[Shy-Drager syndrome. Clinico-pathological report (author's transl)].
Presentation of a caucasian female (70-year-old) who suffered from urinary incontinence and orthostatic hypotension (Shy-Drager syndrome) and died six years after the onset of the dysautonomic clinical disorders. Neuropathological examination demonstrated putamino-nigral degeneration, olivo-ponto-cerebellar atrophy, neuronal depletion in the brain-stem and the spinal cord, mainly in the intermedio-lateral horns. The cerebral cortex did not exhibit senile alteration as often seen in ageing brain. A review of the literature, including 43 autopsied cases, confirmed that the Shy-Drager syndrome results from a diffuse neuronal loss mainly in the putamen, the pigmented nuclei, part of the brain-stem and the spinal cord (multiple system atrophy, with minimal cerebral cortex involvement). The disease occurs sporadically in adults, more frequently in man (sex ratio : 2.5/1). (Acta neurol. belg., 1980, 80, 271-286).
D000328:Adult; D000368:Aged; D001341:Autonomic Nervous System / Q000473:pathology; D001342:Autonomic Nervous System Diseases / Q000473:pathology*; D001933:Brain Stem / Q000473:pathology; D002531:Cerebellum / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D005260:Female; D005727:Ganglia, Spinal / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009420:Nervous System / Q000473:pathology*; D009847:Olivary Nucleus / Q000473:pathology; D011149:Pons / Q000473:pathology; D011699:Putamen / Q000473:pathology; D012791:Shy-Drager Syndrome / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology; D013378:Substantia Nigra / Q000473:pathology
Le syndrome de Shy-Drager. Confrontation anatomo-clinique.