Diffuse Cerebellar Hypertrophy: Report of a Case

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Abstract
This report describes an additional instance of an unusual lesion of the cerebellar cortex in a 24 hour old man with symptoms, signs, and diagnostic studies indicative of a posterior fossa tumor. It involved the left cerebellar hemisphere and was subtotally removed. He has remained improved for more than one year postoperatively. Pathological examination revealed the features characteristic for the entity regarded by the authors as "diffuse cerebellar hypertrophy". These consist of diffuse or focal sites of cerebellar cortical hypertrophy, diminution of central white matter in the involved hemisphere, increased thickness and pallor of the molecular layer due to the presence of myelinated nerve fibers, a zone of abnormal neurons replacing the Purkinje and granule cell layers and calcareous deposits about vessels in the leptomeninges and molecular layer. Utilizing Woodard''s demonstration of the embryological origin of the external granule cell layer, an explanation of a possible patho-genesis of the lesion is offered.

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