Frontotemporal dementia and parkinsonism linked to chromosome 17
| Frontotemporal dementia and parkinsonism linked to chromosome 17 | |
|---|---|
| Other names | FTDP-17, Frontotemporal dementia with parkinsonism-17, Familial Pick's disease, Wilhelmsen-Lynch disease. |
| This condition is inherited in an autosomal dominant manner. | |
| Specialty | Neurology |
| Symptoms | Loss of inhibition, inappropriate emotional responses, restlessness, neglect of personal hygiene, dementia, hallucinations, delusions, Parkinson's-like features, semantic paraphasias, and echolalia.[1] |
| Usual onset | Forties or fifties.[1] |
| Causes | Mutations in the MAPT gene.[1] |
| Diagnostic method | Clinical criteria, molecular genetic analysis, and brain imaging.[2] |
| Differential diagnosis | Pick's disease, sporadic progressive supranuclear palsy, corticobasal degeneration, Parkinson-plus syndromes, dementia with Lewy bodies, Parkinson's disease, and multiple system atrophy.[2] |
| Treatment | Palliative and symptomatic interventions.[2] |
| Frequency | Estimated to affect 1 in 1 million people in the Netherlands.[1] |
Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) is an autosomal dominant neurodegenerative tauopathy and Parkinson plus syndrome.[3] FTDP-17 is caused by mutations in the MAPT (microtubule associated protein tau) gene located on the q arm of chromosome 17, and has three cardinal features: behavioral and personality changes, cognitive impairment, and motor symptoms. FTDP-17 was defined during the International Consensus Conference in Ann Arbor, Michigan, in 1996.[4]
- ^ a b c d "Frontotemporal dementia with parkinsonism-17: MedlinePlus Genetics". MedlinePlus. March 1, 2017. Retrieved November 2, 2023.
- ^ a b c Wszolek, Zbigniew K; Tsuboi, Yoshio; Ghetti, Bernardino; Pickering-Brown, Stuart; Baba, Yasuhiko; Cheshire, William P (August 9, 2006). "Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17)". Orphanet Journal of Rare Diseases. 1 (1). Springer Science and Business Media LLC: 30. doi:10.1186/1750-1172-1-30. ISSN 1750-1172. PMC 1563447. PMID 16899117.
- ^ Mitra K, Gangopadhaya PK, Das SK (Jun 2003). "Parkinsonism plus syndrome—a review". Neurology India. 51 (2): 183–8. PMID 14570999.
- ^ Boeve, Bradley F.; Hutton, Mike (1 April 2008). "Refining Frontotemporal Dementia With Parkinsonism Linked to Chromosome 17: Introducing FTDP-17 (MAPT) and FTDP-17 (PGRN)". Archives of Neurology. 65 (4): 460–464. doi:10.1001/archneur.65.4.460. ISSN 0003-9942. PMC 2746630. PMID 18413467.