Frontotemporal dementia with parkinsonism-17
Dernière mise à jour 13 nov. 2023
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Frontotemporal dementia with parkinsonism-17 (FTDP-17) is a brain disorder. It is part of a group of conditions, called frontotemporal dementia or frontotemporal degeneration, that are characterised by a loss of nerve cells (neurons) in areas of the brain called the frontal and temporal lobes. Over time, a loss of these cells can affect personality, behaviour, language, and movement.
Description
Frontotemporal dementia with parkinsonism-17 (FTDP-17) is a brain disorder. It is part of a group of conditions, called frontotemporal dementia or frontotemporal degeneration, that are characterised by a loss of nerve cells (neurons) in areas of the brain called the frontal and temporal lobes. Over time, a loss of these cells can affect personality, behaviour, language, and movement.
The signs and symptoms of FTDP-17 usually become noticeable in a person's forties or fifties. Most affected people survive 5 to 10 years after the appearance of symptoms, although a few have survived for two decades or more.
Changes in personality and behaviour are often early signs of FTDP-17. These changes include a loss of inhibition, inappropriate emotional responses, restlessness, neglect of personal hygiene, and a general loss of interest in activities and events. The disease also leads to deterioration of cognitive functions (dementia), including problems with judgement, planning, and concentration. Some people with FTDP-17 develop psychiatric symptoms, including obsessive-compulsive behaviors, strongly held false beliefs (delusions), and false perceptions (hallucinations). It may become difficult for affected individuals to interact with others in a socially appropriate manner. They increasingly require help with personal care and other activities of daily living.
Many people with FTDP-17 develop problems with speech and language. They may have trouble finding words, confuse one word with another (semantic paraphasias), and repeat words spoken by others (echolalia). Difficulties with speech and language worsen over time, and most affected individuals eventually lose the ability to communicate.
FTDP-17 is also characterised by problems with movement that worsen over time. Many affected individuals develop features of parkinsonism, including tremors, rigidity, and unusually slow movement (bradykinesia). As the disease progresses, most affected individuals become unable to walk. Some people with FTDP-17 also have restricted up-and-down eye movement (vertical gaze palsy) and rapid abnormal movements of both eyes (saccades).
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal dominant. More than one pattern can apply in different cases.
Related genes
Other names
DDPAC
Disinhibition-dementia-parkinsonism-amytrophy complex
Familial Pick's disease
FTDP-17
Wilhelmsen-Lynch disease
Related patient information and medicines
Patient information leaflet
Perte de mémoire et démence
Lewy body dementia This is a type of dementia caused by lumps of protei... Frontotemporal dementia This is a type of dementia that causes the frontal and temporal lobes of the brain to shrink and stop working. Other types of dementia There are many other types of dementia, tel que : Dementia due to Parkinson's disease. How common is dementia. The.
Patient information leaflet
Causes de la perte de mémoire et de la démence
It is estimated that in 2019, there were almost 885,000 people aged 65 years and over living with dementia in the UK. Parkinson's disease. As beaucoup as half of people with Parkinson's disease peut develop dementia. Risk factors for cardiovascular disease ( angina , he... If Lewy bodies develop in a part of the brain called the brain stem, as well as.
Patient information leaflet
maladie de Parkinson
Parkinson's disease is a progressive neurological degeneration of certain cells (called dopaminergic cells) within a specific pa... About half of people with Parkinson's disease develop dementia at some stage. Si dementia occurs, it tends to develop in older people with PD (aged over 70). Early dementia in younger people with PD virtually never.
Patient information leaflet
Lésions du lobe temporal
Brain tumours may affect th... A serious head injury or a surgical operation to remove a brain tumour may may also cause damage or injury to the temporal lobe. Progressive worsening of language can be part of a type of démence called démence frontotemporale. The temporal lobe can be affected by an infection of the brain (encephalitis) ,.
Patient information leaflet
Médicaments et traitements pour la démence
In the UK, the National Institute for Health and Care Excellence (NICE) recommends that donepezil, galantamine and rivastigmine can be considered as treatment options for people with mild or moderate Alzheimer's disease, providing that: The medicine is started based on the advice given by a specialist in the care of people with dementia - after.
