[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).