[Many faces of West Nile fever--the first case of West Nile fever in the western Galilee, Israel].

West Nile Fever (WNF) is caused by a B arbovirus, which was first isolated in 1937 in Uganda. In Israel the disease bears an epidemic character, and during 1950-1957 several widespread outbreaks of WNF were described in detail. It emerged from obscurity in 1999 when the first incursion of the virus in North America caused 62 cases of encephalitis and 7 deaths in New York. In 1996 an outbreak in Romania signaled that WNF had emerged as clinically important disease in Europe. The virus is transmitted by a mosquito vector. Wild birds serve as amplifying hosts. Human and some domestic animals such as horses, can be infected incidentally. While neurological disease has been prominent in some epidemics, the West Nile virus infection is usually asymptomatic in areas of the world where the virus is endemic. This is a case study of the first patient diagnosed and treated for West Nile Fever in the Western Galilee, Israel. The epidemiology, pathogenesis, clinical manifestations, diagnosis and treatment of the disease are presented in detail.

Fibromyalgia-Like Syndrome Secondary to Addison's Disease.

Fibromyalgia is a syndrome of musculoskeletal pain and tenderness in specific sites, fatigue, and sleep disturbance. Fibromyalgia may occur in patients with rheumatoid arthritis and other connective tissue diseases. Despite the fact that fibromyalgia patients may have alterations in the hypothalamic-pituitary-adrenal axis, no relationship had been established between fibromyalgia and diseases of the adrenal cortex. We report a case of a 46-year-old woman who presented with a new onset of a fibromyalgia-like syndrome meeting all American College of Rheumatology criteria except chronicity. Additional clinical features, including skin pigmentation, mild hypertension, and hyponatremia, as well as further laboratory testing led to a diagnosis of autoimmune Addison's disease. After steroid replacement, the symptoms of fibromyalgia disappeared. This is the first case in English-language literature of fibromyalgia secondary to Addison's disease. We suggest Addison's disease should be considered in the differential diagnosis of any patient with a fibromyalgia-like syndrome.

[Self-limited lymphadenopathy mimicking lymphoma or lupus (Kikuchi-Fujimoto disease)].

Kikuchi-Fujimoto disease in a self-limited lymphadenopathy that can be confused histologically and clinically with lymphoma or systemic lupus erythematosus. It was diagnosed in a 37-year-old woman presenting with fever, cervical, submandibular and axillary lymphadenopathy, weight loss and recurrent urinary tract infections. Lymph node biopsy was consistent with the diagnosis of a histiocytic necrotizing lymphadenitis. Early diagnosis of Kikuchi-Fujimoto disease can prevent harmful treatment.

[Miliary and reticulo-nodular pulmonary brucellosis].

Brucellosis is a classical zoonosis caused by a Gram-negative bacillus of the genus Brucella. Human brucellosis can either be acute or chronic and present with a variety of manifestations, mostly with fever and signs of musculo-skeletal involvement. It may be complicated by involvement of the cardiovascular, central nervous or genito-urinary systems. However, pulmonary brucellosis is a rare complication. We report a case of miliary and reticulo-nodular brucellar pneumonia with positive blood and sputum cultures and positive serological tests. To the best of our knowledge this is the first case to be reported from Israel of miliary pneumonia with sputum positive for brucellosis.

Altered interleukin-2 secretion in patients with primary fibromyalgia syndrome.

Interleukin-2 (IL-2) production was studied in T lymphocytes and isolated CD4+ T lymphocytes from 12 patients with primary fibromyalgia syndrome and 10 healthy volunteers. The dose and time kinetics of IL-2 production by concanavalin A-stimulated T cells and CD4+ T cells of fibromyalgia patients differed from findings in controls by 1) a need for a higher concentration of mitogen in order to achieve optimal IL-2 secretion, and 2) a delay in the peak time of optimal IL-2 secretion. Unlike normal IL-2 secretion, which was higher after removal of CD8+ T cells, the pattern and degree of IL-2 secretion by cells from fibromyalgia patients were not changed following removal of CD8+ T cells. Addition of calcium ionophore in assays using suboptimal concanavalin A concentrations did not correct the reduction in IL-2 secretion by fibromyalgia patient T cells, but addition of phorbol myristate acetate induced normal secretion of IL-2. These findings suggest that there is a defect in the IL-2 pathway, which is related to protein kinase C activation and does not involve impairment of Ca2+ elevation, in patients with fibromyalgia.

Retroperitoneal lymphadenopathy in familial Mediterranean fever.

Peripheral lymphadenopathy is rarely observed, whereas mesenteric lymphadenopathy is found occasionally on laparotomies in patients with familial Mediterranean fever (FMF). Retroperitoneal lymphadenopathy was reported only once in an autopsy of a patient with FMF. Our case is the second one, and the first one to be diagnosed during life, by means of abdominal ultrasonography and computerized tomography. In patients with FMF, where lymph node biopsy was done, the pathological finding was non-specific lymphoid hyperplasia.

Hypercholesterolemic (type II hyperlipoproteinemic) arthritis.

The rheumatic manifestations of familial hypercholesterolemia include recurrent Achilles pain or tendinitis, acute mono/oligoarthritis and migratory (rheumatic fever-like) polyarthritis. Diagnosis is made by finding skin and tendon xanthomas, hypercholesterolemia, and ruling out other rheumatic conditions such as rheumatic fever, gout, pseudogout and septic arthritis. A patient, homozygous for familial hypercholesterolemia, with a rheumatic fever-like migratory arthritis is presented.

