilities; this number was halved in 1999 at 12% with no call. Seven percent reported call every fifth night in 1994 while 20% reported a similar call schedule in 1999. The most frequent call schedule in both years was call every 3-4 nights with 36% in 1994 and 40% in 1999 indicating such an arrange- ment. Only one reported call every night or every other night in 1994 while this was reported by 28% (7/25) in 1999.
Hospital Privileges
In both surveys, graduates were asked about a selection of privileges. A comparison of the data summaries is noted in Table 3. In 1994, 89% of the respondents felt that their hospital privileges were "about right". This trend continues as 96% indi- cated a similar sentiment in the most recent sur- vey.
The vast majority have privileges for adult patients for routine admissions (83% in 1994, 92% in 1999), critical care (78% in 1994, 79% in 1999), and pediatric routine admissions (78% in 1994, 88% in 1999). Forty-four (44%) indicated privileges in
J La State Med Soc VOL 152 January 2000 37
Table 3
Do You Have Hospital Privileges for Patients Classified in the Following Manner?
|
Yes |
1994 No |
Yes |
1995 No |
|
|
Adult Medicine (routine admissions) |
83 |
17 |
92 |
8 |
|
Adult critical care (ICU, CCU) |
78 |
22 |
79 |
21 |
|
Pediatrics (routine admissions) |
78 |
22 |
88 |
12 |
|
Pediatrics critical care (ICU) |
44 |
56 |
54 |
46 |
|
Routine OB care |
22 |
78 |
8 |
92 |
|
High risk OB care |
17 |
83 |
8 |
92 |
|
Caesarean section |
0 |
100 |
4 |
96 |
|
Forceps or vacuum deliveries |
11 |
89 |
8 |
92 |
|
Oxytocin induction |
17 |
83 |
8 |
92 |
|
Postpartum tubal ligation |
0 |
100 |
4 |
96 |
|
Surgery, first assistant |
44 |
56 |
33 |
67 |
|
Primary surgeon appendectomy |
0 |
100 |
0 |
100 |
|
Esophagogastroduodenoscopy |
17 |
83 |
45 |
55 |
|
Colonoscopy |
28 |
72 |
20 |
80 |
All numbers represent percentages.
pediatric intensive care in 1994, while this in- creased slightly in 1999 to 54%. Privileges for first surgical assistant declined from 44% in 1994 to 33% in 1999.
The privilege rate for routine obstetrical care revealed a rather sharp decline between the two surveys. While 22% held such privileges in 1994, by 1999 this figure has dramatically declined to only 8%. A similar decline was noted in the care of high-risk obstetrical patients, although the decline was not quite as dramatic (17% and 8%). The only increase referable to obstetrical care is relative to privileges for Caesarean section and post-partal tubal ligation. In 1994, there were no graduates who had attained such privileges, yet in 1999, one respondent has done so representing 4% of the total. While only two physicians indicated the on- going acceptance of new OB patients in the latest survey, this represents a decline of one from the previous survey in which three physicians indi- cated this offering. This is consistent with findings from the 1994 survey in which one of the three physicians performing OB indicated that there were plans to discontinue this practice.
Patient Characteristics
A number of questions about the kinds of pa- tients served by our graduates were included in the survey. Graduates were asked to estimate the percentage of their patients in a variety of age groups. In a complete parallel to the first study, the vast majority of the respondents indicated serv- ing patients of every age group. In a comparison between the groups, the characteristics did not change very much from one survey to the next. Patients between the ages of 18 and 65 made up 40% of the patient population in 1994, while in 1999 this same age group represented 44% of the total. Patients over 65 years old constituted approxi- mately 25% of the 1994 survey while this was 21% of the 1999 survey. In addition, patients less than 12 years old made up 21% and 22% of the 1994 and 1999 survey, respectively. The age group be- tween 12 and 18 constituted 14% of the practice in 1994 and 13% in 1999.
Both questionnaires asked about the care of patients with human immunodeficiency virus (HIV) infection. In 1994, about one third of the physicians reported managing asymptomatic pa-
38 J La State Med Soc VOL 1 52 January 2000
tients with HIV infection, while in 1999 only 16% indicated caring for this type of patient. Only 2 (11%) indicated that they managed symptomatic patients with HIV infection in 1994. However, the number of 1999 respondents indicating that they were managing the symptomatic HIV patient was zero.
Graduates were also asked to estimate the percentage of their patients who fell into various minority groups. Here, the estimates ranged widely from physician to physician in both sur- veys. Some reported no minorities in their prac- tice. Others reported a practice almost completely made up of one or the other ethnic group. African Americans made up over 30% of the patients. The findings were consistent from the previous study to the present. The "average" practice in 1994 would have 5% Hispanic, 39% African- American, 3% Asian patients with the majority of the remain- der white. The "average" practice in 1999 would have 3% Hispanic, 33% African-American, 1% Asian with nearly the entire remainder (62%) made up of white patients.
Personal Satisfaction with Practice
Table 4 presents the graduates' ratings of sat- isfaction with their career on a scale of 1 (very dis- satisfied) to 5 (very satisfied). On the average, the respondents reported being satisfied with their choice of career in medicine in both survey years with a mean of 4.3 in 1994 and a mean of 4.36 in
Table 4
Satisfaction in Choice of Career
|
1994 |
1999 |
|
|
Choice of medicine as a career |
4.3±0.69 |
4.36±0.95 |
|
Choice of family practice as career |
4.3±0.59 |
4.36±0.99 |
|
Integration of career and other life goals |
3.7±0.91 |
4.00±0.96 |
|
Current practice arrangements |
3.9±0.90 |
4.00±1 .08 |
All numbers represent means plus/minus standard deviation.
These questions utilize Likert scale ratinss from 1 (very dissatisfied) to 5 (very satisfied).
1999. In addition, the indication was that the gradu- ates were satisfied with their choice of family prac- tice as a specialty with similar numbers as before (4.3 in 1994 and 4.36 in 1999). None reported being dissatisfied (rating of 1 or 2) with medicine or fam- ily practice as a career in either survey.
On the average, the graduates were also sat- isfied with the integration of career with other life pursuits in both surveys. In fact, the level of satis- faction has increased from a mean of 3.7 in 1994 to a mean of 4.0 in 1999. This trend was also noted in satisfaction with current practice arrangements (mean 3.9 in 1994, 4.0 in 1999). The vast majority (89% in 1994, 88% in 1999) reported that their sat- isfaction with their choice of medical career has remained about the same or increased. Only two (11%) in 1994 and three (12%) in 1999 reported that their satisfaction has declined.
DISCUSSION
The results of the first study performed in 1994 suggested that the Lafayette Family Practice Resi- dency Program was meeting its goals of training family physicians to practice in Louisiana commu- nities of all sizes. Subsequent to that study, it was the feeling of this residency program that we were in fact performing better than this stated objective. It was hoped that this residency program was pref- erentially preparing family physicians to practice in the more needy areas of our state, ie, those with smaller communities with fewer physicians.
