JOHN BOYD, MD. Durango, Colorado. ALTHOUGH TULAREMIA has declined in incidence since it was first described by Edward Francis in 1925,1 it continues ...
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s TABLE 2.-Clinical D eR matic FeerAccording to the - viXe Jones'Critera
RewsedJones'Cnteria
This PWent
Major
Carditis .................... Arthritis . Erythema marginatum ........................ Rheumatic nodule.............-....... Rheumatic chorea ...........
.........
Minor Fever
.
intseral in ekectmKardogram NOoO#ed PR.
Pencarditis Migratory polyarthritis
Abient
Absent: Absent
LOW-grade fever
.
.......................
Leukocytosis High eythocyft sedimentation rate ..........
Previous rheumatic fever . Prerequisite Evidence of treptococcal infection . Rheumatoid arthritis ruled out ..
High-grade AV block PresentEyhxoqctesedimentation rate 110 mm/h
Absent
Antistreptolysin NO" titer 330 U/mI
Yes
icar AV-iabioventr
matic fever with dominating carditis was confirmed by his subsequent development of polyarthritis and pericarditis. Such a combination of atrial fibrillation and AV block as a presentation of acute rheumatic fever has not been reported previously. Bradytachyarrhythmias are encountered frequently in clinical practice, in particular among the geriatric population. Now that the medical community is facing a possible widespread return of rheumatic fever, this report highlights an atypical manifestation of the disorder and should alert clinicians to consider bradytachyarrhythmias as a presenting dominant manifestation of acute rheumatic carditis. REFERENCES 1. Strasser T: Rheumatic fever and rheumatic heart disease in the 1970s. WHO Chron 1978; 32:18-25 2. Stollerman GH: Rheumatic Fever and Streptococcal Infection. New York, NY, Grune & Stratton, 1975, pp 63-99 3. DiSciascio G, Taranta A: Rheumatic fever in children. Am Heart J 1980;
99:635-658 4. Veasy LG, Wiedmeier SE, Orsmond GS, et al: Resurgence of acute rheumatic fever in the intermountain area of the United States. N Engl J Med 1987; 306:421-427 5. Congeni B, Rizzo C, Congeni J, Sreenivassan VV: Outbreak of acute rheumatic fever in northeast Ohio. J Pediatr 1987; 1 1 1:176-179 6. Hosier DM, Craenen JM, Teske DW, Wheller JJ: Resurgence ofacute rheumatic fever. Am J Dis Child 1987; 141:730-733 7. Chun LT, Reddy DV, Yamamoto LG: Rheumatic fever in children and adolescents in Hawaii. Pediatrics 1987; 79:549-552 8. Giardina AC: Resurgence of acute rheumatic fever. N Engl J Med 1987; 317:507-508 9. Bissenden JG: Transatlantic warning bells sound on rheumatic fever. Br Med J 1989; 296:1215 10. Bland EF: Rheumatic fever: The way it was. Circulation 1987; 76:1190-1213 11. Barnet AL, Terry EE, Persellin RH: Acute rheumatic fever in adults. JAMA 1975; 232:925-928 12. Persellin RH: Acute rheumatic fever: Changing manifestations [Editorial]. Ann Intern Med 1978; 89:1002-1003 13. Majeed HA, Khan N, Dabbagh M, Najdi K, Khateeb N: Acute rheumatic fever during childhood in Kuwait: The mild nature of the initial attack. Ann Tropic Pediatr 1981; 1:13-20 14. Woo KS, Kong SM, Wai KH: The changing prevalence and pattern of acute rheumatic fever and rheumatic heart disease in Hong Kong (1968-1978). Aust NZ J Med 1983; 13:151-156 15. Ad Hoc Committee to Revise the Jones' Criteria (modified), Council on Rheumatic Fever and Congenital Heart Disease of the American Heart Association: Jones' criteria (revised) for guidance in the diagnosis of rheumatic fever. Circulation 1984; 69:204A-208A 16. Stollerman GH: Clinical manifestations of acute rheumatic fever. In Rheumatic Feverand Streptococcal Infection. New York, NY, Grune & Stratton, 1975, pp 147-177 17. Robinson RW: Effect of atropine upon the prolongation of the P-R interval found in acute rheumatic fever and certain vagotonic persons. Am Heart J 1945; 29:378-383 18. Stollerman GH: Connective tissue diseases of the cardiovascular system. In Braunwald E (Ed): Heart Disease-A Textbook of Cardiovascular Medicine. Philadelphia, Pa, Saunders, 1980, pp 1734-1735
19. Thomas WA, Averill JH, Castleman B, Bland EF: The significance of Aschoff bodies in the left atrial appendage. N Engl J Med 1953; 249:761 20. Virmani R, Roberts WC: Aschoff bodies in operatively excised atrial appendages and in papillary muscles-Frequency and clinical significance. Circulation 1977; 55:559-563
Tularemia Presenting as an Isolated Pleural Effusion LINDA M. FUNK, MD STEVEN Q. SIMPSON, MD GREGORY MERTZ, MD Albuquerque, New Mexico JOHN BOYD, MD Durango, Colorado
ALTHOUGH TULAREMIA has declined in incidence since it was first described by Edward Francis in 1925,1 it continues to occur both sporadically and in epidemics in the United States.2 There are several forms of tularemia, but it is the pneumonic form that is often mistaken for other, more common diseases. Because pulmonary tularemia is the most severe form, with the highest reported rates of mortality,3 its recognition and treatment by physicians in and around endemic areas are important. We report a case of tularemia presenting as pleurisy in a young woman.
