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Multiple system atrophy
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Multiple system atrophy

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Contributors: Jamie Adams MD
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Synopsis

A progressive neurodegenerative condition caused by cell loss and alpha-synuclein inclusions in the basal ganglia, brainstem, and/or cerebellum. Average age of onset is 50-55 years. Autonomic dysfunction almost always develops during the course of the disease and can include orthostatic hypotension, erectile dysfunction, urinary incontinence, or supine hypertension.

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, REM-sleep behavior disorder, respiratory stridor, dysphagia, emotional incontinence, anterocollis, and contractures. Cognition remains relatively intact.

There is usually a poor or nonsustained 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

Differential Diagnosis & Pitfalls

Best Tests

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References

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Last Updated: 03/28/2017
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Multiple system atrophy
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Multiple system atrophy (Cerebellar Type) : Ataxia, Dysarthria, Nystagmus, Orthostatic hypotension, Rigidity, Sleep disturbance, Urinary incontinence, Dysphagia
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