This second 5-year follow-up study was un- dertaken to complement our previous work and to identify the trends in our graduates. It does ap- pear from the results of this 1999 survey that we are preparing family physicians for smaller com- munities. The vast majority (92%) of our gradu- ates are not only practicing in Louisiana as family physicians, sixty percent (60%) are practicing in communities that have populations of 25,000 or less. This 60% represents a substantial improve- ment from 1994 in which this percentage was only 33% of the respondents. (This is also considerably better than the 28.8% figure that is indicated by the 1993 American Academy of Family Physicians Computer Usage and Community Information and Current Fees.) Our residency program is accom- plishing one of its goals.
Few other changes were uncovered from the previous survey. Similar to the 1994 survey.
J La State Med Soc VOL 152 January 2000 39
the majority of reimbursements derive from tradi- tional fee-for-service arrangements (private pay. Medicare /Medicaid), but many derive income from various sources. It is interesting to note that private insurance patients represent an increase of 12 percentage points from 1994 to 1999 (33% to 45%). This increase was equal to the decline in patients with Medicaid (24% in 1994 and 12% in 1999). The reasons for this occurrence is not clear at this time and could not be determined by the available data.
As indicated in both surveys, it is demon- strated that hospital privileges are "about right" and are remaining stable. An encouraging trend is the marked increase in privileges for esophagogastroduodenoscopy (17% in 1994 and 45% in 1999). One rather disappointing trend that was realized in the comparison of the two surveys is the decline in obstetrical practice among our graduates. This tendency certainly is a multifacto- rial problem which should be addressed. Family physicians are trained to administer care to the entire family. This should include obstetrical care at some level.
It does appear from both surveys that the re- spondents are caring for patients throughout the age spectrum. This does represent the intent of the family physician and as such this goal has been attained.
The graduates of our program continue to express satisfaction with their careers as family physicians. One aspect of the surveys that deserves emphasis is that even though the majority of the graduates are in smaller communities, the after- hours call appears to be manageable in both 1994 and 1999. This would suggest that conditions in smaller communities with call arrangements lend themselves to satisfactory integration of career and family. In addition, the vast majority are either satisfied or very satisfied in both surveys with very similar numbers. No one expressed dissatisfaction with his or her choice in medicine in general or family practice in particular. This was a consistent finding in 1994 and 1999. Similar to the 1994 sur- vey, the few that expressed dissatisfactions seemed to be referring to particular practice arrangements or challenges integrating their careers and personal lives. The reported data certainly seemed to indi- cate that after-hour call arrangements were quite satisfactory. Our feeling has not changed since 1999
that satisfaction is very important in retaining phy- sicians in their chosen careers. The satisfaction ex- pressed by our graduates in both 1994 and 1999 makes us very optimistic about the retention of these family physicians in the kinds of practices and locales where they are needed, ie, south Loui- siana communities in general and smaller commu- nities in particular.
The findings from these two surveys engen- der a feeling of accomplishment within our resi- dency program and will serve to inspire our con- tinued efforts to supply Louisiana communities with much needed family physicians.
REFERENCES:
1. Jones GN, Rees AC. Lafayette's Family Practice Residency Program: practice patterns of graduates. J La State Med Soc. 1996;148:359-363.
Dr LeBlanc is Resident Program Director of the Department of Family Practice at Louisiana State University Health Services Center
in Lafayette, Louisiana.
Mr Jones is Associate Professor in the Department of Family Medicine at Louisiana State University Health Services Center in New Orleans, Louisiana.
40 J La State Med Soc VOL 1 52 January 2000
QUADRICEPS SPARING MYOPATHY
Timothy J. Dozier, MD; John Kalmar, MD
Magnetic resonance imaging (MRI) has been proven to be a useful tool in the evaluation of myopathy. Myopathic changes secondary to processes such as inflammatory disease, neuropathy, and neuromuscu- lar disorders often involve several muscle groups. We describe a unique case of lower extremity myopa- thy with sparing of the quadriceps muscle group on MRI evaluation.
The use of magnetic resonance imaging (MRI) in the evaluation of soft tissue abnormali- ties, including the evaluation of myopathy, is widespread. The ability of MRI to delineate and evaluate myopathic changes in muscle groups is excellent. Myopathic changes affecting skeletal muscle have many different etiologies, including inflammatory disease, granulomatous disease, and neuromuscular disorders. The pattern of myo- pathic change often involves several muscle groups, and in this case MRI is used to delineate a unique pattern of myopathic change affecting the muscle groups of the lower extremity.
CASE REPORT
A 45-year-old man complained of a 4- to 5- year history of progressive lower extremity muscle weakness. On initial presentation, he demon-
strated weakness in the iliopsoas muscle groups bilaterally (+1 / 5) and the hamstring muscle groups bilaterally (+2/5). No weakness was detected in the quadriceps muscle group bilaterally (+5/5) or in his upper extremities. His past medical history was significant for sarcoid, asthma, hypertension, and diabetes.
Electromyographic findings indicated poly- myositis. Muscle biopsy demonstrated areas of dense endomysial inflammation with associated myopathic changes. These findings suggested an inflammatory myopathy, and the dense focal na- ture of the inflammation suggested a granuloma- tous myositis such as sarcoidosis. MRI of the lower extremities revealed myopathic changes in several muscle groups. Although radiological findings were consistent with a generalized myopathy of the lower extremities, the quadriceps muscle
J La State Med Soc VOL 1 52 January 2000 4 1
groups were spared bilaterally. Figure la (T1 axial) and lb (coronal short tau inversion recovery) dem- onstrate diffuse fatty replacement and atrophy in- volving several muscle groups of the lower ex- tremities; however, the quadriceps and gracilis muscle groups are spared bilaterally. The imag- ing findings of inflammatory myopathy with quad- riceps and gracilis sparing is an interesting clini- cal and radiographic finding.
DISCUSSION
The only references to quadriceps sparing myopathy in our literature search are related to a familial and hereditary inclusion body myopathy, predominately seen in the Iranian Jewish popula- tion.1"3 These patients demonstrated rimmed vacu- oles on muscle biopsy. Our patient did not fit into this category.
We do not have a definitive pathological di- agnosis for our case; however, it is interesting nonetheless from an imaging standpoint. Quadri- ceps sparing myopathy is an extremely rare find- ing and, as previously mentioned, only described in relation to inclusion body myopathy, which this patient did not demonstrate on biopsy. Several potential causes of our patient's myopathy include sarcoid myopathy, polymyositis, diabetic neuropa- thy, neuromuscular disorders, or a combination of these disease entities.
REFERENCES
1. Argov Z, Yarom R. "Rimmed vacuole myopathy" sparing the quadriceps. A unique disorder in Iranian Jews. / Neurol Sci 1984;64:33-43.
2. Sadeh M, Gadoth N, Hadar H, et al. Vacuolar myopathy sparing the quadriceps. Brain 1993;116:217-232.
3. Sivakumar K, Dalakas MC. The spectrum of familial inclusion body myopathies in 13 families and a description of a quadriceps-sparing phenotype in non- Iranian Jews. Neurology 1996;47:977-984.
Figure la. T1 axial image demonstrates diffuse fatty replacement and atrophy involving several muscle groups of the lower extremities with sparing of the quadriceps and gracilis muscle groups bilaterally.
Figure 1b. Coronal short tau inversion recovery demonstrates diffuse fatty replacement and atrophy involving several muscle groups of the lower extremities with sparing of the quadriceps and gracilis muscle groups bilaterally.
