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.

All labels observed (2)

Label Occurrences
multiple system atrophy canonical 3
Shy–Drager syndrome 1

How this entity was disambiguated

Statements (62)

Predicate Object
instanceOf movement disorder ⓘ
neurodegenerative disease ⓘ
rare disease ⓘ
synucleinopathy ⓘ
affectsSystem autonomic nervous system ⓘ
central nervous system ⓘ
cerebellar system ⓘ
extrapyramidal motor system ⓘ
hasAbbreviation MSA ⓘ
hasCause abnormal alpha-synuclein aggregation ⓘ
hasClinicalFeature REM sleep behavior disorder ⓘ
linked to: Parasomnias

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 ⓘ
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 ⓘ
linked to: MSA

cerebellar type multiple system atrophy ⓘ
parkinsonian type multiple system atrophy ⓘ
hasSynonym Shy–Drager syndrome ⓘ
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 ⓘ

How these facts were elicited

Referenced by (4)

Full triples — surface form annotated when it differs from this entity's canonical label.

Lewy bodies → associatedWithDisease → multiple system atrophy ⓘ
alpha-synuclein → associatedWithDisease → multiple system atrophy ⓘ
multiple system atrophy → hasSynonym → Shy–Drager syndrome ⓘ
linked to: multiple system atrophy
nigrostriatal pathway → associatedWithDisease → multiple system atrophy ⓘ