Clinical spectrum of frontotemporal lobar degeneration

Autor: Paić, Marina
Přispěvatelé: Boban, Marina, Malojčić, Branko, Habek, Mario
Jazyk: chorvatština
Rok vydání: 2015
Předmět:
Popis: Frontotemporalna lobarna degeneracija (FTLD) je u dobi do 65. godine života druga najučestalija vrsta demencije uz Alzheimerovu bolest. Od Alzheimerove bolesti se razlikuje po tome što amnestički sindrom nije dominantan u ranijim stadijima bolesti. Klinički se očituje progresivnim poremećajima ponašanja i govora uzrokovanih degeneracijom u frontalnim i prednjim dijelovima temporalnih režnjeva. U oko polovine oboljelih nalazi se nasljedna podloga, što se povezuje najčešće s mutacijom MAPT gena. FTLD obuhvaća spektar klinički, patološki i genetski raznolikih neurodegenerativnih poremećaja. U zavisnosti od anatomske raspodjele patoloških promjena može se razlikovati više kliničkih sindroma: bihevioralna (frontalna) varijanta FTLD-a, progresivna nefluentna afazija, semantička demencija i dr.
Frontotemporal lobar degeneration (FTLD) is the second most common type of dementia under the age of 65, first being Alzheimer's disease. In contrast to Alzheimer's disease, amnestic syndrome is not characteristic early feature of FTLD. It's clinically characterised by a progressive decline in behaviour and language caused by degeneration of frontal and anterior temporal lobes. In about half of patients there is a hereditary basis, which is primarily associated with mutations in MAPT gene. FTLD includes a range of clinical, pathological and genetic variety of neurodegenerative disorders. Depending on the anatomical distribution of pathological lesions, multiple clinical syndromes can be distinguished: behavioural (frontal) variant frontotemporal lobar degeneration, progressive non-fluent aphasia, semantic dementia and others.
Databáze: OpenAIRE