Shy-Drager syndrome

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Classification according to ICD-10
G23.8 Multisystem atrophy
- Shy-Drager syndrome (neurogenic orthostatic hypotension with multisystem atrophy)
ICD-10 online (WHO version 2019)

The Shy-Drager syndrome is a so-called multiple system atrophy of the central nervous system , as more functional systems degenerate. Other names are: neurogenic orthostatic hypotension or primary orthostatic hypotension . The focus is on the disturbance of the autonomic nervous system . Because of Parkinson's-like movement disorders, it is also counted among the Parkinson-plus syndromes . The cause is unknown. The disease was described in 1960 by G. Milton Shy (1919-1967) and Glenn Drager (1917-1967). Overall, the term Shy-Drager syndrome should no longer be used today. Instead, the syndrome is counted among the multiple system atrophies with Parkinson's symptoms.

Epidemiology

The disease is comparatively rare. It usually begins in middle to older adulthood. Men get sick significantly more often.

Symptoms

The first symptoms are often orthostatic hypotension when standing up, which is not counter-regulated by increasing the pulse rate. As a result, dizziness , light-headedness and impaired vision occur more frequently , especially when standing. In the further course, vegetative deficits such as the loss of sweating ( anhidrosis ), impotence and impaired bladder and rectal function ( urinary or faecal incontinence ) occur, but also failure symptoms of other systems: Parkinson-like movement disorders , paralysis of eye muscles ( ophthalmoplegia ), muscle weakness and - wasting as well as cerebellar symptoms . A dementia is not part of the clinical picture.

forecast

The disease is currently not curable. Nor can progress be effectively stopped. The symptoms can be alleviated with sympathomimetics and Parkinson's medication ( levodopa ). The mean survival time after the first symptoms appear is several years.

literature

  • K. Poeck, W. Hacke: Neurology. 13th edition, Springer, 2010, ISBN 3-642-12381-3 , pp. 540-541.
  • T. Klockgether: Ataxic diseases. In: Neurologist. Volume 76, 2005, pp. 1275-1285.