disease 1,135 words KG: ent-dise-2364bda9
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Semantic Variant Primary Progressive Aphasia (svPPA)

Disease Info
PrevalencesvPPA accounts for approximately 20-30% of all PPA cases
Age of onsetTypically between 50-70 years, with a mean onset age of 60 years
Sex distributionEqual male-to-female ratio
ProgressionDisease progression leads to widespread cognitive and behavioral changes over 6-12 years
Loss of empathyDiminished emotional responsiveness to social cues
Food preferencesDevelopment of unusual food preferences, particularly sweet foods
Compulsive behaviorsMay include ritualistic or repetitive actions
Loss of personal awarenessDiminished concern about personal appearance or hygiene
Anterior temporal lobesMost severely affected, particularly the left temporal pole
Mesial temporal structuresHippocampal involvement in later stages
Posterior brain regionsRelatively preserved until late disease stages
TDP-43 type C inclusionsMost common pathology (approximately 75% of cases)
DatabasesOMIMOrphanetClinicalTrialsPubMed

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