Frontotemporal dementia and parkinsonism linked to chromosome 17

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.
Usual onset	Forties or fifties.
Causes	Mutations in the MAPT gene.
Diagnostic method	Clinical criteria, molecular genetic analysis, and brain imaging.
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.
Treatment	Palliative and symptomatic interventions.
Frequency	Estimated to affect 1 in 1 million people in the Netherlands.

Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) is an autosomal dominant neurodegenerative tauopathy and Parkinson plus syndrome. 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.

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