Report of a Case The patient, a 37-year-old woman, was referred to the University of New Mexico Medical Center (Albuquerque) from her primary physician in southwestern Colorado, where she had been seen in mid-July 1991 with fever, chills, and pleuritic chest pain of two days' duration. Other presenting symptoms included diffuse myalgias, headache, night sweats, and a nonproductive cough. The results of chest (Funk LM, Simpson SQ, Mertz G, Boyd J: Tularemia presenting as an isolated pleural effusion. West J Med 1992 Apr; 156:415-417) From the Pulmonary Division (Drs Funk and Simpson), and the Division of Infectious Disease (Dr Mertz), Department of Medicine, University of New Mexico, Albuquerque. Dr Boyd is in private practice in Durango, Colorado. Reprint requests to Linda Funk, MD, Pulmonary Division, Department of Medicine, 5th Flr, Ambulatory Care Center, University of New Mexico, 2211 Lomas Blvd NE, Albuquerque, NM 87131-5271.
ALERTS, NOTICES, AND CASE REPORTS
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x-ray films, a ventilation-perfusion lung scan, and initial laboratory tests, including rheumatoid and antinuclear antibody serologic tests, were unremarkable. Blood cultures and malaria smears were negative. Her symptoms persisted, and she was admitted to the University of New Mexico Hospital for further evaluation. On admission to hospital ten days after presentation, the patient continued to have pleuritic pain, nonproductive cough, fever, and myalgias. She did not have any associated rash, insect bites, joint pain, or exposure to tuberculosis. Her medical history was unremarkable. She was married with two children; no family member had been ill. She worked extensively as a landscaper, and she admitted to having exposure to numerous animals at home, including a pet pig and a neighbor's dog that had recently become ill. She also had traveled in Honduras for ten days in May 1991 and there had mosquito bites. On physical examination, she had a temperature of 39°C (102.2°F), a heart rate of 96 beats per minute, and a respiratory rate of 24. She was flushed and had bilateral cervical adenopathy. Her chest was dull to percussion in the left base, with decreased breath sounds and no rales, rhonchi, or wheezing. A radiograph of the chest revealed a left-sided free-flowing effusion with left hilar adenopathy and no associated infiltrate. A diagnostic thoracentesis the next day yielded 20 ml of sanguineous fluid with 52 grams per liter (5.2 grams per dl) of protein, a lactate dehydrogenase level of 1,080 IU, glucose 30.5 mmol per liter (55 mg per dl), and a leukocyte count of 25.0 x 109 per liter (25,000 per II), with 0.49 granulocytes and 0.48 lymphocytes. Serum values included a total protein of 71 grams per liter (7.1 grams per dl), with a peripheral blood leukocyte count of 10.6 x 109 per liter (10,600 per Ml); 0.73 were granulocytes. A Gram's stain of the fluid did not reveal organisms, and initial cultures were negative. Skin tests were started for tuberculosis, and therapy with isoniazid, rifampin, and pyrazinamide was initiated. She became afebrile within 24 hours of starting therapy. On July 30 a pleural biopsy was done using an Abrams' needle. Microscopic examination revealed fragments of skeletal muscle and fibrinopurulent exudate, with no organisms or granulomata identified. Acid-fast and periodic acidSchiff-stained sections were negative for mycobacteria and fungi. Therapy was changed to rifampin, 600 mg a day orally, and doxycycline, 100 mg orally twice a day. On August 1, Francisella tularensis was cultured incidentally from the pleural fluid that had been collected in standard aerobic blood culture bottles. A serologic test for F tularensis was done on a serum specimen from admission and was negative. A repeat titer done on August 7 yielded a positive result at 1: 1,280. A Brucella titer on July 31 was positive at 1:40. She was treated for tularemia with streptomycin, 1 gram intramuscularly daily for 14 days, with resolution of her symptoms. Interestingly, the neighbor's dog had a titer negative for F tularensis but was later diagnosed with systemic lupus
erythematosus. Discussion In 1911 a new plague-like illness was discovered in squirrels by McCoy and Chapin while they were investigating outbreaks of bubonic plague after the 1906 San Francisco earthquake. The organism was isolated on a coagulated egg
