Encephalitis Periaxialis Diffusa with Survival for Thirty-Six Years

Abstract

In 1928 Symonds (1928) recorded a case of Schilder's encephalitis, the disease being remarkable for its familial incidence and the presence of a progressive double hemiplegia. Since this date other writers have fully confirmed his observations.Familial forms of Schilder's Encephalitis have been described by Ferraro (1928) and Van Bogaert and Nyssen (1936) while Bouman (1934) has emphasized that in children quadriplegia is the usual clinical finding.The present report deals with the clinical and pathological findings in a girl who was the second of thirteen children in the family studied by Symonds. Clinically, the case was remarkable for the very long period of survival and pathologically, by reason of the extensive degree of spinal cord degeneration with but little evidence of sclerosis in the cerebral hemispheres.