Shy-Drager syndrome


Authors: Magdaléna Dušejovská;  Tomáš Vařeka;  Jaroslav Macášek;  Karel Hrubant;  Aleš Žák;  Miroslav Zeman
Authors‘ workplace: Univerzita Karlova v Praze, 1. lékařská fakulta, IV. interní klinika VFN
Published in: Čas. Lék. čes. 2010; 149: 225-228
Category: Case Report

Overview

Shy-Drager syndrome is a rare neurological disease with a poor prognosis causing a generalised autonomy dysfunction. The disorder is also known as multiple system atrophy, the orthostatic hypotension syndrome or Shy-McGee-Drager syndrome. Patients have mainly dysautonomic symptoms. Patients suffer from orthostatic hypotension, bradycardia, anhidrosis, failure of accommodation, sialoporia, low tears secretion, gastrointestinal dysmotility and incomplete emptying of the urinary bladder. Neuropathological examination of patient’s brains demonstrated neurodegenerative changes of the structures of central nervous system, mainly of brainstem. The Shy-Drager syndrome results from striatonigral and olivo-ponto-cerebellar atrophy and from accumulation of alpha- synuclein in these structures. The patients suffering from the Shy-Drager syndrome are very often misdiagnosed because of overlap of symptomatology with psychiatric and psychosomatic diseases. It is also very difficult to make the diagnosis because of complexity of symptoms. The prognosis of Shy-Drager syndrome is very poor; patients are markedly disabled and have shorter survival.

Key words:
Shy-Drager syndrome, multiple system atrophy, catecholamines, parkinsonian syndrome, autonomy dysfunction, orthostatic hypotension.


Sources

1. Shy M, Drager GA. A neurological syndrome associated with ortostatic hypotension: a clinical – pathologic study. Arch Neurol 1960; 2: 511–527.

2. Schatz IJ. Farewell to the „Shy-Drager syndrome“. Ann Int Med 1996; 125: 74–75.

3. Gilman S, Wenning GK, Low PA, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology 2008; 71: 670–676.

4. Vanacore N. Epidemiological evidence on multiple system atrophy. J Neural Transm 2005; 112: 1605–1612.

5. Ward C. An ethical dilemma involving a Shy-Drage patient: a case study. Journal of Neuroscience Nursing 2006; 38: 6.

6. Quinn N, Wenning GK. Multiple system atrophy. Current Opinion in Neurology 1995; 8: 323–326.

7. Benarroch EE. New finding on the neuropathology of multiple system atrophy. Autonomic Neuroscience: Basic and Clinical 2002; 96: 59–62.

8. Wenning GK, Braune S. Multiple System Atrophy. CNS Drugs 2001; 15: 839–852.

9. Mathias C J, Matthews WB, Spalding JMK. Postural changes in plasma renin activity and responses to vasoactive drugs in a case of Shy-Drager syndrome. J Neurol Neurosurg Psychiatry 1977; 40: 138–143.

10. Furlan R, Piazza S, Bevilacqua M, et al. Pure Autonomic Failure: Complex abnormalities in the neural mechanisms regulating the cardiovascular system. J Auton Nerv Syst 1995; 51: 223–235.

11. Smith GDP, Watson LP, Pavitt DV, Mathias CJ. Abnormal cardiovascular and catecholamine responses to supine exercise in human subjects with sympathetic dysfunction. J Physiol 1995; 484: 255–265.

12. Gilman S, Low PA, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci 1999; 163: 94–98.

13. Gross G, Nagel-Hiemke M, Krause KH. Plasma Catecholamines During Activation of the Sympathetic Nervous System in a Patient with Shy-Drager Syndrome. J Neurol 1980; 224: 125–131.

14. van Boekel MA, Vossenaar ER, van den Hoogen FH, van Venrooij WJ. Autoantibody systems in rheumatoid arthritis: specificity, sensitivity and diagnostic value. Arthritis Res 2002; 4: 87–93.

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