Dr Dozier is a resident in the Department of Radiology of Ochsner Clinic and Alton Ochsner Medical Foundation in New Orleans, Louisiana.
Dr Kalmar is a radiologist with Ochsner Clinic and Alton Ochsner Medical Foundation in New Orleans, Louisiana.
42 J La State Med Soc VOL 152 January 2000
CALENDAi
FEBRUARY 2000
6- 8 Symposium on E-Healthcare Strategies for
Physicians, Hospitals & Integrated Delivery Systems
Scottsdale, AZ. Contact: Linda Jenkins, coordinator; phone: (760) 771-5102; fax: (760) 771-3183; e-mail: lindaihi@aol.com.
7- 9 11th Annual Rural Health Policy Institute
Washington D.C. Contact: Elizabeth Briggs, National Rural Health Association, Government Affairs; phone: (202) 232- 6200; e-mail: briggs@NRHArural.org; Internet: www.NRHArural.org.
18-19 6th Annual Patrick Hanley Colorectal Surgery Symposium
New Orleans, LA. Contact: Brandi Orgeron, Alton Ochsner Medical Foundation; phone: (800) 778-9353; fax (504) 842-4805; e- mail: borgeron@ochsner.org
20-25 Advances in Imaging: 2000
Breckenridge, CO. Contact: Shirley K. Cospolich, Tulane University Medical Center, Center for Continuing Education TB5 1 , 1 430 Tulane Ave., New Orleans, A 701 1 2-2699; phone: (504) 588-5466, (800) 588-5300; fax: (504) 584-1779.
25-27 Prevention of Violence: The Role of the Physician
Baton Rouge, A. Contact: Pamela Schmidt, Tulane University Medical Center, Center for Continuing Education TB51, 1430 Tulane Ave., New Orleans, A 701 1 2; phone: (504) 588-5466, (800) 588-5300; fax: (504)584- 1779.
MARCH 2000
4-6 15th Annual Mardi Gras Anesthesia Update
New Orleans, A. Contact: Judy Lua
Esporotu, Tulane University School of Medicine, 1430 Tulane Ave., New Orleans, A 70112; phone: (800) 588-5300, (504) 588-5466; e-mail: cme@tulane.edu.
14-17 12th National HIV/AIDS Update Conference: HIV/AIDS at the Crossroads- Confronting Critical Issues
San Francisco, CA. Contact: American Foundation for AIDS Research (amfAR); Internet: www.amfar.org/nauc.
29-2 2000 International Conference on Physi-
cian Health: “Recapturing the Soul of Medicine"
Charleston, SC. Contact: Roger Brown, PhD, AMA Science and Public Health Advocacy Programs, phone: (800) 621-8335, (312) 464-5066; fax: (312) 464-5841.
April 2000
10-14 29th Family Practice Update
New Orleans, LA. Contact: Kathleen Melancon, Louisiana State University School of Medicine Institute of Professional Education; phone: (504) 568-5272; e-mail: cme@lsumc.edu.
27-29 10th Annual Endocrinology Update
New Orleans, A. Contact: Jocelyn Arnold, Alton Ochsner Medical Foundation; e-mail: jarnold@ochsner.org; phone: (504) 842- 3702.
29-30 Annual Tri-State Anesthesiology Conference
New Orleans, A. Contact: Jocelyn Arnold, Alton Ochsner Medical Foundation; e-mail: jarnold@ochsner.org; phone: (504) 842- 3702.
«
J La State Med Soe VOL 1 52 January 2000 43
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Journal of the Louisiana State Medical Society Capsules
You receive a copy of each of these publications monthly. The Journal is one of the oldest established publications of its kind in the country. It features scientific information on clinical trials and treatments, including several special issues each year, highlighting such topics as Heart Disease, Cancer in Louisiana, Sports Medicine and Mental Illness. In addition, the March issue of the Journal is supplemented by the LSMS Annual report, including highlights of the Annual Meeting of the House of Delegates. Capsules is the monthly newsletter of the LSMS, featuring timely articles on local and national issues affecting organized medicine and physician practices, as well as member accomplishments and regular columns.
As a member of the Louisiana State Medical Society , one of the many benefits you receive is a number of important and informative publications, designed to keep you up-to-date on the most current information available on issues and concerns of physicians. Here is just a sample of our regular publications.
The annual LSMS Membership and Resource Directory
Your name is listed in this widely-recognized premier publication used by more than 7,000 physicians, clinics, hospitals and other healthcare and business organizations around the state. In addition to membership listings, this 323-page directory contains extensive resource information that makes it a must-have item referred to throughout the year.
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Throughout the year, you receive a number of other special communications pieces. These include Special Bulletins to inform LSMS members about current issues of concern to medicine, such as reimbursement and fraud and abuse issues. In addition, the LSMS relays up-to-the-minute bulletins and Calls to Action during the Legislative Session and other pertinent times during the course of the year, via the LSMS website, www.lsms.org, or the use of blast fax and e-mails. These are designed to keep members informed and involved in the issues that directly affect them and the well-being of their patients.
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Elizabeth T.H. Fontham, DrPH |
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PUBLIC HEALTH IN LOUISIANA: AN INTRODUCTION |
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Trade M. Bellanger, BA Erica M. Ceasar, MSPH Louis Trachtman, MD, MPH |
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BLOOD MERCURY LEVELS AND FISH CONSUMPTION IN LOUISIANA |
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Louis Trachtman, MD, MPH Bobby Savoie, MPH |
74 |
ARE YOU SANITARY? |
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Kim B. Overstreet, MA |
78 |
PUBLIC HEALTH EDUCATION OPPORTUNITIES FOR PHYSICIANS IN LOUISIANA |
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LOUISIANA PARISH HEALTH PROFILES 1999: USING INFORMATION TO DRIVE LOCAL ACTION |
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INFORMATION FOR AUTHORS |
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PRESIDENT’S MESSAGE 2000 Legislative Session |
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LSMS ALLIANCE LSMSA Website Bursts Onto the Scene |
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Information for Authors
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Citing a reference entry. Use superior arabic numerals placed at the logical site in the sentence; insert immediately after a word or mark of punc- tuation; set close. Cite in the main text, in tables, and in the legends for illustrations; do not cite in the abstract.
Parts of the Manuscript
Title page. The title page should carry the following information: (1) The title of the manuscript, which should be concise but informative. (2) The full name of each author together with his highest academic degree relevant to the subject matter of the paper. List authors in the order of the magnitude of their contribution. List as authors only those who have contributed mate- rially to the conduct of the study or to the preparation of the manuscript. (3) The affiliation of each author at the time die study was done. (4) Explanatory notes that give (a) a brief biographical note for each author indicating his academic appointments, hospital affiliations, and practice location; and (b) the name and address of the author to whom requests for reprints should be addressed or a statement that reprints will not be available.
Abstract and Keywords. Give a brief recapitulation of the purpose of the paper, the methods and subjects used, the results, and die conclusions; avoid acronyms and abbreviations, do not cite sources listed in the references section (the abstract must stand alone); limit the abstract to 150 words.
