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Progress in Pediatrics
December 1942

EARLY INFANTILE "DIFFUSE SCLEROSIS" OF THE BRAIN (KRABBE'S TYPE): REPORT OF TWO CASES, WITH A REVIEW OF THE LITERATURE

Author Affiliations

THIELLS, N. Y.
From the Research Department, Letchworth Village.

Am J Dis Child. 1942;64(6):1055-1072. doi:10.1001/archpedi.1942.02010120095009
Abstract

Although published reports of diffuse sclerosis of the brain occurring in infancy and in childhood are not numerous, three types can already be recognized, which differ in age incidence, symptomatology and clinical course. The first type, described by Krabbe,1 begins before the first year of age, shows a fairly rapid clinical course, of several months, and is characterized by muscular hypertonicity and tonic fits. The second type, first described by Scholz,2 shows an age incidence between 3 and 10 years and a course extending usually over a period of a few years. Spastic paralysis, tremors, choreatic movements and epileptic attacks make up the clinical picture. Finally, a third type, described by Pelizaeus and later by Merzbacher,3 begins in early infancy and runs a chronic course extending into adulthood.

The pathologic state of the brain underlying these different clinical types is essentially the same, its main features being

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