medium and named Bacterium tularense after Tulare County, California, the site ofthe discovery.4 The first bacteriologically confirmed human case of infection was described by Wherry and Lamb in 1914.2 Francis followed with seven more cases in 1921. In 1928, drawing on his experience with more than 800 cases, Francis summarized the clinical and epidemiologic features of the disease. In 1959 the genus name ofthe organism was changed to Francisella in recognition of the major contributions he had made to the study of tularemia. Several clinical forms of the disease have been described, based on the portal of entry. Lymph node involvement is uniformly present. Most commonly, a primary ulcer is found on the skin (ulceroglandular), in the eye (oculoglandular), or in the nasopharynx (oropharyngeal). Lymphadenopathy can occur without a recognizable cutaneous ulcer (glandular). Infection is often localized in these forms, but more disseminated disease can occur as a result of bacteremia. Systemic illness is much more common when the organism enters through the gastrointestinal tract (typhoidal) or the lungs (pneumonic). Tularemia presenting as pleurisy and pleural effusion without pneumonia is uncommon. Pleural effusion is much more likely to occur when parenchymal abnormalities are present and has been reported in as many as 82% of cases with pneumonia.5.6 The incidence of pleural effusion increases as untreated disease progresses, but it has been found as early as three days after the onset of symptoms and is often bilateral."'8 Pleural effusions caused by F tularensis have characteristically been exudates, with protein values as high as 62 grams per liter and leukocyte counts that range from 6.3 to 16.0 x 109 per liter.2 Although lymphocytic-predominant fluid does occur, most earlier reports suggest that granulocytic predominance is more common.9'10 The fluid can be turbid or serosanguineous. Working with cultures of F tularensis is hazardous to laboratory personnel3 because the organisms are easily aerosolized and inhaled, placing workers at risk for the illness developing. In addition, culture of the organism requires special media; therefore, directed cultures of pleural fluid are rarely done. F tularensis has been recovered from pleural fluid by direct culture,11 but this is decidedly rare. Ftularensis does not stain well with Gram's stain, and the demonstration of the organism in pleural fluid by this method has not been reported. Serologic tests are used to diagnose tularemia. A titer of 1: 160 is presumed diagnostic.2 This value is somewhat arbitrary as there have been no studies that correlate cultureproven natural infections with antibody titers. Most titers are diagnostic within 10 to 14 days after presumed exposure; however, laboratory studies of volunteers using killed vaccine found that diagnostic titers were not achieved until the third week of illness. 12 Follow-up serologic titers often remain mildly elevated for years.12 Tularemia infection may stimulate antibodies to Brucella species through antigenic cross-reactivity. This is especially the case if the tularemia titer is greater than 1:320. Conversely, patients with brucellosis may have elevations in their tularemia titers.13 As our patient showed, this serologic cross-reactivity can lead to confusion regarding the correct
diagnosis. Our patient likely acquired her infection from the bite of a deer fly during the course of her landscaping work. Although
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rabbits have classically been linked to the illness, arthropodborne illness is becoming more common, with ticks being the principal reservoir.2 Rabbits and other mammals represent a source of infection, particularly in the winter months; these animals rarely represent true reservoirs, however, because most infected mammals either become sick and die or recover with loss of the organism.I Although little is known about the actual arthropod infection, it is thought that transovarial transmission occurs. Ticks may live as long as 21 years, with great potential for harboring the organism. 14(pp227,323) The organism has been identified in more than 55 different arthropods. Francis was the first to describe cases of tularemia caused by the deer fly. The disease had popularly been known as "deer fly fever," with a predominance noted in the Western states of Utah and Colorado and an associated peak incidence in the summer months. Typical of arthropod-borne infections, a definite source of infection is often not identified, as in our case. Streptomycin is the drug of choice in tularemia. In vitro it is both bacteriostatic and bactericidal. Patients treated with streptomycin generally defervesce in 48 hours and require
only one 14-day course of therapy. Gentamicin, tetracycline, and erythromycin have been used with only varying success.2 It was of interest that. our patient's fever resolved within 24 hours of the start of therapy with rifampin. Tularemia continues to present a diagnostic challenge to physicians in and around endemic areas. An understanding of its protean manifestations is essential in its recognition and appropriate treatment. In our case, tularemia presented as pleurisy with a pleural effusion. Although isolated pleural effusion with associated pleuritic pain is often self-limited and presumed viral in origin, we suggest that tularemia be considered in those patients whose illness fails to resolve in a timely manner. Serologic tests are diagnostic, and a rapid clinical response can be expected with streptomycin therapy. REFERENCES 1. Francis E: Tularemia. JAMA 1925; 84:1243-1250 2. Evans ME, Gregory DW, Schaffner W, McGee ZA: Tularemia: A 30-year
