{"?xml":{"@version":"1.0"},"edm:RDF":{"@xmlns:dc":"http://purl.org/dc/elements/1.1/","@xmlns:edm":"http://www.europeana.eu/schemas/edm/","@xmlns:wgs84_pos":"http://www.w3.org/2003/01/geo/wgs84_pos","@xmlns:foaf":"http://xmlns.com/foaf/0.1/","@xmlns:rdaGr2":"http://rdvocab.info/ElementsGr2","@xmlns:oai":"http://www.openarchives.org/OAI/2.0/","@xmlns:owl":"http://www.w3.org/2002/07/owl#","@xmlns:rdf":"http://www.w3.org/1999/02/22-rdf-syntax-ns#","@xmlns:ore":"http://www.openarchives.org/ore/terms/","@xmlns:skos":"http://www.w3.org/2004/02/skos/core#","@xmlns:dcterms":"http://purl.org/dc/terms/","edm:WebResource":[{"@rdf:about":"http://www.dlib.si/stream/URN:NBN:SI:DOC-GNWRBOXO/bb91e1c4-442b-4ae0-bef7-14219b5dacae/HTML","dcterms:extent":"42 KB"},{"@rdf:about":"http://www.dlib.si/stream/URN:NBN:SI:DOC-GNWRBOXO/ddc97055-5a0b-461a-99bc-48664783929b/PDF","dcterms:extent":"109 KB"},{"@rdf:about":"http://www.dlib.si/stream/URN:NBN:SI:DOC-GNWRBOXO/be3466c4-fe93-48d1-a47a-510b5728ddbd/TEXT","dcterms:extent":"21 KB"}],"edm:TimeSpan":{"@rdf:about":"1929-2026","edm:begin":{"@xml:lang":"en","#text":"1929"},"edm:end":{"@xml:lang":"en","#text":"2026"}},"edm:ProvidedCHO":{"@rdf:about":"URN:NBN:SI:DOC-GNWRBOXO","dcterms:isPartOf":[{"@rdf:resource":"https://www.dlib.si/details/urn:nbn:si:spr-a30mfzkp"},{"@xml:lang":"sl","#text":"Zdravniški vestnik"}],"dcterms:issued":"2008","dc:creator":"Liščić, Rajka","dc:format":[{"@xml:lang":"sl","#text":"številka:2"},{"@xml:lang":"sl","#text":"4 strani"},{"@xml:lang":"sl","#text":"letnik:77"},{"@xml:lang":"sl","#text":"str. II-71-II-74"}],"dc:identifier":["ISSN:1318-0347","COBISSID:24290009","URN:URN:NBN:SI:doc-GNWRBOXO"],"dc:language":"en","dc:publisher":{"@xml:lang":"sl","#text":"Slovensko zdravniško društvo"},"dc:subject":[{"@xml:lang":"sl","#text":"Alzheimerjeva bolezen"},{"@xml:lang":"en","#text":"Alzheimer's Disease"},{"@xml:lang":"sl","#text":"Demenca"},{"@xml:lang":"en","#text":"Dementia"},{"@xml:lang":"en","#text":"Diagnosis"},{"@xml:lang":"en","#text":"Diagnosis, Differential"},{"@xml:lang":"en","#text":"diagnostika"},{"@xml:lang":"sl","#text":"Diagnostika diferencialna"},{"@xml:lang":"en","#text":"DNA-Binding Proteins"},{"@xml:lang":"sl","#text":"DNA-vezalne beljakovine"},{"@xml:lang":"sl","#text":"Fenotip"},{"@xml:lang":"sl","#text":"fronto-temporalna degeneracija"},{"@xml:lang":"en","#text":"Genetics"},{"@xml:lang":"sl","#text":"molekularna genetika"},{"@xml:lang":"en","#text":"neurology"},{"@xml:lang":"sl","#text":"nevrologija"},{"@xml:lang":"en","#text":"Phenotype"}],"dcterms:temporal":{"@rdf:resource":"1929-2026"},"dc:title":{"@xml:lang":"sl","#text":"How to differentiate frontotemporal from Alzheimer's dementia? Recent developments in molecular genetics and neuropathology| Kako ločiti fronto-temporalno od Alzheimerjeve demence? Najnovejša odkritja molekularne genetike in nevropatologije|"},"dc:description":[{"@xml:lang":"sl","#text":"Frontotemporal dementia is a major cause of non-Alzheimer dementia (AD). Frontotemporallobar degeneration (FTLD) is used here as an umbrella term for both clinical and neuropathological entities starting before age of 65 years. FTLD differs clinically from ADbecause memory loss is rarely an early symptom.Instead, the dementia of FTLD is usually denoted by behavioral and language difficulties, although clinical and cognitive features of FTLD may overlap with AD. Aphasia may be prominent, either fluent or nonfluent. Clinical FTLD is associated with a variety of different neuropathological entities, which share common feature of preferential degeneration of the frontal and temporal lobes. Whereas, in the past, most attention focused on FTLD pathology associated with tau-positive inclusions and microtubule associated protein tau gene (MAPT) mutations (tauopathies), there has recentlybeen greater attention paid to non-tau, ubiquitin positive inclusions (FTLD-U) or non-tauopathies. It is now recognized that FTLD-U is the most common pathology associated with clinical FTLD. Clinically, cases with FTLD-U may additionally present with or without motor neuron disease and parkinsonism. Majority of familial cases of FTLD-U have mutations in the progranulin (PRGN) gene. Some families of FTLD-U with PGRN mutation (hereditary dysphasic disinhibition dementia 1 and 2) are characterized, besides behavior and language difficulties, by additional memory loss and AD-type pathology. Recently, the ubiquitinated pathological protein in FTLD-U has been identified as TAR DNA binding protein (TDP 43) and found in an increasing number of neurodegenerative diseases, including AD. The overlap between FTLD-U and AD is important since as many as 20 % of AD cases show someFTLD-U type TDP-43 pathology. Recent developments have helped to clarify the relationship between different types of FTLD and related conditions. (Abstract truncated at 2000 characters)"},{"@xml:lang":"sl","#text":"Fronto-temporalna demenca je najpogostejša ne-Alzheimerjeva demenca (AD). Frontotemporalne lobarne degeneracije (FTLD) je širše ime za klinične in nevropatološke bolezni z začetkom pred 65 letom starosti. FTLD se klinično razlikuje od Alzheimerjeve demence, saj je izguba spomina le redko prvi simptom bolezni. Za demence v sklopu FTLD so značilne motnje vedenja in jezika, čeprav se klinične in kognitivne značilnosti obeh lahko prekrivajo. Afazija je lahko izrazita, tako fluentna kot nefluentna. Klinična oblika FTLD je lahko povezana z različnimi nevropatološkimi izvidi, ki jim je skupna degeneracija pretežno frontalnih in temporalnih režnjev. Medtem, ko je bilo v preteklosti več pozornosti posvečene patologiji FTLD povezani s tau pozitivnimi vključki in mutacijami gena za mikrotubule povezane s tau genom (tauopatije), se v zadnjem času več pozornosti posveča ne-tau, ubikvitin pozitivnim vključkom (FTLD-U) ali ne-tauopatijam. Znano je, da je FTLD-U najpogostejša patološka najdba povezana s klinično obliko FTLD. Klinično, se lahko primeri FTLD-U kažejo tudi z ali brez bolezni motoričnega nevrona ali parkinsonizma. Večina družin z FTLD-U ima mutacijo gena za progranulin (PRGN).Nekatere družine s FTLD-U in mutacijo PRGN (hereditarna disfazična dezinibitorna demenca 1 in 2) imajo klinično poleg motenj vedenja in jezika šemotnje spomina in patologijo, značilno za AD. Nedavno je bil pri FTLD-U odkrit ubikvitiran patološki protein, TAR DNA protein 43 (TDP-43), ki ga najdemo pri velikem številu nevrodegenerativnih bolezni, vključno z AD. Prekrivanje FTLD-U in AD je pomembno, ker kar 20 % primerov z AD kaže nekaj TDP-43 patologije tipa FTLD-U. Zadnja odkritja so pripomogla k razjasnitvi odnosa med različnimi vrstami FTLD in sorodnimi stanji. Razumevanje in razlikovanje med FTLD in AD je zelo pomembno za postavitev diagnoze, še posebej, ko bodo za razpolago novi diagnostični testi in zdravljenja"}],"edm:type":"TEXT","dc:type":[{"@xml:lang":"sl","#text":"znanstveno časopisje"},{"@xml:lang":"en","#text":"journals"},{"@rdf:resource":"http://www.wikidata.org/entity/Q361785"}]},"ore:Aggregation":{"@rdf:about":"http://www.dlib.si/?URN=URN:NBN:SI:DOC-GNWRBOXO","edm:aggregatedCHO":{"@rdf:resource":"URN:NBN:SI:DOC-GNWRBOXO"},"edm:isShownBy":{"@rdf:resource":"http://www.dlib.si/stream/URN:NBN:SI:DOC-GNWRBOXO/ddc97055-5a0b-461a-99bc-48664783929b/PDF"},"edm:rights":{"@rdf:resource":"http://creativecommons.org/licenses/by-nc/4.0/"},"edm:provider":"Slovenian National E-content Aggregator","edm:intermediateProvider":{"@xml:lang":"en","#text":"National and University Library of Slovenia"},"edm:dataProvider":{"@xml:lang":"sl","#text":"Slovensko zdravniško društvo"},"edm:object":{"@rdf:resource":"http://www.dlib.si/streamdb/URN:NBN:SI:DOC-GNWRBOXO/maxi/edm"},"edm:isShownAt":{"@rdf:resource":"http://www.dlib.si/details/URN:NBN:SI:DOC-GNWRBOXO"}}}}