Hereditary (Familial) Spastic Paraplegia

Electrophysiological studies in familial spastic paraplegia.

“…The inheritance was autosomal dominant in two of the families (A and B) and probably recessive in the third (C). The pathological features of the uncomplicated cases are degeneration of the corticospinal tracts and posterior column degeneration which may be associated with clinical impairment of deep sensation in later life (Strumpell, 1880(Strumpell, , 1886(Strumpell, , 1904Schwarz, 1952;Schwarz and Liu, 1956;Behan and Maia, 1974).…”

Hereditary spastic paraparesis: a review of new developments

“…Schwarz and Lui reviewed the early pathological reports in 1952, identifying seven cases of pure HSP and added their own description of cases in 1956 8124 Only a handful of pathological reports have been published since 8082-84 The major neuropathological feature of pure HSP is axonal degeneration that is maximal in the terminal portions of the longest descending (corticospinal tracts) and ascending (dorsal column pathways) tracts within the spinal cord.…”

Peripheral Nerve Involvement in Familial Spastic Paraplegia

“…Pathologically, the condition is characterized by corti¬ cospinal tract degeneration, more marked caudally, and posterior col¬ umn degeneration with a more marked involvement rostrally and without loss of the posterior root fibers.2 Betz cells may be reduced and atrophie.3 4 Recently, Behan and Maia5 have reported necropsy findings in two cases of uncomplicated familial spastic paraplegia, confirming the previously reported involvement of the corticospinal tract and posterior columns. They stressed the prominent involvement of the larger fibers in their distal part.…”