Shy-Drager syndrome

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

Terminology

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)

Clinical presentation

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. 

Neurodegenerative diseases

Neurodegenerative diseases are legion and their classification just as protean. A useful approach is to divide them according to underlying pathological process, although even using this schema, there is much overlap and thus resulting confusion.

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Article Information

rID: 13005
Section: Syndromes
Synonyms or Alternate Spellings:

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