Farewell to the Shy-Drager Syndrome

@article{Schatz1996FarewellTT,
  title={Farewell to the Shy-Drager Syndrome},
  author={Irwin J. Schatz},
  journal={Annals of Internal Medicine},
  year={1996},
  volume={125},
  pages={74-75}
}
  • I. Schatz
  • Published 1 July 1996
  • Medicine
  • Annals of Internal Medicine
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… 
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Shy-Drager Syndrome
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It is important to note that the existing cases were redefined as Multiple System Atrophy with autonomic involvement, although the term ‘Shy–Drager syndrome’ is still used occasionally when the primary symptom is severe autonomic failure.
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Analysis of sympathetic innervation of the heart in patients with acquired, idiopathic dysautonomias using thoracic positron-emission tomography and assessments of the entry rate of the sympathetic neurotransmitter norepinephrine into the cardiac venous drainage found signs of sympathetic neurocirculatory failure and responsiveness to treatment with levodopa–carbidopa.
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Conceivably, this disorder is close to, but still not identical with E‐D, although the autonomic dysfunction pattern seems to differ somewhat from that of progressive autonomic failure (Shy‐Drager syndrome).
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In vivo neurochemical evidence for central noradrenergic deficiency in patients with OH due to multiple system atrophy or pure autonomic failure is sought, and findings are consistent with central Noradrenaline deficiency in both MSA+OH and PAF.
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Background—Central baroreflex failure in Shy-Drager syndrome and traumatic spinal cord injuries results in severe orthostatic hypotension and often confines the patient to the bed. We proposed a
Association Between Supine Hypertension and Orthostatic Hypotension in Autonomic Failure
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The finding of lower plasma NE levels in patients with than without supine hypertension suggests involvement of pressor mechanisms independent of the sympathetic nervous system.
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References

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TLDR
Evidence is provided that the basic disease is a primary atrophy of a selection of neurones in the CNS, and the features of sympathetic failure, including orthostatic hypotension, are attributable to cell loss in the intermediolateral columns; nicotine sensitivity is associated with cerebellar system disorders, which are present in some cases of the Shy-Drager syndrome.
A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study.
TLDR
Patients with idiopathic orthostatic hypotension may show wide swings in blood pressure, but do not have the pulse changes or symptoms, such as yawning, nausea, or increased sweating, associated with other types of syncope in which the nervous system is intact.
Circulatory Studies in Wernicke's Encephalopathy: With Special Reference to the Occurrence of a State of High Cardiac Output and Postural Hypotension
TLDR
This study describes the postural response to tilting in 12 patients and the determination of the cardiac output in 7 patients, as determined by the Evans blue dye-dilution technic.
[Shy-Drager syndrome and multiple system atrophy].
TLDR
The features favoring a clinical diagnosis of Shy-Drager syndrome are marked orthostatic hypotension, erectile impotence in males, urinary symptoms, nocturnal stridor, rigidity and akinesia without tremors.
Differentiation of multiple system atrophy from idiopathic Parkinson's disease using proton magnetic resonance spectroscopy
TLDR
In vivo quantification of absolute metabolite concentrations was possible and confirmed an absolute reduction of choline‐containing compounds and NAA in the MSA group compared with controls with no significant difference in the creatine concentrations, which probably reflects neuronal loss, occurring predominantly in the putamen.
Late systolic murmurs and non-ejection ("mid-late") systolic clicks. An analysis of 90 patients.
TLDR
An analysis of 90 subjects with either a late systolic murmur, a nonejection click, or both is presented and the intracardiac origin of these murmurs and clicks is reaffirmed and their possible mode of production is considered.
Positron emission tomography studies on the dopaminergic system and striatal opioid binding in the olivopontocerebellar atrophy variant of multiple system atrophy
TLDR
It is suggested that subclinical nigrostriatal dysfunction is present in the majority of patients with sporadic olivopontocerebellar atrophy, in accordance with it being part of the spectrum of multiple system atrophy.
Isolated failure of autonomic noradrenergic neurotransmission. Evidence for impaired beta-hydroxylation of dopamine.
TLDR
The autonomic disorder is associated with central nervous system damage in both the sympathetic and the parasympathetic systems, and the use of statins to treat the latter is a natural application.
Mendelian Inheritance in Man. Catalogs of Autosomal Dominant, Autosomal Recessive, and X-linked Phenotypes
TLDR
The book succeeds admirably in its object and there can be few geneticists who would not find it of the utmost value in understanding the very stuff of their science.
Orthostatic hypotension as a predictor of two year mortality in elderly men: The Honolulu Heart Program
  • Clin Auton Res
  • 1995