frontotemporal dementia

Summary
Synonym
  • Wilhemsen-Lynch disease
  • frontotemporal lobar degeneration
  • multiple system tauopathy with presenile dementia
  • pallidopontonigral degeneration
Definition
A dementia characterized by progressive neuronal loss predominantly involving the frontal and/or temporal lobes of the brain resulting in a gradual and progressive decline in behavior or language.
Super Class
brain disease dementia
Related Genes
Displaying all 3 entries
Gene ID Gene Symbol Description Source
2896 GRN granulin precursor
4137 MAPT microtubule associated protein tau
25978 CHMP2B charged multivesicular body protein 2B
Displaying 1 entry
Gene ID Gene Symbol Description Source
83433 Trem2 triggering receptor expressed on myeloid cells 2
The Human Phenotype Ontology
Displaying entries 1 - 10 of 50 in total
HPO ID HPO Term
HP:0000474 Thickened nuchal skin fold
HP:0000708 Atypical behavior
HP:0000711 Restlessness
HP:0000716 Depression
HP:0000739 Anxiety
HP:0000751 Personality changes
HP:0001268 Mental deterioration
HP:0001297 Stroke
HP:0001300 Parkinsonism
HP:0002071 Abnormality of extrapyramidal motor function
Displaying 1 entry
Gene ID Gene Symbol Description
7415 VCP valosin containing protein

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Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01


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Last updated: August 4, 2025