experience with 88 cases. Medicine (Baltimore) 1985; 64:251-269 3. Overholt EL, Tigertt WD, Kadull PJ, et al: An analysis of forty-two cases of laboratory-acquired tularemia: Treatment with broad-spectrum antibiotics. Am J Med 1961; 30:785-806 4. McCoy GW, Chapin CW: Studies of plague, a plague-like disease and tuberculosis among rodents in California. Public Health Bull 1912; 53:3-11 5. Dennis JM, Boudreau RP: Pleuropulmonary tularemia: Its roentgen manifestations. Radiology 1957; 68:25-30 6. Warring WB, Ruffin JS: A tick-borne epidemic of tularemia. N Engl J Med 1946; 234: 137-140 7. Miller RP, Bates JH: Pleuropulmonary tularemia-A review of 29 patients. Am Rev Respir Dis 1969; 99:31-41 8. Rubin SA: Radiographic spectrum of pleuropulmonary tularemia. AJR 1978;
131:277-281 9. Blackford SD, Casey CJ: Pleuropulmonary tularemia. Arch Intern Med 1941; 67:43-7 1 10. Kennedy JA: Pleuropulmonary tularemia-A discussion of the disease as a clinical entity, with report of three cases. JAMA 1942; 118:781-787 11. Jager BV, Ransmeier JC: Constrictive pericarditis due to bacterium tularenseReport of a case and review of reported cases of pericarditis occurring with tularemia. Bull Johns Hopkins Hosp 1943; 72:166-178 12. Saslaw S, Carhart S: Studies with tularemia vaccine in volunteers challenged with Pasteurella tularensis. Am J Med Sci 1961; 241:689-699 13. Francis E, Evans AC: Agglutination, cross-agglutination and agglutination absorption in tularemia. Public Health Rep 1926; 41:1273-1295 14. James MT, Harwood RF: Herm's Medical Entomology. London, Macmillan, 1969
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Medicolegal Complications of Postpartum Catatonia GEORGE BACH-Y-RITA, MD ALBERT DE RANIERI, MD San Francisco, Califomia
SINCE PRE-REVOLUTIONARY times and with the adoption from England of the mandate of parens patriae, protecting severely impaired mentally ill persons has been the responsibility of the state. This mandate calls for protecting all citizens or subjects unable to protect themselves, such as minors or the insane. To fulfill its social objectives and to protect the mentally ill from the abandonment and despair common before the development of modern psychiatric institutions, the state charged the medical and psychiatric community with responsibility for the care and treatment of the individual. Concomitantly, the legal community developed laws ensuring guardianship of the insane and incompetent. This partnership of care reached its zenith in the late 1960s with the crafting of laws such as the Lanterman-Petris Short Act of California, which weighed the medical care and the legal protection needs of the mentally ill, in an attempt to balance requirements and rights in a humane fashion. The Lanterman-Petris Short Act of California mandated the formation of an office of patients' rights advocacy. Among the many responsibilities defined under the California Welfare and Institution Code, Section 5500(a), patient advocacy for the mentally ill was "to protect their rights or to secure or upgrade treatment or other services to which they are entitled." An advocate's role in practice occurs in adversarial-type hearings with an attorney sitting in lieu of a judge. In the hearings, a patient's rights advocate protects the patient from the physician, who attempts to retain and treat the patient. The individual rights movement in the 1970s gave rise to a phenomenon that can best be described as distrust of the professional community. The field of psychiatry, which for a number of years had been retreating from ascribing a medical basis to psychiatric illness, was undermined further by prominent psychiatrists who perceived mental illness or psychosis as a myth or adaptation rather than an illness, advancing the perception of medical psychiatry as an instrument of abuse rather than treatment. Fostered by this distrust, a series of legal decisions and laws closely regulated care through the courts, exemplified by legislation restricting the use of electroconvulsive therapy. More recently, the decision in Riese v St Mary's Hospital separated involuntary admission to hospital from involuntary treatment with antipsychotic medications.1 Under this decision, these medications can be given to nonvoluntary patients only after a separate judicial hearing. In the two years since the Riese v St Mary's Hospital decision, we have become aware of its devastating consequences. Report of a Case The patient, a 22-year-old woman with no previous psychiatric history, was admitted to a psychiatric service ten days
(Bach-y-Rita G, DeRanieri A: Medicolegal complications of postpartum catatonia. West J Med 1992 Apr; 156:417-419) Reprint requests to George Bach-y-Rita, MD, 2100 Webster St, Ste 314, San Francisco, CA 94115.
