PSEUDOHYPERTROPHIC MUSCULAR DYSTROPHY: A CASE REPORT

Abstract
A 10-year-old boy was admitted to the hospital with acute meningococcic meningitis and meningococcemia. At the same time enlarged calf muscles, lumbar lordosis, tapir -facies, Gowers'' sign, etc., were observed which led to the diagnosis of pseudohypertrophic muscular dystrophy. A family history was obtained which revealed the presence of a sex-linked progressive muscular disease transmitted only by females in the family and manifest only in males. Seven males in four generations were affected. The patient recovered from the meningitis following which studies of creatine-creatinine metabolism were undertaken. Defects in the metabolism of these substances consisting of creatinuria, diminished creatine tolerance and decreased excretion of creatinine, were observed. Muscle biopsy taken from the thigh and calf revealed the typical lipomatous pseudohypertrophy of progressive muscular dystrophy.

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