progressive supranuclear palsy
E804589
Progressive supranuclear palsy is a rare neurodegenerative movement disorder characterized by early postural instability, vertical gaze palsy, and cognitive and behavioral changes due to widespread tau pathology in the brain.
Statements (58)
| Predicate | Object |
|---|---|
| instanceOf |
movement disorder
ⓘ
neurodegenerative disease ⓘ tauopathy ⓘ |
| affectsAnatomicalStructure |
basal ganglia
ⓘ
brainstem ⓘ frontal cortex NERFINISHED ⓘ globus pallidus ⓘ substantia nigra NERFINISHED ⓘ subthalamic nucleus NERFINISHED ⓘ |
| hasAbbreviation | PSP ⓘ |
| hasCause | abnormal tau metabolism ⓘ |
| hasCourse | progressive ⓘ |
| hasDiagnosticTool |
brain MRI
ⓘ
clinical examination ⓘ functional imaging (e.g., PET, SPECT) ⓘ |
| hasEpidemiology | rare worldwide ⓘ |
| hasMedianSurvival | approximately 6 to 10 years from symptom onset ⓘ |
| hasNoCure | true ⓘ |
| hasOnset | late adulthood ⓘ |
| hasPathology |
4R tauopathy
ⓘ
gliosis ⓘ neurofibrillary tangles ⓘ neuronal loss ⓘ tau protein aggregation ⓘ |
| hasPrognosis | poor ⓘ |
| hasRiskFactor |
genetic susceptibility
ⓘ
older age ⓘ |
| hasSexPredominance | slight male predominance ⓘ |
| hasSymptom |
apathy
ⓘ
axial rigidity ⓘ behavioral changes ⓘ bradykinesia ⓘ cognitive impairment ⓘ dysarthria ⓘ dysphagia ⓘ dystonia ⓘ early falls ⓘ executive dysfunction ⓘ frontal lobe syndrome ⓘ gait freezing ⓘ neck extension (retrocollis) ⓘ postural instability ⓘ pseudobulbar affect ⓘ sleep disturbances ⓘ supranuclear ophthalmoplegia ⓘ vertical gaze palsy ⓘ |
| hasTreatment |
occupational therapy
ⓘ
physiotherapy ⓘ speech therapy ⓘ supportive care ⓘ symptomatic pharmacologic therapy ⓘ |
| hasTypicalAgeOfOnset | 60s ⓘ |
| isCharacterizedBy |
early postural instability
ⓘ
frontal cognitive and behavioral changes ⓘ vertical supranuclear gaze palsy ⓘ |
| isOftenMisdiagnosedAs |
Parkinson's disease
ⓘ
atypical parkinsonism ⓘ |
| medicalCondition | rare disease ⓘ |
Referenced by (1)
Full triples — surface form annotated when it differs from this entity's canonical label.