On the lower part of the same page, list three to five key words or short phrases that will assist indexers. Use terms from Medical Subject Headings as used in Index Medicus when possible.
Main Text. Avoid highly technical expressions and jargon; the article should be easily understood by the general readership.
Use subheads freely to break the typographic monotony, make the paper easier to read, and fortify the sequence of the author’s argument. Commonly used subheads are: introduction or background, methods and subjects, re- sults, discussion, and conclusions.
Acknowledgments. Acknowledgments must be made for financial assis- tance (grants, equipment, drugs) and for the use of previously published ma- terial. Acknowledgment may be made for technical assistance and intellectual participation in conducting the study or preparing the manuscript.
References. Each source cited in the main text, tables, or legends must be listed in the references section; and, conversely, all entries in the references section must have been cited in the main text, tables, or legends.
Each reference entry is composed of three elements: (1) the name of rite author, (2) the title of the article or book, and (3) die imprint. The following
three examples illustrate the reference style adopted by the Journal for ( 1 i a reference to an article in a journal, (2) a reference to a book or monograph, and (3) a reference to a part of a larger work.
1. Brush JE Jr, Cannon RO III, Schenke WH, et al. Angina due to coro- nary microvascular disease in hypertensive patients without left ven- tricular hypertrophy. N Engl J Med 1988;319:1302-1307.
2. Hajdu SI. Pathology of Soft Tissue Tumors. Philadelphia, Pa: Lea & Febiger; 1979:60-83.
3. Robinson BH. Lactic acidemia. In: Scriver CR, Beaudet AL, Sly WS, et al (editors). The Metabolic Basis of Inherited Disease , 6 th edition. New York: McGraw-Hill; 1989:869-888.
Type each reference entry as a separate hanging paragraph; number the en- tries consecutively in the order cited; do not list alphabetically; double-space reference entries; punctuate as shown in the examples above. Limit refer- ences to 15 unless special arrangements have been made with the editors.
Tables. Tables should be self-explanatory and supplement, not duplicate, the main text. All tables should have been referred to in the main text.
Type each table on a separate page; number tables in the order first cited; provide a title; consult recent issues of the Journal for examples.
Legends. Legends identify and describe the illustrations. A legend con- sists of a figure number, a description of the figure, an explanation of an- notations on the figure, the techniques used, and an acknowledgment of the source if the figure has been previously published.
Type legends together on a separate page; use block paragraphs. Number the figures in the order first cited in the text.
Illustrations
All illustrations should be referred to in the text. An illustration and its legend must stand alone. Illustrations should be professionally prepared. Four-color illustrations are acceptable at the author’s expense.
Cover Letter
The manuscript must be accompanied by a cover letter which (1) requests consideration of the paper for publication in the Journal ; (2) states that the paper has not been published previously and is not currently being considered by another journal; (3) acknowledges any potential conflict of interest; (4) states that the final manuscript has been seen by all of the authors; and (5) designates one of the authors as corresponding author and lists his full mailing address, telephone number, and fax number.
Permissions
Written permission must be obtained from (1) any individual w-ho is recog- nizable in text or illustration, (2) the copyright owner of any previously pub- lished matter (text, table, or figure) which is to be incorporated in the manu- script, and (3) any individual mentioned in the acknowledgments.
Copyright Transfer
Authors will be asked to sign a form transferring to the Journal copyright ownership of any article accepted for publication. Such articles may not be published elsewhere, in witole or in part, without written permission from the editors.
Galley Proofs
Galley proofs will be mailed to the corresponding author for review.
Editorial Assistance
An expanded version of Information for Authors is published in die January and July issues. For help in preparing your manuscript or for questions about the editorial process, write the Editor or die Managing Editor as below. Or, contact the Editor, Dr Magee, at (337) 439-8450, FAX (337) 439-7576; e- mail: conwaystonemagee@compuserve.com; or the Managing Editor, Anne Shirley at (225) 763-8500, FAX (225) 7 63-2332, or e-mail: publicaffairs@lsms.org.
Submission of the Manuscript
Submit the manuscript (in triplicate), the illustrations (two copies each), die required permissions, and a cover letter to:
Editor, Journal of the Louisiana State Medical Socict v 6767 Perkins Rd.
Baton Rouge, LA 70808
50 J La State Med Soc VOL 152 February 2000
President's Messaae
^eP
As you know, on November 20 of last year, the General Election was held for statewide and legislative races. I am pleased to report that of the 135 candidates the Medical Society supported through LAMPAC, 124 won. The LSMS is very proud of this 92% success rate and is excited about the prospect of working closely with the new legislature.
On April 24, the 2000 Regular Legislative Session will convene for a fiscal only session and must adjourn no later than 6:00 pm on June 7. The constitution provides that, in even- numbered years, the Legislature is restricted to consideration of legislation which provides for enactment of a general appropriations bill, implementation of a capital budget, and consideration of tax matters. However, the Governor may call a special session in which a limited number of items, fiscal or general, may be heard as determined by the Governor. Although a date has not been confirmed. Governor Foster has stated that he intends to call a special session approximately one month prior to the regular session to deal with funding for teacher pay raises.
You have probably heard or read recent reports advising that the 2000-2001 state budget deficit could be as great as $500 million. In late December, Department of Health and Hospitals (DHH) Secretary David Hood testified before the Joint Legislative Committee on the Budget that state government is facing a $153 million shortfall in the current 1999-2000 Medicaid budget. In fact, on December 7, 1999, Governor Foster issued an Executive Order implementing a spending freeze throughout the executive branch of state government to achieve a state general fund savings of at least $50 million for the remainder of the 1999-2000 fiscal year, which ends June 30, 2000. Within the same Executive Order, DHH was ordered to cut $22.4 million from its budget towards the $50 million total savings sought by the Governor.
It is safe to say that the primary focus of the LSMS Department of Governmental Affairs in 2000 will be on the state budget. More specifically, given the dire financial situation and predictions, the LSMS will face a colossal task to simply maintain the present
J La State Med Soc VOL 152 February 2000 51
level of Medicaid reimbursement for private physician services. The administration has publicly proclaimed that its primary goal of the coming legislative session is to find a way to raise classroom teacher pay to the southern average. Given that state income and corporate tax collections appear to be flat, several new or increased taxes have been proposed to fund the pay raises and operating deficit. In addition, there has been some discussion regarding removing some tax exemptions to raise revenue. However, at this time, neither the Governor nor the new leadership of the legislature has issued any concrete proposals to solve the budget deficit. Of course, the fear of the collective health care industry is that the DHH budget will suffer serious cuts or, at a minimum, no increased funding.
In 1995, private physician services were budgeted at $235 million. In 1998, the budget for private physicians' services had been reduced to $193 million. In the current 1999-2000 fiscal year, it is estimated that DHH will expend approximately $211 million for private physician services. Since 1995, the budget for private physician services has suffered a 9% reduction, while the costs of providing health care have steadily increased.
Rural and inner city physicians throughout the state treat a high percentage of Medicaid patients and, thus, rely heavily on reasonable Medicaid payment rates. Many of these physicians face the threat to financial survival and represent the health care safety net for poor patients. Repeated reductions in payment rates will continue to reduce access to health care statewide because fewer physicians are willing, or able, to accept below-cost reimbursement. Medicaid payments, on average, are 52% of private sector reimbursement, and, as such, are below practice overhead.
