Strumpell's pure familial spastic paraplegia: case study and review of the literature

SUMMARY A family with pure Strumpell's familial paraplegia is presented. There were 11 afflicted members involving three generations. The mode of inheritance was dominant, the onset in the first decade, and in this family the disease was mild. Literature data from 104 families with 536 members dating from 1880 are tabulated. This report confirms others regarding mode of inheritance, age of onset, distribution between sexes, and disease manifestations. However, con-trary to other reports, we found the dominant and recessive form of pure Strumpell's familial spastic paraplegia to be similar in severity. There are now clinical and pathological data supporting the separation of pure Strumpell's familial spastic paraplegia from the other heredodegenerative diseases of the nervous system. Credit for the first description of familial spastic paraplegia is usually given to Strumpell (1880) although a very similar condition was described by Seeligmuller (1876) four years earlier. Strumpell's original description was of a rare hereditary dis-order characterised by progressive weakness an