SINCE THE REPORTS of Wohlfart et al1 and Kugelberg and Welander,2 there have been a number of case reports3-10 describing patients with slowly progressive proximal limb weakness of neurogenic Origin. The original descriptions include these features: onset of weakness between the ages of 2 and 17 years, slow progression of weakness with atrophy, fasciculations, hyporeflexia, an autosomal recessive inheritance, electromyographic findings, and histological changes on biopsy consistent with a neuropathic lesion.
This report is about a single Negro family of 64 members. Nineteen have been affected with a slowly progressive neurogenic weakness.
Report of Cases
CASE 1.—III-5 is a 37-year-old man presently employed as a taxicab driver and serving as a minister. Weak since childhood, he was able to work as a construction laborer in early adolescence and lift weights of 100 lb. He served two years in the army medical and quartermaster corps and on
ARMSTRONG RM, FOGELSON MH, SILBERBERG DH. Familial Proximal Spinal Muscular Atrophy. Arch Neurol. 1966;14(2):208–212. doi:10.1001/archneur.1966.00470080092014
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