In the 1999 Regular Legislative Session, the LSMS achieved a great deal of success with its legislative agenda. This success was due in large part to the grassroots involvement of physicians across the state. Once again, the LSMS is calling on its membership to join the advocacy effort to
persuade the Legislature to keep physician reimbursement at a level that will continue to allow access to care for our less fortunate citizens.
Since there is a significant number of new legislators, now is the time to contact your legislators and begin building a relationship that will enable you to more easily communicate during the session. The LSMS strongly encourages you to offer to be a health care resource to as many legislators as possible. It's never too early to develop effective dialogue with your elected officials who will determine the future state of health care in Louisiana.
Prior to, and during, the session, the LSMS will post talking points, calls to action, and weekly updates on our web site. I encourage you to regularly check the site for information that will aid you in your discussions and communications with your legislators. In addition, please remember that you may e-mail your legislator through our Grassroots Action Center found on the LSMS web site.
Remember physician involvement has always been the key to our legislative efforts and success.
52 J La State Med Soc VOL 1 52 February 2000
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Don ’t Miss Out
On These Important Publications !
Journal of the Louisiana State Medical Society Capsules
You receive a copy of each of these publications monthly. The Journal is one of the oldest established publications of its kind in the country. It features scientific information on clinical trials and treatments, including several special issues each year, highlighting such topics as Heart Disease, Cancer in Louisiana, Sports Medicine and Mental Illness. In addition, the March issue of the Journal is supplemented by the LSMS Annual report, including highlights of the Annual Meeting of the House of Delegates. Capsules is the monthly newsletter of the LSMS, featuring timely articles on local and national issues affecting organized medicine and physician practices, as well as member accomplishments and regular columns.
As a member of the Louisiana State Medical Society , one of the many benefits you receive is a number of important and informative publications, designed to keep you up-to-date on the most current information available on issues and concerns of physicians. Here is just a sample of our regular publications.
The annual LSMS Membership and Resource Directory
Your name is listed in this widely-recognized premier publication used by more than 7,000 physicians, clinics, hospitals and other healthcare and business organizations around the state. In addition to membership listings, this 323-page directory contains extensive resource information that makes it a must-have item referred to throughout the year.
Other Special Publications
Throughout the year, you receive a number of other special communications pieces. These include Special Bulletins to inform LSMS members about current issues of concern to medicine, such as reimbursement and fraud and abuse issues. In addition, the LSMS relays up-to-the-minute bulletins and Calls to Action during the Legislative Session and other pertinent times during the course of the year, via the LSMS website, www.lsms.org, or the use of blast fax and e-mails. These are designed to keep members informed and involved in the issues that directly affect them and the well-being of their patients.
LSMS Alliance
LSMSA Website Bursts Onto the Scene
Mrs Karen Depp
With much gratitude to the LSMS staff, and especially Cathy Lewis, the LSMSA now has a state of the art (science?) website that is already drawing positive comment from across the country. We are one of the first state Alliances to have a comprehensive site and we are working to make it interactive and image-delivering! Take a look - you may see yourself right on our page!
As I travel the state visiting with our membership at alliance and auxiliary meetings, it becomes clear that "we" are ready to meet the next century head on and well prepared. More and more of our members are not only computer proficient, but innovative and receptive to using this venue as our primary means of communication. Our on-line pub- lications can be updated in a second rather than months as in the printed media. When we read a notice, it is now timely rather than historical! I am hopeful that each of the individual parishes will use their web page in new and exciting ways that will teach the rest of us some new tricks. Once we have gotten over the hurdle of restricted access (password-keyed site locations) we will be able to publish our
directories on-line and save a tremendous amount of time and cost in the process. And they will always be as current as our most recent information allows!
I see a trend toward re-involvement in our Alliances and Auxiliaries and I am planning to build on this as we continue our re-structuring. It is an interesting observation that many of those things that "we always do" are the very things that have driven away members. When I speak of a new system and structure, I see renewed interest and participation. I know that when the year comes to a close we will look back and feel confident that we have taken the right path into 2000 with our plans and projects. We are always proud and honored to be standing beside the LSMS helping to bring positive change and dynamic leadership to "our" profession.
Mrs Depp is President of the Louisiana State Medical Society Alliance.
J La State Med Soc VOL 152 February 2000 55
ECG 3 1 >i h
Sinister Implications
Jorge I. Martinez-Lopez, MD
A 76-year-old woman was hospitalized following a syncopal episode at home. She was taking oral propranolol for long-standing hypertension. The monitor rhythm strip shown below, taken on her fourth hospital day, consists of three separate leads recorded simultaneously: from top to bottom are leads II, and III.
What is your diagnosis? Elucidation begins on page 57.
56 J La State Med Soc VOL 152 February 2000
ECG of the Month Presentation is on page 56.
DIAGNOSIS - Transient cardiac standstill
Examination of lead II provides the best data with which to interpret the rhythm strip. The first six cardiac cycles in that lead show regu- larly occurring P waves, at a rate of 100 times a minute. P waves in these cycles are inverted (P') and originate from an ectopic focus, either in the low right atrium or in the AV junctional area, with retrograde conduction of the electrical im- pulses into the atria. Every P' wave conducts into the ventricles with a short P'R interval, a finding that suggests close proximity of the ec- topic supraventricular focus to the AV junction. Intraventricular conduction is normal, as de- picted by the narrow QRS complexes. T waves are low and the QT interval is normal. After the sixth cardiac cycle of the tracing, disturbing events occur, which portend sinister implica- tions. First, these events are triggered by an up- right P wave, with a P'-P interval that is much shorter than previous P'-P' intervals. The exact location of the focus responsible for this P wave is problematic: it could represent either a pre- mature sinus impulse or a premature, ectopic atrial impulse.
More worrisome than ascertainment of the location of the atrial focus is the second finding. The seemingly benign, upright, premature P wave effectively shuts down all subsequent car- diac electrical activity, as manifested by the pause that it triggers. The pause occurs abruptly, is unexpected, and lasts about 4.6 seconds, dur- ing which there is no recorded atrial or ventricu- lar activity (cardiac standstill). Eventually, the pause ends with the late appearance of a tiny P wave, which is significantly different in mor- phology and amplitude than all previous P waves. The tiny P wave is very close to the nar- row QRS that follows it; the short PR interval of less than 0.06 second raises the possibility that this P wave is not conducted into the ventricles, but is dissociated from the ventricular complex that follows it. In either case, the late appear-
ance of atrial and ventricular activity signals the resurgence of previously dormant pacemaker activity. A final finding is the marked prolonga- tion of the QT interval (0.48 sec) recorded only in this last cycle.
What are the sinister implications of these ECG findings? Clearly, two major electrical problems are brought into sharp focus by the abrupt appearance of the long pause. One re- lates to the integrity of the SA node. The absence of sinus P waves during the pause suggests that either the SA node has stopped firing tempo- rarily (sinus arrest) or that it continues to fire, but the impulses generated fail to exit the SA node and to reach the atrial musculature (SA nodal exit bloc). There being no basic sinus rhythm present in the tracing, the distinction between these two entities cannot be established with certainty and either one remains a diagnos- tic possibility.
