Shy-Drager syndrome; multiple system atrophy with postural hypotension; parkinsonism with prominent dysautonomia
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Introduction
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
- loss of cells in:
- Lewy bodies may be seen
- gliosis
Genetics
- implicated genes: HH3R
Clinical manifestations
- early autonomic failure
- postural (orthostatic) hypotension
- urinary incontinence
- disturbances in bowel function
- impotence
- anhidrosis (inability to sweat)
- Parkinsonian manifestations
- pupil asymmetry may be present
- ataxia may be present
- laryngeal paralysis & sleep apnea may occur
- iris atrophy
- dementia is uncommon
Differential diagnosis
- other variants of multiple system atrophy
Management
- symptomatic, orthostatic hypotension most disabling initial symptom
- see treatment of orthostatic hypotension
- treatment of Parkinsonian symptoms
- centrally-acting alpha-adrenergic agonists
More general terms
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
Patient information
Shy-Drager syndrome (multiple system atrophy with postural hypotension) patient information