Multisystem involvement of the central nervous system in Strümpell's disease. A neurophysiological and neuropsychological study.

The multisystem involvement of the central nervous system (CNS) in familial spastic paraplegia (FSP) has not been fully investigated by means of complete neurophysiological and neuropsychological examinations. The classification which distinguishes pure and complicated forms of FSP, is based on clinical features and does not take into account the possibility that clinically silent lesions of the CNS can be identified by means of adequate tests. The study was intended to assess the subclinical and multisystem involvement of the CNS in a group of 11 patients affected by FSP, clinically distinguished in 7 pure forms and 4 complicated forms. Neurophysiological tests included saccadic eye movements analysis, visual and auditory brain stem evoked responses. Neuropsychological examination was devised by means of a special purpose mental deterioration battery. Our results, showing a high incidence of multisystemic subclinical involvement of the CNS, confirm and extend the concept that FSP is a multisystemic degenerative disease of the CNS, and that the existence of "pure" forms should be reconsidered.