The second and perhaps more serious abnor- mality found in the tracing is the apparent de- pressed electrical activity of subsidiary pacemak- ers in the AV junction and in the ventricular Purkinje fibers. Ordinarily, when the SA node relinquishes its role as the dominant cardiac pacemaker, subsidiary (secondary) pacemakers in the heart assume command and control of the ventricular rate and rhythm. Not so in this case. Subsidiary pacemakers failed to rise to the oc- casion, when the pause was triggered, until a long time had elapsed.
The patient had several similar sequences of ectopic atrial rhythm and abrupt pauses trig- gered by upright premature P waves during her hospital stay. During these brief episodes, she experienced dizziness but no syncope. Although propranolol may have contributed to the appear- ance of these ECG events, it was excluded as the major factor after its administration was stopped. Her hypertension was managed with diuretics and ACE inhibitors. Also excluded as potential causes for the ECG abnormalities were myocar- dial infarction and ischemic heart disease. In the absence of clinically recognizable mechanisms responsible for the symptomatic ECG abnormali- ties, the sequences leading to cardiac standstill
J La State Med Soc VOL 152 February 2000 57
were attributed to SA nodal dysfunction (sick sinus syndrome). A permanent, dual-chamber cardiac pacemaker was implanted.
The choice of therapy for patients with symp- tomatic SA nodal dysfunction is relatively straightforward, provided that a correlation be- tween the arrhythmia and the symptoms is docu- mented. If this correlation does not exist, it is inappropriate to implant permanent cardiac pacemakers in these patients. By the same to- ken, in patients with documented, symptomatic episodes of SA nodal dysfunction, pharmaco- logic treatment has no place in the management of bradyarrhythmia. Drugs which further de- press either SA nodal function or the inherent automaticity of subsidiary pacemakers, or both, must be avoided at all costs. If such drugs have to be used, use with caution and only under the protective umbrella of an implanted cardiac pacemaker.
Dr Martinez-Lopez is a specialist in cardiovascular diseases affiliated with the Cardiology Service, Depart- ment of Medicine, Texas Tech University Health Sciences Center and Thomason General Hospital in El Paso, Texas.
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58 J La State Med Soc VOL 152 February 2000
Th jrnal 1 50 & 1 00 Years Aao
*:&
January 1850 and 1900
Gustavo A. Colon, MD
Interestingly enough, advertisements for medical schools were apparently common place in the 19th Century in contrast to to- day where the applications certainly exceed the number of spots in various medical schools. In the 19th Century, there were many proprietary medical schools all seeking students. What fol- lows is an advertisement or sort of an advertorial so to speak in The Journal for the University of Louisiana's Medical School. As you can see, to induce students, they even reduced the fee of the tickets that each professor charged. Common in those times was not to charge a curriculum fee but rather to charge a specific ticket, very much like going to the theater, by each profes- sor for appropriate admission to the lectures.
"Without seeking to disparage any of the many excellent medical schools scattered throughout the country, we feel called upon to advert in this connection to the advantages, of- fered by this city to the medical student, for ac- quiring a thorough and practical knowledge of his profession. With a faculty thoroughly and permanently organized, with lecture rooms spa- cious enough to accommodate five or six hun- dred students, with cabinets furnished with
models, drawings, wax preparations, morbid specimens, both wet and dry, with a hospital handy by the college hall, at all times accessible to the student and abounding in a great variety of rare and interesting cases of disease, it cannot be denied that New Orleans, with the foregoing advantages, holds out strong inducements to those who aspire to become practical physicians. The perfectly independent position which we occupy in relation to the medical department of the University of Louisiana justifies this free and candid expression of opinion of its high claims to the patronage of the southern student. The lec- tures will commence for the session, 1849-50, on the 12th of November and continue four months. We see from the circular, published by the Uni- versity, that the tickets of each professor, have been reduced to fifteen, and the diploma-fee to thirty dollars."
In the February 1900 issue of The Journal, the most interesting articles are in the communica- tion section of the editorial page. The first one is written to the members of the profession by the editor regarding a Bill that had been brought up in the District of Columbia to prohibit vivisec- tion and to encourage passage of similar bills in
J La State Med Soc VOL 152 February 2000 59
all State Legislatures. It was pointed out that this would seriously interfere not only with sci- entific research but also the experimental work of the Bureau of Animal Industry and the three government services, the Army, Navy, and Marine hospital services, which could no longer do any type of animal experimental work. The author stated that one example of the inesti- mable value of scientific research is to prevent disease, and he gives the example of modern surgery and the antitoxin treatment of diphthe- ria which was discovered through animal re- search. He stated that it was of the utmost im- portance that any physician who read the edi- torial appeal immediately and communicate with the Senators from the State as well as in- voke the aid of the Representatives in order to prevent this Bill from passing.
The second editorial regards the Prosser case. Apparently, Dr S S Prosser, who formed the "ABBO" Clinic in New Orleans and was apparently a notorious self-promoter and ad- vertising physician of the period, had been de- nied a license in the State of Louisiana. He had been found in violation of the medical law by the Parish of Orleans. It states that, several weeks before the meeting of the Board in May 1899, Dr Prosser, a graduate of Jefferson Medi- cal College, applied for and obtained a tempo- rary certificate to practice medicine in Louisi- ana until the next regular meeting of the Board. Dr Prosser then founded the "ABBO" Clinic, apparently much to the chagrin of the local medical committee. On the day that the Board met in session. Dr Prosser did not present him- self until the examination had been in progress for 4 to 5 hours. Therefore, it was impossible for him to stand examination in such a limited time and he was told that his temporary certifi- cate had expired and it would be a violation of law to continue practicing until he obtained an- other certificate. Therefore, he was denied prac- ticing in Louisiana by the United States Court. This injunction was issued very quickly, the day before the Federal Judge left for summer vaca- tion. Subsequently, in November, Dr Prosser was fined for Contempt of Court for disregard-
ing the injunction which had been issued in the summer. Dr Prosser came before the Board but did not obtain a certificate and then a few days afterward he obtained a mandamus to try and force the Board to issue him a certificate to prac- tice, claiming that the Board was biased and prejudiced against him because he advertised in the Daily Press! When these proceedings went to trial, they were continued indefinitely because of the illness of the Judge. However, the follow- ing letter was received from the Attorney of the Board which stated, "Dear Sir: I have the honor to inform you that Dr S S Prosser has left the city in consequence of the proceedings taken up by your Board against him. He has abandoned his suit in the State Court, the proceedings of the United States Court will hereafter be merely informal so as to make perpetual the injunction already issued." The advertisements that fol- lowed of the "ABBO" Medical Institute stated that Dr Brisbane, who was a practicing physi- cian in the State, was now the Director of the Institute and that Dr Prosser had left town. The editorial comment continues, stating that "the Board successfully ousted soi-diasant 'ABBO' and has prevented Prosser from practicing in the State of Louisiana. The Journal has from time to time reflected upon the Board and feels now that credit is due and should be given for the suc- cessful issue of the legal proceedings in the case. It yet remains for the Board to bury the name of 'ABBO' by instituting the necessary measures to stop his successor from practicing under the same false title."
