Abstract
The term 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 but incomplete demyelination, followed by slowly progressive gliosis.1Schilder's2so-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,3which 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. Neuburger and Schob4divided diffuse scleroses into three groups: (a) blastomatous, (b) exogenous-inflammatory and (c) endogenous-degenerative. The first
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