multiple system atrophy
E804586
Multiple system atrophy is a rare, progressive neurodegenerative disorder characterized by a combination of autonomic failure, parkinsonism, and/or cerebellar ataxia due to widespread nervous system damage.
Statements (62)
| Predicate | Object |
|---|---|
| instanceOf |
movement disorder
ⓘ
neurodegenerative disease ⓘ rare disease ⓘ synucleinopathy ⓘ |
| affectsSystem |
autonomic nervous system
NERFINISHED
ⓘ
central nervous system ⓘ cerebellar system ⓘ extrapyramidal motor system ⓘ |
| hasAbbreviation | MSA NERFINISHED ⓘ |
| hasCause | abnormal alpha-synuclein aggregation ⓘ |
| hasClinicalFeature |
REM sleep behavior disorder
NERFINISHED
ⓘ
autonomic failure ⓘ cerebellar ataxia ⓘ constipation ⓘ dysarthria ⓘ dysphagia ⓘ erectile dysfunction ⓘ gait disturbance ⓘ orthostatic hypotension ⓘ parkinsonism ⓘ postural instability ⓘ pyramidal signs ⓘ stridor ⓘ urinary incontinence ⓘ urinary retention ⓘ |
| hasCourse | progressive ⓘ |
| hasDiagnosticMethod |
autonomic function testing
ⓘ
brain MRI ⓘ clinical evaluation ⓘ |
| hasEpidemiology | rare ⓘ |
| hasFirstDescription | 1960s as Shy–Drager syndrome ⓘ |
| hasImagingFinding |
cerebellar atrophy
ⓘ
hot cross bun sign NERFINISHED ⓘ pontine atrophy ⓘ putaminal atrophy ⓘ |
| hasMedianSurvival | 6 to 10 years after symptom onset ⓘ |
| hasNoTreatment | disease-modifying therapy currently unavailable ⓘ |
| hasOnset | adult ⓘ |
| hasPathology |
glial cytoplasmic inclusions
ⓘ
oligodendroglial alpha-synuclein inclusions ⓘ |
| hasPrevalence | approximately 3 to 5 per 100000 people ⓘ |
| hasPrognosis | poor ⓘ |
| hasRiskFactor |
environmental factors not well defined
ⓘ
possible genetic susceptibility ⓘ |
| hasSexRatio | slight male predominance ⓘ |
| hasSubtype |
MSA-C
ⓘ
MSA-P NERFINISHED ⓘ cerebellar type multiple system atrophy ⓘ parkinsonian type multiple system atrophy ⓘ |
| hasSynonym |
Shy–Drager syndrome
NERFINISHED
ⓘ
olivopontocerebellar atrophy ⓘ striatonigral degeneration ⓘ |
| hasTreatment |
autonomic support measures
ⓘ
dopaminergic therapy with limited response ⓘ symptomatic treatment ⓘ |
| hasTreatmentGoal | symptom control ⓘ |
| hasTypicalAgeOfOnset | 50 to 60 years ⓘ |
| isClassifiedAs | orphan disease ⓘ |
| isDistinctFrom |
Parkinson's disease
ⓘ
dementia with Lewy bodies ⓘ pure autonomic failure ⓘ |
| isIncludedIn | atypical parkinsonian syndromes ⓘ |
Referenced by (1)
Full triples — surface form annotated when it differs from this entity's canonical label.