Farewell to the “Shy-Drager Syndrome”
Authors: Irwin J. Schatz
Journal: Annals of Internal Medicine (1996)

Editorials1 July 1996Farewell to the "Shy-Drager Syndrome"Irwin J. Schatz, MDIrwin J. Schatz, MDUniversity of Hawaii, Honolulu, HI 96813-2427Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-125-1-199607010-00012 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail A recent consensus statement generated by the American Autonomic Society and the American Academy of Neurology [1] defines the various primary neurogenic causes of autonomic dysfunction. Implicit in this document is the need to bid good-bye to the use of the term "Shy-Drager syndrome" to identify a condition that was first described in 1962 [2].Milton Shy and Glen Drager detailed the clinical features of two patients who had both orthostatic hypotension and central nervous system signs, and they carefully studied the neuropathologic changes in one of these patients [2]. Their conclusion that orthostatic hypotension and central nervous system dysfunction ...References1. Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. Neurology. 1996; 46:1470. Google Scholar2. Shy M, Drager GA. A neurological syndrome associated with orthostatic hypotension. Arch Neurol. 1960; 3:511-27. Google Scholar3. Hammarstrom S, Lindgren AG. Postural hypotension in a patient with multiple encephalomalacias. Acta Med Scand. 1942; 3:537-54. Google Scholar4. Gravallese MA Jr, Victor M. Circulatory studies in Wernicke's encephalopathy: with special reference to the occurrence of high cardiac output and postural hypotension. Circulation. 1957; 15:836-44. Google Scholar5. Sandroni P, Ahlskog E, Fealey RD, Low PA. Autonomic involvement in extrapyramidal and cerebellar disorders. Clin Auton Res. 1991; 1:147-55. Google Scholar6. Mathias CJ, Williams AC. The Shy Drager syndrome and multiple system atrophy. In: Calne DB, ed. Neurodegenerative Diseases. Philadelphia: WB Saunders; 1994:743-67. Google Scholar7. Graham JG, Oppenheimer DR. Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy. J Neurol Neurosurg Psychiatry. 1969; 32:28-34. Google Scholar8. Barlow JB, Bosman CK, Pocock WA, Marchand P. Late systolic murmurs and non-ejection (mid-late) clicks. An analysis of 90 patients. Br Heart J. 1968; 30:203-18. Google Scholar9. Roberts L, Goodin RR. Barlow syndrome. J Ky Med Assoc. 1973; 71:667-70. Google Scholar10. Robertson D, Goldberg MR, Onrot J, Hollister AS, Wiley R, Thompson JG Jr, et al. Isolated failure of autonomic noradrenergic neurotransmission. Evidence for impaired β-hydroxylation of dopamine. N Engl J Med. 1986; 314:1494-7. Google Scholar11. McKusick VA, Francomano CA, Antonarakis SE. Mendelian Inheritance in Man: Catalogs of Autosomal Dominant, Autosomal Recessive, and X-Linked Phenotypes. 9th ed. Baltimore: Johns Hopkins Univ Pr; 1990: xxv. Google Scholar12. Schatz IJ, Masaki KH, Burchfiel CM, Curb JD, Chiu D. Orthostatic hypotension as a predictor of two year mortality in elderly men: The Honolulu Heart Program. Clin Auton Res. 1995; 5:321. Google Scholar13. Davie CA, Wenning GK, Barker GJ, Tofts PS, Kendall BE, Quinn N, et al. Differentiation of multiple system atrophy from idiopathic Parkinson's disease using proton magnetic resonance spectroscopy. Ann Neurol. 1995; 37:204-10. Google Scholar14. Rinne JO, Burn DJ, Mathias CJ, Quinn NP, Marsten CD, Brooks NJ. Positron emission tomography studies on the dopaminergic system and striatal opioid binding in the olivopontocerebellar atrophy variant of multiple system atrophy. Ann Neurol. 1995; 37:568-73. Google Scholar Author, Article, and Disclosure InformationAffiliations: University of Hawaii, Honolulu, HI 96813-2427Corresponding Author: Irwin J. Schatz, MD, University of Hawaii, Department of Medicine, 1356 Lusitana Street, 7th Floor, Honolulu, HI 96813. 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