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[Shy-Drager syndrome (multisystem atrophy with progressive autonomic insufficiency). Clinicopathologic study of a
1Unità Operativa di Neurologia-Neurofisiopatologia, Presidio Ospedaliero della USL 23 di Arezzo.
Abstract:
The clinical and neuropathological findings in a case of Shy-Drager syndrome in a 56-years-old woman are reported. The patient showed slowly progressive autonomic dysfunctions and additional non-autonomic abnormalities, including pyramidal, extrapyramidal and cerebellar features. The neuropathological examination revealed striatonigral degeneration and olivopontocerebellar atrophy; neuronal loss and reactive gliosis were found also in the pigmented nuclei of the brainstem and in the spinal cord, mainly in the intermediolateral columns. The nosological place of the Shy-Drager syndrome in systemic degenerative diseases is pointed out, and the possible etiopathogenetic mechanisms are briefly discussed.