Frontotemporal dementia and parkinsonism linked to chromosome 17 information
Medical condition
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]
^ abcd"Frontotemporal dementia with parkinsonism-17: MedlinePlus Genetics". MedlinePlus. March 1, 2017. Retrieved November 2, 2023.
^ abcWszolek, 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. doi:10.1186/1750-1172-1-30. ISSN 1750-1172. PMC 1563447.
^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.
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