Brachial plexitis complicating disseminated gonococcal infection.

A patient with brachial plexitis associated with disseminated gonococcal infection is presented. This is, to the best of our knowledge, the first case to be reported of this association.

The diagnosis of polyarteritis nodosa. I. A literature-based decision analysis approach.

We investigated diagnostic testing in polyarteritis nodosa (PAN) by calculating, from published data, the sensitivity and specificity of visceral angiography and muscle, nerve, testicle, kidney, and liver biopsy. Test sequence strategies were constructed by Bayesian inference using a computer program written for this purpose. Test sequences were compared with an aggressive strategy consisting of repeated tests until there was a positive finding or until the available tests were exhausted, and a conservative strategy consisting of 1 biopsy procedure plus angiography. The Bayesian analysis agreed most closely with the conservative approach for most prior probabilities (degree of suspicion) that a patient had PAN. The aggressive strategy had an overall sensitivity of 90% and specificity of 91%, whereas the conservative strategy was 85% sensitive and 96% specific. Furthermore, the aggressive strategy was more costly ($2,986 versus $1,961) and had a higher rate of morbidity (3.8 versus 2.7 days of hospitalization per patient evaluated) than did the conservative strategy. The mortality rates of both strategies were equivalent (approximately 0.05 deaths per hundred patients evaluated). The per-case cost of diagnosis increased as prevalence decreased, and at 10% prevalence, the aggressive strategy cost more than $17,000 per case diagnosed. Sensitivity analysis revealed that the strategies were moderately affected by the test characteristics, within reasonable assumptions, but that the differences in conservative and aggressive approaches remained. Thus, our analysis based on available data and the assumption of test independence suggests that the preferred diagnostic evaluation of patients with symptoms suggestive of PAN consists, in most cases, of a single biopsy procedure, with angiographic evaluation if necessary.

Cardiomyopathy and multiple myeloma. Complications of scleredema adultorum.

Multiple myeloma and congestive heart failure developed in a patient with long-standing scleredema adultorum. Staining of the myocardium, performed after her death, was positive for acid mucopolysaccharide and negative for amyloid. To the best of our knowledge, this is the first case in which acid mucopolysaccharide has been demonstrated in the myocardium, thus explaining the cardiomyopathy of scleredema adultorum. Review of the world literature enabled us to identify a statistically significant increased prevalence of plasma cell dyscrasia among patients with protracted scleredema. In all patients, plasma cell dyscrasia appeared years after the onset of scleredema. Immunofluorescent studies were negative for immunoglobulin deposition. We assume, therefore, that the plasma cell dyscrasia was secondary to scleredema.

Selective expression of phosphoribosylpyrophosphate synthetase superactivity in human lymphoblast lines.

Phenotypic expression of 5-phosphoribosyl 1-pyrophosphate (PRPP) synthetase superactivity was examined in lymphoblast lines derived from six unrelated male patients. Fibroblasts from these individuals have increased rates of PRPP and purine nucleotide synthesis and express four classes of kinetic derangement underlying enzyme superactivity: increased maximal reaction velocity (catalytic defect); inhibitor resistance (regulatory defect); increased substrate affinity (substrate binding defect); and combined catalytic and regulatory defects. Lymphoblast lines from three patients with catalytic defects and from three normal individuals were indistinguishable with respect to enzyme activities, PRPP concentrations and generation, and rates of purine synthesis. Enzyme in lymphoblasts from a patient with combined defects also showed normal maximal reaction velocity but expressed purine nucleotide inhibitor resistance. A second regulatory defect and a substrate binding defect were also demonstrable in lymphoblasts and were identical to the enzyme defects in fibroblasts from the respective patients. Regulatory and substrate binding defects in lymphoblasts were accompanied by increased rates of PRPP and purine nucleotide synthesis. Among explanations for selective expression of enzyme superactivity, reduced concentrations of catalytically superactive enzymes seemed unlikely: immunoreactive PRPP synthetase was comparable in normal-derived and patient-derived cells. Activation of normal enzyme in transformed lymphocytes was also unlikely because absolute specific activities of lymphoblast PRPP synthetases corresponded to those of normal fibroblast and erythrocyte enzymes. Abnormal electrophoretic mobilities and thermal stabilities, identified in certain catalytically superactive fibroblast PRPP synthetases, were not found in the corresponding lymphoblast enzymes. Thus, lymphoblast PRPP synthetases from patients with catalytic superactivity appeared to differ structurally and functionally from their fibroblast counterparts.

Behçet's disease: clinical and immunological response to immunosuppressive therapy. A case report.

A patient with Behçet's disease had recurrent bilateral uveitis with retinal exudates and visual deterioration while under intermittent corticosteroid treatment. His peripheral blood lymphocytes showed the following abnormalities: low percentage of T cells; low T cell suppressor activity; high spontaneous B cell proliferation; and high response to phytohemagglutinin. Prostaglandin-producing adherent cells were found to have suppressive activity. Chlorambucil induced a clinical and immunological remission.

Clofibrate-induced muscular syndrome. Case report with ultrastructural findings and review of the literature.

A 70-year-old man with chronic renal failure was treated with clofibrate, 2.0 g daily for 6 days, and subsequently developed clofibrate-induced muscular syndrome (CMS), i.e., myalgia with severe muscle weakness and tenderness. Elevated muscular enzyme activities were found upon examination of his blood. Electron microscopic studies revealed atrophy of some muscle fibers and massive degenerative changes. The atrophic fibers were associated with damaged neuromuscular junctions. A literature survey of 44 cases with CMS is included.