[Lhermitte-Duclos disease. Case report and review of the literature].

Abstract

A new case of Lhermitte-Duclos disease was recognized as an accidental finding during the autopsy of a 58-year-old male who had died because of acute enteritis and shock-induced cardiac failure. Gross examination revealed focally disturbed cerebellar cortical structure in two foci. Microscopical investigation resulted in findings typical of Lhermitte-Duclos disease: 1. Broadened molecular layer containing myelinated axonal projections; 2. Disappearance of Purkinje cells; 3. Progressive hypertrophy of the granular cells within the granular layer; 4. Loss of the central white matter core of the cerebellar folia. The etiology and pathogenesis of the lesion is still unclear. With this first report in Hungarian the authors wish to call the attention to this rare disease.