semantic dementia
Findings
No curated finding names semantic dementia yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Semantic dementia (SD) is a form of frontotemporal dementia (FTD), characterized by the progressive, amodal and profound loss of semantic knowledge (combination of visual associative agnosia, anomia, surface dyslexia or dysgraphia and disrupted comprehension of word meaning) and behavioral abnormalities, attributable to the degeneration of the anterior temporal lobes.
Definition from the Mondo Disease Ontology (MONDO:0010857), read 2026-09-29. CC BY 4.0.
- Onset and course
- Middle age onset · Late young adult onset
HPO, annotations 2026-09-02
Features
12 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Amyotrophic lateral sclerosisHPOHP:0007354
- 8 of 10 reported patients
- Anomic aphasiaHPOHP:0030784
- Very frequent (80% to 99% of cases)
- AphasiaHPOHP:0002381
- Very frequent (80% to 99% of cases)
- Brain atrophyHPOHP:0012444
- Very frequent (80% to 99% of cases)
- Visual agnosiaHPOHP:0030222
- Very frequent (80% to 99% of cases)
- DementiaHPOHP:0000726
- 7 of 10 reported patients
- Frequent (30% to 79% of cases)
Genes
2 genes
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
Where it sits
- Narrower terms (1)
Other names
4 names
Resolves to: semantic dementia
- Also called
- dementia, frontotemporaldementia, frontotemporal, with or without parkinsonismsemantic primary progressive aphasiasemantic variant PPA