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November 1952


Author Affiliations


From the Department of Neurology, University of Pennsylvania School of Medicine.

AMA Arch NeurPsych. 1952;68(5):655-682. doi:10.1001/archneurpsyc.1952.02320230081010

SINCE 1876,1 a heredofamilial disease has been known in which the only disturbance, or the most prominent disturbance, has been a slowly progressive weakness and spasticity of the lower extremities. Contributions by Strümpell2 and Lorrain3 have led writers to designate this disease by their names. By 1939, according to Price,4 reports on 158 families presumed to have this condition had accumulated in the literature, although another writer,5 in the same year, included in her excellent study only 74 pedigrees, with a total of 316 affected members. She added one more pedigree of familial spastic paraplegia of her own. Since that time several more families have been reported in the literature.

There exists a considerable variation of opinion about many aspects of this disease, e. g., the clinical identity of the disease, that is, whether it is really a separate syndrome or represents but one of