There is a case by Dr Orville Hurwitz of the treatment of cases of psychic impotence. He has two cases of sexual impotence in which he states a 33-year-old patient had an attack of anterior urethritis which ran the usual course and gave him no special trouble. However, as he stated, there was complete abeyance of his "sexual power". He became greatly depressed, he had pains in parts of the body and suffered episodes of dyspepsia and nervous alopecia. Examination showed no pathological condition except a slight hyperesthesia in the posterior urethra. He had been under treatment for several months and
60 J La State Med Soc VOL 1 52 February 2000
nearly recovered the use of his lost powers. He goes on to formulate that here was a case of im- potency without cause and an illustration of pure psychic impotence. The main point of treatment was to produce a mental impression upon the patient. He goes on to state that "under no cir- cumstances are you, the physician, to let him think that the cause of his trouble is mental. Some simple medicine should be given to the patient such as placebos. They should be given for the mental effect and not for its clinical use. One must listen with interest and attention to the man's story and gain his confidence which is the greatest aid in handling these cases. The pass- ing of a cold bougie or sound will produce an impression on the mind and may be a slight stimulation of the nerves though in itself it's re- ally of no use. The physical condition of the pa- tient should be improved as much as possible by careful psychological guidance." However, he goes on to state, "a great mental impression is made by the use of electricity, if applied in cases of atonic impotency from early 'sexual ex- cess'. One electrode is carried into the rectum, the bone resting against the prostate, the other one is applied against the perineum by means of a sponge. The current is then passed from the rectum towards the perineum. Two or three mil- liamperes should be used, but, if a current indi-
cator is not used, the current should be strong enough to produce a tingling sensation but not shock. This situation should be applied daily for 5 minutes at a time and then gradually increased to 10. This treatment will be found of service in the class of cases mentioned as well as in an atonic condition resulting from other causes. This, as well as psychological support, helps these patients get over their cases of psychic impotence."
Dr Colon has a plastic surgery practice in Metairie, Louisiana and has lectured on history of medicine at Louisiana State University Health Services Center and Tulane University School of Medicine, both in New Orleans, Louisiana.
J La State Med Soc VOL 152 February 2000 01
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Is it good medicine?
A call to lead: A challenge to serve
Make plans now to join virtually every leader in organized medicine today in sunny Miami Beach
for the 2000 National Leadership Development Conference.
Plenary sessions will feature nationally acclaimed speakers and panel participants.
Tom Peters, PhD, acclaimed author of In Search of Excellence and The Circle of Innovation, is the keynote speaker.
Ian Morrison, PhD, Senior Fellow, Institute for the Future, and noted author and consultant, will address Healthcare in the New Millennium.
Interactive break-out and optional sessions will include opportunities such as the AM. A /Intel Internet Health Road Show and:
• Future role of medical associations
• Breakfast and luncheon dialogues with your AMA leadership
• How to write a speech
• Media interview skills update
• Regaining physician collegiality in the medical profession
• Medical practice management
• Association management and team building
• Leadership skill building
To register for the NLDC and for additional information:
Call the AMA registration hotline, 800 262-3211 or visit the NLDC Web site, www.ama-assn.org
To reserve a hotel room:
Call 800 548-8886 or 305 538-2000 or visit the hotel Web site, www.hilton.com
To receive special room rates, be sure to:
Identify yourself as a participant in the 2000 National Leadership Development Conference. Register before the February 25, 2000, cut-off date.
AMA/ Glaxo Wellcome
Emerging Leaders Development Program
This day-long skill-building experience on March 25,
2000, is by invitation only and is limited to 50 physicians. The program aims to help physicians succeed in the legislative /regulatory, organized medicine, and managed care arenas. An application, which must be postmarked by December 17, 1999, can be found on the AMA Web site at www.ama-assn.org. Participation includes complimen- tary registration for the NLDC and CME credit.
American Medical Association
Physicians dedicated to the health of America
Public Health
Public Health in Louisiana: An Introduction
Elizabeth T.H. Fontham, DrPH
We are pleased to welcome you to this special public health issue of The Journal of the Louisiana State Medi- cal Society, the result of a collaborative project with Louisiana State University Health Sci- ences Center, Louisiana Public Health Asso- ciation, and the Louisiana State Medical Soci- ety. Focusing on important health topics which link medicine and public health in Louisiana, this issue features articles addressing rural health, the state health code, ingested lead lev- els, and public health projects throughout the State. Physicians who are interested in expand- ing their public health knowledge can read about the variety of educational opportunities available to them throughout the State, includ- ing some innovative distance learning pro- grams.
This project has been supported through the Cooperative Actions for Health Program (CAHP), a collaborative grant co-sponsored by the American Public Health Association and the American Medical Association, with fund- ing from The Robert Wood Johnson Founda- tion. We believe that collaboration between medicine and public health is essential for improving the health of the people we serve.
We congratulate the leaders of the Loui- siana CAHP project, Drs Larry Hebert and James Osterberger, co-chairmen of the project, and Dr Anne Jordan, who has served as the Project Coordinator. We hope that their efforts, and the work of the authors who have contributed to this special issue, inspire new and expanded partnerships between medicine and public health in service to the people of Louisiana.
Dr Fontham is Professor and Chairman of the Department of Public Health and Preventive Medicine , LSU Health Sciences Center in New Orleans , Louisiana
J La State Med Soc VOL 152 February 2000 S3
Public Health
Blood Mercury Levels and Fish Consumption in Louisiana
Tracie M. Bellanger, BA; Erica M. Caesar, MSPH; Louis Trachtman, MD, MPH
The primary source of non-occupational exposure to mercury is through the consumption of contaminated fish. Since 1994, the Louisiana Department of Environmental Quality has reported mercury contamination in fish obtained from bodies of water throughout the state and has issued fish consumption advisories accordingly. To determine the extent of mercury intoxication in Louisiana, screening for blood mercury levels was offered to volunteers residing near selected advisory areas. A total of 313 residents participated in the screening; 6 were found to have elevated levels. No level was detected in 48 of the participants, while the remaining participants had normal levels. Significantly higher levels were found in those associated with commercial fishing and those reporting increased fish consumption. For most people, ordinary consumption of fish contaminated with mercury does not currently appear to pose a public health hazard in Louisiana; however, educational efforts regarding the risks of fish consumption in great quantities should be continued.
Whether from natural sources such as mineral deposits, oceanic emissions, and volcanic eruptions, or from hu- man activities such as mining, combustion of fossil fuels, and industrial emissions, mercury is a substance present everywhere in the envi- ronment. Methyl mercury, the most common organic form of mercury, is produced when mi- croorganisms in the soil and water interact with inorganic mercury. Because it has a high affin- ity for protein sulfhydryl groups,1 methyl mer- cury accumulates in organisms and is enriched along the food chain.2
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While terrestrial food is a negligible source of methyl mercury for the general population, certain aquatic species, particularly large preda- tory fish, accumulate this organic form of mer- cury in liver, kidney, brain, and muscle tissues.3 Thus, the primary source of non-occupational exposure to mercury for humans is through the consumption of contaminated fish.4
The potential for mercury toxicity in Louisi- ana residents is a legitimate concern for two rea- sons. First of all, fish is a main dietary compo- nent in this state and much of the fish consumed is caught in local waters. Secondly, the Louisi-
Public Health
ana Department of Environmental Quality has reported the presence of mercury in fish ob- tained from local bodies of water. Since 1994, this department has conducted annual testing in more than 100 bodies of water throughout the state. The edible portions of the fish tested were found to have mercury contamination in all tested areas. Advisories regarding the consump- tion of mercury contaminated fish have been is- sued by the Louisiana Department of Health and Hospitals, the Louisiana Department of Environ- mental Quality, and the Louisiana Department of Wildlife and Fisheries (Appendix).
