[Apropos of a case of myopathy with histologic and electrophysiologic findings of both a myogenic and neurogenic nature].
The authors describe the case of a 36 years old woman suffering from muscular weakness with proximo-distal distribution to legs, and proximal to arms. The disease, appeared during the third decade of life, is slowly becoming more serious. Hematochemical analyses are all within a normal standard; EMG and histopathologic findings prove the existence of both a protopathic and neurogenic trouble in studied muscles. A therapy based on prednisone (50 mg/die) and ACTH (1 mg each 5 days) for a fourty days period doesn't cause essential changes in symptomatology. A cousin (on her mother's side) of our patient suffers from probable "sporadic distal myopathy". The authors discuss if the disease, shown by this patient, may be considered as an atypical form of SDM or if, what appears more probable, it must be nosographically framed as "scapuloperoneal atrophy".
D000328:Adult; D004576:Electromyography; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D009132:Muscles / Q000201:enzymology / Q000473:pathology*; D009431:Neural Conduction; D009468:Neuromuscular Diseases / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D010525:Peripheral Nerves / Q000473:pathology* / Q000503:physiopathology
A proposito di un caso di miopatia con reperti istopatologici ed elettrofisiopatologici di natura sia miogena che neurogena.