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Shy-Drager syndrome; multiple system atrophy with postural hypotension; parkinsonism with prominent dysautonomia
Parkinsonism plus autonomic insufficiency.
A multisystem degeneration originally described in 1960 by Shy & Drager who described neuropathologic changes in the brainstem & basal ganglia. Later, loss of autonomic neurons in the inter-mediolateral columns of the thoracic spinal cord were described by other investigators.
Epidemiology: age of onset in 50's
Pathology:
1) loss of cells in:
a) brainstem
- substantia nigra
- locus ceruleus
- olivary nuclei
- dorsal vagal nuclei
b) striatum (caudate nucleus)
c) intermediolateral columns of the thoracic spinal cord
d) cerebellum (in some cases)
2) Lewy bodies may be seen
3) gliosis
Clinical manifestations:
1) early autonomic failure
a) postural (orthostatic) hypotension
b) urinary incontinence
c) disturbances in bowel function
d) impotence
e) anhidrosis (inability to sweat)
5) Parkinsonian manifestations
a) rigidity
b) tremor
6) pupil asymmetry may be present
7) ataxia may be present
8) laryngeal paralysis & sleep apnea may occur
9) iris atrophy
10) dementia is uncommon
Genetics:
- implicated genes: HH3R
Differential diagnosis:
- other variants of multiple system atrophy
Management:
1) symptomatic, orthostatic hypotension most disabling initial symptom
2) see treatment of orthostatic hypotension
3) treatment of Parkinsonian symptoms
4) centrally-acting alpha-adrenergic agonists
Interactions
disease interactions
General
multiple system atrophy (MSA)
Properties
PATHOLOGY: neuronal loss
SITE: brainstem
intermediolateral column
Database Correlations
OMIM 146500
References
- Harrison's Principles of Internal Medicine, 13th ed.
Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 2286
- Bronstein J, In: Intensive Course in Geriatric Medicine &
Board Review, Marina Del Ray, CA, Sept 29-Oct 2, 2004
- National Institute of Neurological Disorders and Stroke (NINDS)
- NINDS Multiple System Atrophy Information Page
https://www.ninds.nih.gov/Disorders/All-Disorders/Multiple-System-Atrophy-Information-Page
- NINDS Multiple System Atrophy Fact Sheet
https://www.ninds.nih.gov/disorders/patient-caregiver-education/fact-sheets/multiple-system-atrophy