Developmental Neuropathology

pp 64-75

Lesions of Basal Ganglia, Brain Stem and Cord

  • Reinhard L. FriedeAffiliated withCase Western Reserve UniversityUniversity of Zurich

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Marbled state of the basal ganglia was described first by Anton (1893) for a 9-year-old choreo-athetotic boy in whom abnormal whitish areas and interspaced irregular zones of gray matter were found in the posterior halves of both putamina. Microscopic examination showed scar tissue containing myelinated nerve fibers. Anton concluded that the lesions were residual to vascular softenings dating to the first year of life, and were the anatomical substrate of the chorea. Vogt and Vogt (1920, 1926) and their co-workers reported several cases of the disease, which they considered a congenital selective malformation of the striatum; they introduced the terms “état marbré” or “status marmoratus”. Their interpretation was not sustained in subsequent clinico-pathological studies in which the relation between marbled state and complicated birth or postnatal disease processes was documented (Norman, 1947; Malamud, 1950). Scholz (1924) had emphasized the correspondence between areas of abnormal myelin staining and zones of glial scarring.