Multiple system atrophy
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Synopsis
Some patients may present with predominantly parkinsonian symptoms including bradykinesia, rigidity, tremor, and/or postural instability (parkinsonian type multiple system atrophy [MSA-P]). Others present with predominantly cerebellar signs including ataxia, dysarthria, and oculomotor abnormalities (cerebellar type MSA [MSA-C]). There may also be hyperreflexia, extensor plantar responses, myoclonus, orofacial dystonia, rapid eye movement (REM)-sleep behavior disorder, respiratory stridor, dysphagia, emotional incontinence, Raynaud phenomenon (cold hands / feet), anterocollis, and contractures. Early onset of falling is common. Cognition remains relatively intact.
There is usually a poor or unsustained response to levodopa. Rapid progression is common and prognosis is poor; death typically occurs within 10 years of symptom onset.
Codes
ICD10CM:G90.3 – Multi-system degeneration of the autonomic nervous system
SNOMEDCT:
230297002 – Multiple system atrophy
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Differential Diagnosis & Pitfalls
- Parkinson disease
- Progressive supranuclear palsy
- Corticobasal syndrome
- Dementia with Lewy bodies
- Vascular parkinsonism
- Spinocerebellar ataxia
- Pure autonomic failure
- Fragile X-associated tremor ataxia syndrome
- Wilson disease
- Neurodegeneration with brain iron accumulation
- Neuroacanthocytosis
- Neurosyphilis
- Neurosarcoidosis (see sarcoidosis)
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Last Reviewed:01/22/2019
Last Updated:09/28/2021
Last Updated:09/28/2021
Multiple system atrophy