Patient information leaflet
Médicaments antipsychotiques
This appears to be a particular problem with some of the atypical antipsychotics - notably, clozapine and olanzapine. Movement disorders develop in some cases. These include: Parkinsonisme - this can cause symptoms similar to those that occur in people with médicaments pour la maladie de Parkinson disease - for example, tremor and muscle stiffness. Akathisia - this is like a.
Lectures complémentaires et références
- Frontotemporal dementia with parkinsonism-17; MedlinePlus, National Library of Medicine.
- Boeve BF, Hutton M. Refining frontotemporal dementia with parkinsonism linked to chromosome 17: introducing FTDP-17 (MAPT) and FTDP-17 (PGRN). Arch Neurol. 2008 Apr;65(4):460-4. doi: 10.1001/archneur.65.4.460.
- Cohn-Hokke PE, Elting MW, Pijnenburg YA, van Swieten JC. Genetics of dementia: update and guidelines for the clinician. Am J Med Genet B Neuropsychiatr Genet. 2012 Sep;159B(6):628-43. doi: 10.1002/ajmg.b.32080. Epub 2012 Jul 19.
- Hutton M, Lendon CL, Rizzu P, Baker M, Froelich S, Houlden H, Pickering-Brown S, Chakraverty S, Isaacs A, Grover A, Hackett J, Adamson J, Lincoln S, Dickson D, Davies P, Petersen RC, Stevens M, de Graaff E, Wauters E, van Baren J, Hillebrand M, Joosse M, Kwon JM, Nowotny P, Che LK, Norton J, Morris JC, Reed LA, Trojanowski J, Basun H, Lannfelt L, Neystat M, Fahn S, Dark F, Tannenberg T, Dodd PR, Hayward N, Kwok JB, Schofield PR, Andreadis A, Snowden J, Craufurd D, Neary D, Owen F, Oostra BA, Hardy J, Goate A, van Swieten J, Mann D, Lynch T, Heutink P. Association of missense and 5'-splice-site mutations in tau with the inherited dementia FTDP-17. Nature. 1998 Jun 18;393(6686):702-5. doi: 10.1038/31508.
- Ludolph AC, Kassubek J, Landwehrmeyer BG, Mandelkow E, Mandelkow EM, Burn DJ, Caparros-Lefebvre D, Frey KA, de Yebenes JG, Gasser T, Heutink P, Hoglinger G, Jamrozik Z, Jellinger KA, Kazantsev A, Kretzschmar H, Lang AE, Litvan I, Lucas JJ, McGeer PL, Melquist S, Oertel W, Otto M, Paviour D, Reum T, Saint-Raymond A, Steele JC, Tolnay M, Tumani H, van Swieten JC, Vanier MT, Vonsattel JP, Wagner S, Wszolek ZK; Reisensburg Working Group for Tauopathies With Parkinsonism. Tauopathies with parkinsonism: clinical spectrum, neuropathologic basis, biological markers, and treatment options. Eur J Neurol. 2009 Mar;16(3):297-309. doi: 10.1111/j.1468-1331.2008.02513.x.
- Rosso SM, Donker Kaat L, Baks T, Joosse M, de Koning I, Pijnenburg Y, de Jong D, Dooijes D, Kamphorst W, Ravid R, Niermeijer MF, Verheij F, Kremer HP, Scheltens P, van Duijn CM, Heutink P, van Swieten JC. Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study. Brain. 2003 Sep;126(Pt 9):2016-22. doi: 10.1093/brain/awg204. Epub 2003 Jul 22.
- Spillantini MG, Van Swieten JC, Goedert M. Tau gene mutations in frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). Neurogenetics. 2000 Mar;2(4):193-205. doi: 10.1007/pl00022972.
- van Swieten J, Spillantini MG. Hereditary frontotemporal dementia caused by Tau gene mutations. Brain Pathol. 2007 Jan;17(1):63-73. doi: 10.1111/j.1750-3639.2007.00052.x.
- van Swieten JC, Stevens M, Rosso SM, Rizzu P, Joosse M, de Koning I, Kamphorst W, Ravid R, Spillantini MG, Niermeijer, Heutink P. Phenotypic variation in hereditary frontotemporal dementia with tau mutations. Ann Neurol. 1999 Oct;46(4):617-26. doi: 10.1002/1531-8249(199910)46:43.0.co;2-i.
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