POLIODYSTROPHIA CEREBRI PROGRESSIVA (INFANTILIS)

Abstract

DURING the last thirty years, cases of hereditary disease have occurred in which the white matter of the brain was extensively destroyed and the cerebral cortex and the gray matter were on the whole preserved. Originally, this condition was called diffuse sclerosis of the brain. The name is inappropriate, for two reasons: First, it is more natural to name a disease after its initial stage than after its cicatrizing, terminal, stage. Second, the name diffuse sclerosis of the brain is not explicit, for it has been used to designate very different conditions: Schilder's disease,¹ which is probably a chronic virus infection; extensive syphilitic sclerosis of the brain; certain forms of glioblastoma, and, finally, three types of hereditary diseases: the acute early infantile type,² the subchronic infantile type of Scholz³ and the chronic juvenile type of Pelizaeus and Merzbacher.⁴ Bielschowsky⁵ proposed the designation leukodystrophia cerebri progressiva