Chronic neurogenic quadriceps amyotrophy.
Two patients with chronic neurogenic quadriceps amyotrophy are reported. A 61-year-old woman had symmetrical wasting and weakness of the quadriceps femoris of eight years' duration. A biopsy revealed neuropathic changes, and electromyograms showed neurogenic as well as myopathic patterns in the quadriceps and other muscles. A 29-year-old woman had long-standing wasting limited to the quadriceps and hip muscles. EMG and biopsy were compatible with neurogenic atrophy. She had a brother suffering from a more widespread and severe form of the disease. These cases suggest that chronic neurogenic quadriceps amyotrophy is a forme fruste of Kugelberg-Welander disease.
D000328:Adult; D001706:Biopsy; D002908:Chronic Disease; D003402:Creatine Kinase / Q000097:blood; D004576:Electromyography; D005260:Female; D006801:Humans; D008875:Middle Aged; D009123:Muscle Hypotonia / Q000175:diagnosis* / Q000201:enzymology / Q000473:pathology; D009132:Muscles / Q000473:pathology; D009133:Muscular Atrophy / Q000473:pathology; D009431:Neural Conduction; D009468:Neuromuscular Diseases / Q000175:diagnosis* / Q000473:pathology; D013848:Thigh
D003402:Creatine Kinase