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Still’s disease is a rare disorder that occurs in both children and adults. It is designated as the systemic form of juvenile rheumatoid arthritis (JRA) in children younger than 16 years, and is called adult-onset Still’s disease (AOSD) in older patients. It is characterized by high fever, a salmon-colored skin rash, arthritis or arthralgias, and a markedly increased leukocyte count. It may be accompanied by sore throat, splenomegaly, or lymphadenopathies. Although involvement of the heart, lungs, liver, and gastrointestinal tract has been reported, neurologic involvement is unusual. We report five episodes of acute neutrophilic meningitis occurring in four female patients with Still’s disease. Diagnoses of systemic onset JRA (Patients 1 to 3) and AOSD (Patient 4) were based on the presence of the four major Reginato criteria.1 Patients 1, 2, and 4 exhibited persistent or intermittent fever, an evanescent erythematous maculopapular rash, poly- or oligoarthritis, and neutrophilic leukocytosis. Patient 3 satisfied only three major criteria (no rash), …
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Blockmans et al. (2000) studied this question.
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