Abstract Summary Frontotemporal Dementia
Frontotemporal dementia is characterised by progressive behavioural disturbance, aphasia, and a decline in frontal cognitive functions.The diagnosis is made according to the Lund and Manchester criteria, supported structural imaging and psychometric testing. With the aid of functional imaging it is currently possible to diagnose patients in relatively early stages of the disease.A familial form with an autosomal dominant pattern of inheritance is seen in 20 percent of patients. As there is still no cure for this disease, therapeutic intervention is aimed primarily at symptomatic alleviation of behavioural disturbances. Information regarding diagnosis and prognosis is important for family members, as is genetic counselling in familial cases.
Keywords: Frontotemporal dementia, progressive aphasia, semantic dementia, Pick’s disease, epidemiology, diagnosis
Samenvatting
Frontotemporale dementie wordt gekenmerkt door progressieve gedragsveranderingen, fatische stoornissen en uitval van frontale cognitieve functies. De Lund en Manchester criteria bieden een richtlijn bij het stellen van de diagnose, waarbij aanvullende diagnostiek in de vorm van beeldvorming en neuropsychologisch onderzoek de diagnose kunnen ondersteunen. Met functionele beeldvorming is het mogelijk de diagnose met redelijke zekerheid reeds in een vroeg stadium te stellen. Een erfelijke vorm met een autosomaal dominant overervingspatroon is aanwezig in ongeveer 20% van de patiënten. Therapeutische mogelijkheden ontbreken, behoudens symptomatische medicamenteuze behandeling van gedragsveranderingen. Duidelijkheid omtrent diagnose en prognose is belangrijk, alsook uitleg over mogelijke erfelijke factoren.
Sleutelwoorden: Frontotemporale dementie, progressieve afasie, semantische dementie, ziekte van Pick, epidemiologie, diagnositiek
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Afdeling Neurologie, Erasmus MC Rotterdam.
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Rosso, M.S., van Swiete, J.C. Frontotemporale Dementie. TVVG 31, 50–55 (2006). https://doi.org/10.1007/BF03075142
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DOI: https://doi.org/10.1007/BF03075142