Frontotemporal dementia with parkinsonism-17
آخر تحديث 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.
الوصف
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
فقدان الذاكرة والخرف
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, مثل: Dementia due to Parkinson's disease. How common is dementia. The.
Patient information leaflet
أسباب فقدان الذاكرة والخرف
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. كـ many as half of people with Parkinson's disease مايو 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
مرض باركنسون
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. إذا 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
آفات الفص الصدغي
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 الخرف called frontotemporal dementia. The temporal lobe can be affected by an infection of the brain (encephalitis) ,.
Patient information leaflet
الأدوية والعلاج للخرف
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
الأدوية المضادة للذهان
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: الشلل الرعاش - this can cause symptoms similar to those that occur in people with باركنسون disease - for example, tremor and muscle stiffness. Akathisia - this is like a.
قراءة إضافية ومراجع
- Frontotemporal dementia with parkinsonism-17; MedlinePlus, National Library of Medicine.
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- 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.
تاريخ المقال
المقال متاح أيضًا باللغة الإنجليزية, الألمانية, إسبانية, الفرنسية, إيطالي, البرتغالية, الهندية, العبرية, العربية ,، و السويدية.
13 Nov 2023 | أحدث إصدار
1 Mar 2011 | نُشر في الأصل
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