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February 1966

Familial Proximal Spinal Muscular Atrophy

Author Affiliations

From the Department of Neurology, Hospital and School of Medicine, University of Pennsylvania, Philadelphia.

Arch Neurol. 1966;14(2):208-212. doi:10.1001/archneur.1966.00470080092014

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

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