Strumpell's familial spastic paraplegia: genetics and neuropathology

Abstract

Uncomplicated Strumpell's disease (Strumpell's familial spastic paraplegia) with a dominant mode of inheritance is recorded in six families. The neuropathologic findings in two cases from these families are given, bringing the total of similar histologically documented reports in the literature to 11. It is concluded that, although exact classification and identification of the many different hereditary neurologic degenerative diseases is not yet practicable, cases conforming to the picture described by Strumpell can be separated from the larger general group of familial spastic paraplegias, show a consistent clinical picture, and have a standard pathology. It is suggested that, since the lesions are confined to the longest fiber tracts in the central nervous system, the pathologic process may be different from that found in the 'system' degenerations.