Congenital Pick cell encephalopathy: a distinct disorder characterized by diffuse formation of Pick cells in the cerebral cortex.
Diffuse degeneration of the cerebral cortex and claustrum was found in the brain of a 7-week-old baby with profound psychomotor retardation, multiple ankyloses, seizures, and hypothalamic dysfunction. There was ubiquitous Pick cell formation and gliosis in the affected gray matter. The cortex was not atrophic; in fact, the brain was moderately enlarged. The clinical and pathological findings suggest that the disorder should be distinguished from Pick's disease, as well as from other congenital encephalopathies.
D002540:Cerebral Cortex / Q000473:pathology* / Q000648:ultrastructure; D003704:Dementia / Q000151:congenital* / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008854:Microscopy, Electron; D009132:Muscles / Q000473:pathology