Shy-Drager syndrome

Last revised by Yaïr Glick on 8 Jun 2022

The Shy-Drager syndrome is a neurodegenerative disease, and one of the manifestations of multiple systemic atrophy (MSA).

It is important to note that the current belief that olivopontocerebellar degeneration, Shy-Drager syndrome and striatonigral degeneration are different manifestations of the same underlying disease, namely multiple systemic atrophy (MSA), is recent, and as such many older publications will describe these as separate entities 1-2.  

For a discussion of epidemiology and pathology, please refer to multiple systemic atrophy (MSA)

The principle clinical finding in Shy-Drager syndrome is arterial orthostatic hypotension. Other autonomic signs caused by degeneration of brainstem reticular formation nuclei are also present.

American neurologist George Milton Shy (1919-1967) and physician Glenn Albert Drager (1917-1967) described in 1960 the cases of two young men who presented with identical symptoms, related to the central autonomic nervous system.

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