disease 1,172 words KG: ent-dise-57c41ec4 2026-03-24
kind:diseasesection:diseasesstate:published
Contents

Progressive Supranuclear Palsy - Richardson Syndrome

Disease Info
Prevalence5-6 per 100,000 population[2]
Incidence0.5-1.0 per 100,000 annually[2]
Age of onsetTypically 60-65 years[2]
Disease durationMedian 6-9 years[2]
MAPT H1 haplotypeStrongest genetic risk factor[3]
C9orf72 expansionsOccasionally found in PSP-FTD spectrum[3]
Familial aggregationRare but reported in some families[3]
Substantia nigraPars compacta degeneration[4]
Globus pallidusInternal segment (GPi) involvement[4]
Subthalamic nucleusNeuronal loss[4]
Superior colliculusVertical gaze center[4]
Periaqueductal grayOculomotor control[4]
DatabasesOMIMOrphanetClinicalTrialsPubMed

Knowledge Graph