CONGENITAL DEMYELINATING ENCEPHALOPATHY

Abstract

The term diffuse sclerosis has been used to cover a wide variety of pathologic conditions. Pelizaeus-Merzbacher "disease," arising late in childhood and apparently familial, is characterized by diffuse but incomplete demyelination, followed by slowly progressive gliosis.¹ Schilder's² so-called encephalitis periaxialis diffusa, on the other hand, occurs in infancy or early childhood, is not familial and runs a much more rapid course. The demyelination is more acute and complete and the gliosis less profound, being proportional to the chronicity of the process. A third type was described by Krabbe,³ which resembles Schilder's disease but is familial and leads to more marked gliosis. The literature contains the reports of many cases, each differing in some respect from the others, and the task of bringing order out of chaos seems insuperable. Neubürger and Schob⁴ divided "diffuse scleroses" into three groups: (a) blastomatous, (b) exogenous-inflammatory and (c) endogenous-degenerative. The first