Shy-Drager syndrome (historical)
Citation, DOI, disclosures and article data
At the time the article was created Frank Gaillard had no recorded disclosures.
View Frank Gaillard's current disclosuresAt the time the article was last revised Frank Gaillard had no financial relationships to ineligible companies to disclose.
View Frank Gaillard's current disclosuresThe Shy-Drager syndrome is now recognized as a clinical manifestation of multiple systemic atrophy, no longer considered a separate entity.
Clinical presentation
The most striking clinical finding in Shy-Drager syndrome is arterial orthostatic hypotension. This remains a core clinical feature for the diagnosis of multiple system atrophy 3. Other autonomic signs caused by degeneration of brainstem reticular formation nuclei are also present.
History and etymology
American neurologist George Milton Shy (1919-1967) and physician Glenn Albert Drager (1917-1967) described in 1960 the cases of two young men who presented with identical symptoms related to the central autonomic nervous system.
Quiz questions
References
- 1. Matsusue E, Fujii S, Kanasaki Y et-al. Cerebellar lesions in multiple system atrophy: postmortem MR imaging-pathologic correlations. AJNR Am J Neuroradiol. 2009;30 (9): 1725-30. doi:10.3174/ajnr.A1662 - Pubmed citation
- 2. Valery N. Kornienko, I.N. Pronin. Diagnostic Neuroradiology. (2008) ISBN: 9783540756521 - Google Books
- 3. Wenning G, Stankovic I, Vignatelli L et al. The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy. Mov Disord. 2022;37(6):1131-48. doi:10.1002/mds.29005 - Pubmed
Incoming Links
Related articles: Neurodegenerative diseases
Neurodegenerative diseases are legion and their classification just as protean. A useful approach is to divide them according to underlying pathological process, although even using this schema, there is much overlap and thus resulting confusion.
-
neurodegenerative MRI brain (an approach)
- measurements and ratios
- midbrain to pons area ratio (for PSP)
- Magnetic Resonance Parkinsonism Index (MRPI) (for PSP)
- frontal horn width to intercaudate distance ratio (FH/CC) (for Huntington disease)
- intercaudate distance to inner table width ratio (CC/IT) (for Huntington disease)
- signs
- hummingbird sign (of PSP)
- Mickey Mouse sign (of PSP)
- morning glory sign (of PSP)
- hot cross bun sign (of MSA-C)
- hockey stick sign (of Creutzfeldt-Jakob disease)
- pulvinar sign (of Creutzfeldt-Jakob disease)
- scoring systems
- measurements and ratios
-
neurodegenerative diseases
-
synucleinopathies
- diseases with Lewy bodies
-
multiple systemic atrophy (MSA)
- MSA-P (striatonigral degeneration)
- MSA-C (olivopontocerebellar degeneration)
- Shy-Drager syndrome
-
tauopathies
-
Alzheimer disease
- typical/classical Alzheimer disease
- variant (e.g. posterior cortical atrophy)
- chronic traumatic encephalopathy (CTE)
- corticobasal degeneration
- frontotemporal lobar degeneration (FTLD) (not all are tauopathies)
- Pick disease
- progressive supranuclear palsy (PSP)
-
Alzheimer disease
- amyloidoses
- TDP-43 proteinopathies
- spinocerebellar ataxias
- Huntington disease
- hereditary spastic paraplegia
- clinically unclassifiable parkinsonism (CUP)
- Unverricht-Lundborg disease
-
prion diseases (not always included as neurodegenerative)
- Creutzfeldt-Jakob disease (sporadic, variant, familial, and iatrogenic)
- fatal familial insomnia
- Gerstmann-Straussler-Scheinker disease
- kuru
- variably protease-sensitive prionopathy
-
synucleinopathies
Unable to process the form. Check for errors and try again.