Louisiana is not facing this problem alone. In 1992, the state of Arkansas began creating similar advisories after discovering mercury lev- els in local fish exceeded the Food and Drug Ad- ministration (FDA) tolerance limits.5 To deter- mine the extent of mercury intoxication, the Ar- kansas Department of Health provided baseline blood mercury screening to volunteers who lived in eight affected counties. Two hundred thirty-six participants, who confirmed their fish consumption was a minimum of two meals per month of fish caught in the lower Saline or Ouachita Rivers, were tested. Fifteen percent of those tested were found to have blood mercury levels in the elevated range of 20-75 parts per billion (ppb), while 25% were found to have no detectable blood mercury levels.6
Increasing public awareness of the presence of mercury in our environment and the poten- tial for toxicity has prompted the Louisiana state legislature to follow in Arkansas' footsteps and provide similar screening for elevated blood mercury levels in residents living near selected advisory areas. This screening is essential to determine the extent of mercury intoxication in Louisiana residents and to evaluate the poten- tial health risks of consuming contaminated fish caught in local waters.
I n this article, we review the historical and toxicological considerations of mercury poison- ing and discuss the results of this screening with regard to age, occupation, race, education, fre- quency of fish consumption, and pregnancy.
Historical Considerations
Mercury was first used medically to treat syphilis in the late 15th century.7 Gradually, its toxicity became known and by the 19th century it was generally accepted as toxic and its me- dicinal use was markedly decreased. The toxic- ity of mercury was brought to the attention of the scientific community by its use in industry. In 1863, Frankland and Duppa used dimethyl mercury to determine the valency of metals and metallic compounds.8 Later that year, two labo- ratory technicians participating in the study died of mercury intoxication.9 Consequently, mercury's toxicity became well known among chemists; however, physicians did not appreci- ate the potential for mercury intoxication until an industrial accident brought it to the forefront. In 1940, four industrial workers were hospital- ized with methyl mercury poisoning as a result of an industrial accident.10 In 1950, one of those workers died; the subsequent autopsy revealed destruction of neurons with cerebral and cerebel- lar atrophy. These pathological findings along with the presenting symptoms of progressive ataxia, impaired speech, and constricted visual field became known as Hunter-Russell syn- drome and methyl mercury was designated the etiologic agent.11
Meanwhile, in the early 1950s, the Chisso Corporation chemical factory was discharging its waste effluent, contaminated with methyl mercury, into Minamata Bay in Japan — a com- mon practice at the time. A disease similar to Hunter-Russell syndrome emerged, but it also presented with deafness and sensory abnormali- ties. Minamata Disease, as it was named, was not officially recognized by the Japanese gov- ernment as being caused by environmental pol- lution with methyl mercury until 1968. 12 More than 2,250 patients have been officially recog- nized as having Minamata Disease, 1,043 of whom have died.13 The number of unofficial sufferers is believed to be much greater.
A second epidemic of mercury poisoning occurred in 1976 in Iraq. More than 6,000 people
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Public Health
were affected when they consumed bread made from grain treated with methyl mercury fungi- cide. At least 500 of those people died as a result of the intoxication.14 Similar episodes have oc- curred with seed grain contaminated with me- thyl mercury in Guatemala15 and Pakistan.16
Several regulations have been established regarding methyl mercury since these outbreaks have occurred. In 1973, an allowable mercury concentration in fish was established by Japan's Ministry of Health and Welfare — total mercury at 0.4 gg/g and methyl mercury at 0.3 gg/g.17 Government authorities also established that there should be no detectable mercury or me- thyl mercury in industrial waste water.
In the United States, the FDA established an action level of 1 ppm methyl mercury; commer- cial fish and shellfish, as well as treated seed grain, sold through interstate commerce found to have levels exceeding 1 ppm cannot be sold to the public. The Environmental Protection Agency (EPA), in conjunction with the FDA, has set a limit of 2 ppb inorganic mercury in drink- ing water. The EPA also currently recommends the level of inorganic mercury in rivers, lakes, and streams should be less than 144 parts per trillion (ppt) to protect human health.18
Toxicological Considerations
The critical organ system affected in humans by methyl mercury is the central nervous sys- tem. Mercury is a neurotoxic agent, affecting pri- marily the occipital cortex and cerebellum.19 Me- thyl mercury poisoning is evident with low lev- els of exposure and presents most commonly with nonspecific signs and symptoms, includ- ing paresthesias, ataxia, constriction of the vi- sual field, and impairment of hearing.20 With prolonged daily methyl mercury intake of 3-7 gg/kg body weight, the incidence of poisoning is 5%. 21
Prenatal exposure to high-dose methyl mer- cury is particularly devastating and can cause mental retardation and cerebral palsy in the new- born. In all cases of reported fetal methyl mer- cury poisoning, the source was dietary; however.
only in Minamata and Niigata, Japan, was fish consumption involved.22 The fish consumed in these areas contained very high methyl mercury levels secondary to local waterway pollution. Consumption of fish with low levels of methyl mercury, below 1 ppm, during pregnancy has not been shown to place the fetus at neurodevelopmental risk.23
The International Commission of Occupa- tional Health and the International Union of Pure and Applied Chemistry Commission on Toxi- cology have determined the average baseline whole blood level of mercury to be approxi- mately 2 ppb in people who do not eat fish.24 In people who do eat fish, a normal blood level of mercury is between 2 and 20 ppb. Levels greater than 20 ppb are considered elevated and it is recommended that those people decrease fish consumption. Levels greater than 80 ppb in the general population25 and 40 ppb in children and pregnant women26 need medical evaluation, as well as decreased consumption of fish. Levels greater than 200 ppb are associated with a 5% incidence of poisoning.27 This level results from chronic daily methyl mercury intake of 3-4 gg/ kg body weight for at least 1 year.28
Screening Protocol
From February to March of 1998, the State Office of Public Health offered free blood mer- cury screening through local parish health units to residents in thirteen parishes, including Acadia, Caldwell, Evangeline, Iberia, Jefferson Davis, Lafayette, Morehouse, Ouachita, St Landry, St Martin, St Tammany, Vermilion, and Washington parishes. Participants also com- pleted a written questionnaire regarding risk factors for mercury poisoning.
Whole blood samples, collected by venipunc- ture, were analyzed by cold vapor atomic ab- sorption in the Office of Public Health Central Laboratory, New Orleans, Chemistry Section. This laboratory complies with the quality con- trol procedures recommended by the Center for Disease Control and Prevention (CDC). The limit for detection of blood mercury is 0.30 ppb.
66 J La State Med Soc VOL 152 February 2000