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February 1967

The Orthostatic Hypotension Syndrome of Shy-Drager: A Clinicopathologic Report

Author Affiliations

From the Department of Neurologv, School of Medicine, University of Pennsylvania, Philadelphia.

Arch Neurol. 1967;16(2):123-139. doi:10.1001/archneur.1967.00470200011002

CHRONIC orthostatic hypotension may develop in the course of a number of different neurologic diseases.1-3 Among others, we have encountered it in multiple sclerosis and in a high cervical calcified hematomyelia. Shy and Drager4 directed attention to a progressive neurologic disorder in which orthostatic hypotension was a significant feature along with urinary and rectal incontinence, anhidrosis, iris atrophy, external ophthal-moparesis, rigidity, tremor, loss of associated movements, impotence, atonic bladder, generalized weakness, fasciculations, and neuropathic muscle wasting. They demonstrated pathologic changes in the autonomic nervous system, the corticobulbar and corticospinal tracts, certain of the basal ganglia, and portions of the cerebellar system in one of their patients. They concluded that the progressive clinical course of the disease and the extensive neuropathologic changes indicated that this was a primary degenerative disease of the central nervous system (CNS). Subsequent reports5,6 have helped to fortify their idea and to

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