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August 1959

Familial Spastic Paraplegia with Amyotrophy, Oligophrenia, and Central Retinal Degeneration

Author Affiliations

From the Department of Neurology, Karolinska Institutet, Serafimerlasarettet.

AMA Arch Neurol. 1959;1(2):133-140. doi:10.1001/archneur.1959.03840020007002

About 200 pedigrees with familial spastic paraplegia have been recorded in the literature. In many of the cases other abnormalities were observed in addition.* Cases with coexisting spastic paraplegia and ataxia have been described as hereditary ataxia of the spastic type by Bell and Carmichael in their critical study of familial spastic paraplegia.2

In the present article a syndrome comprising paraplegia, amyotrophy, oligophrenia, and central retinal degeneration occurring in two pairs of brothers (A and B) is described. This syndrome does not appear to have been reported earlier.

Report of Cases  Case Al.—The patient was a 37-year-old man, the older brother of Patient A2. According to the relatives, he had always been mentally retarded, probably with no deterioration. Since the age of 30(?) he had observed increasing stiffness and weakness in his legs when walking, especially in going down stairs. The difficulty in walking had become so pronounced that

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