Frontotemporal dementia and parkinsonism linked to chromosome 17
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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]
Quick Facts Other names, Specialty ...
Frontotemporal dementia and parkinsonism linked to chromosome 17 | |
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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] |
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