Farewell to the Shy-Drager Syndrome

  title={Farewell to the Shy-Drager Syndrome},
  author={Irwin J. Schatz},
  journal={Annals of Internal Medicine},
  • I. Schatz
  • Published 1 July 1996
  • Medicine, Psychology
  • 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… 
An immaculate misconception?
In this issue of Neurology, Kaufmann et al.1 describe two patients who presented with isolated autonomic failure; years later, one developed features of Parkinson disease and the other features of
Pure autonomic failure
In this issue of Neurology , Kaufmann et al.1 describe two patients who presented with isolated autonomic failure; years later, one developed features of Parkinson disease and the other features of
Different phenoconversion pathways in pure autonomic failure with versus without Lewy bodies
Clinical and postmortem data show that the form of PAF that involves sympathetic intraneuronal synucleinopathy and noradrenergic deficiency can phenoconvert to PD or DLB—but not to MSA; Conversely, PAF without these features leaves open the possibility of premotor MSA.
Sympathetic cardioneuropathy in dysautonomias.
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.
From orthostatic hypotension to Shy-Drager syndrome
  • J. Pearce
  • Medicine
    Journal of Neurology, Neurosurgery & Psychiatry
  • 2004
A pioneer of percussion and pleximetry, best remembered for his work in chest diseases and for coining the term “uraemia”, postural syncope in adrenal failure is described.
Familial mydriasis, cardiac arrhythmia, respiratory failure, muscular weakness and hypohidrosis
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).
Cerebrospinal fluid levels of catechols in patients with neurogenic orthostatic hypotension.
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.
Novel Therapeutic Strategy Against Central Baroreflex Failure
The results suggest the feasibility of a BBS approach for central baroreflex failure and evaluated the performance of the BBS in response to rapid-progressive hypotension secondary to sudden sympathetic withdrawal evoked by the local imposition of a pressure step on carotid sinus baroreceptors in 16 anesthetized rats.
Association Between Supine Hypertension and Orthostatic Hypotension in Autonomic Failure
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.


Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy.
A detailed post-mortem examination of one of their cases showed neuronal degeneration at many sites, including the intermediolateral columns of the spinal cord, but they did not comment on the relation of this finding to the autonomic features of their patient's disorder.
A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study.
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
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].
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
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.
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
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.
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
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