SPASTIC PSEUDOSCLEROSIS (DISSEMINATED ENCEPHALOMYELOPATHY; CORTICOPALLIDOSPINAL DEGENERATION)

Abstract

Spastic pseudosclerosis was first recognized as a clinical entity by Creutzfeld¹and Jakob.²Characteristic symptoms are those of pyramidal and extrapyramidal involvement, muscular atrophies and a slowly evolving mental state, usually regarded at the outset as belonging to the neuroses. Anxiety, irritability and depression are early symptoms, followed by definite changes in personality. Mild sensory disturbances have been described. Three cases reported here are of unusual interest because of the familial incidence. This group, consisting of the cases of 2 brothers and a sister—1 of whom was previously described by one of us (Davison)—presented in life the clinical picture of spastic pseudosclerosis. The diagnosis was confirmed histopathologically in 2 of the cases. The fourth case in this presentation was of interest because of the sensory disturbances. REPORT OF CASES Familial Type.— Case 1.— The case of J. R., a man aged 32, a brother of S. R., was