Citation, DOI & article data
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.
History and etymology
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.
- 1. Matsusue E, Fujii S, Kanasaki Y et-al. Cerebellar lesions in multiple system atrophy: postmortem MR imaging-pathologic correlations. AJNR Am J Neuroradiol. 2009;30 (9): 1725-30. doi:10.3174/ajnr.A1662 - Pubmed citation
- 2. Kornienko VN, Pronin IN. Diagnostic Neuroradiology. Springer Verlag. (2008) ISBN:3540756523. Read it at Google Books - Find it at Amazon