{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T15:13:42Z","timestamp":1786979622906,"version":"build-2736575974"},"reference-count":36,"publisher":"Springer Science and Business Media LLC","issue":"10","license":[{"start":{"date-parts":[[2020,7,28]],"date-time":"2020-07-28T00:00:00Z","timestamp":1595894400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,7,28]],"date-time":"2020-07-28T00:00:00Z","timestamp":1595894400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Universit\u00e4tsklinikum M\u00fcnster"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Nervenarzt"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Hintergrund<\/jats:title>\n                    <jats:p>Aufgrund des demographischen Wandels sind Demenzen ein h\u00e4ufiger und dramatisch zunehmender Grund f\u00fcr \u00e4rztliche Vorstellungen. In etwa 8\u202f% der F\u00e4lle treten sie bereits vor dem 65.\u00a0Lebensjahr auf. Gerade bei j\u00fcngeren Patienten sind die psychosozialen und \u00f6konomischen Folgen oft gravierend. Die Behandler stehen vor gro\u00dfen diagnostischen Herausforderungen. Eine rasche Diagnose ist f\u00fcr das Patientenmanagement von zentraler Bedeutung.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Ziel der Arbeit\/Fragestellung<\/jats:title>\n                    <jats:p>Dieser \u00dcbersichtsartikel stellt die Besonderheiten der Demenzen bei j\u00fcngeren Menschen sowie die wichtigsten zugrunde liegenden Krankheitsbilder vor und vermittelt ein strukturiertes klinisch-diagnostisches Vorgehen.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methoden<\/jats:title>\n                    <jats:p>Narrativer Review. Die Literatursuche wurde in PubMed durchgef\u00fchrt.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Ergebnisse<\/jats:title>\n                    <jats:p>Das differenzialdiagnostische Spektrum von Demenzen bei j\u00fcngeren Menschen vor dem 65.\u00a0Lebensjahr ist sehr breit. Die h\u00e4ufigsten Ursachen stellen die Alzheimer-Krankheit mit typischen oder atypischen klinischen Pr\u00e4sentationen sowie die frontotemporale Lob\u00e4rdegeneration dar. Je j\u00fcnger das Erkrankungsalter, desto h\u00f6her ist der Anteil an behandelbaren und potenziell reversiblen Ursachen eines demenziellen Syndroms.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Diskussion<\/jats:title>\n                    <jats:p>Die Diagnostik prim\u00e4r neurodegenerativer Erkrankungen hat sich zunehmend verbessert, insbesondere unter Ber\u00fccksichtigung einer stetig steigenden Zahl an klinischen, molekularen und bildgebenden Biomarkern. Dennoch muss die Diagnostik der Demenzen mit fr\u00fchem Erkrankungsbeginn hypothesengeleitet erfolgen, d.\u202fh. nach einer pr\u00e4zisen klinisch-syndromalen Zuordnung der Symptome. So k\u00f6nnen unn\u00f6tige und belastende Untersuchungen vermieden werden.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00115-020-00967-0","type":"journal-article","created":{"date-parts":[[2020,7,28]],"date-time":"2020-07-28T03:51:41Z","timestamp":1595908301000},"page":"936-945","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Fr\u00fch beginnende Demenzen","Young onset dementia"],"prefix":"10.1007","volume":"91","author":[{"given":"Matthias","family":"Pawlowski","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Johnen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Duning","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,7,28]]},"reference":[{"key":"967_CR1","doi-asserted-by":"publisher","first-page":"325","DOI":"10.1016\/j.jalz.2017.02.001","volume":"13","author":"Alzheimer\u2019s Association","year":"2017","unstructured":"Alzheimer\u2019s Association (2017) 2017 Alzheimer\u2019s disease facts and figures. Alzheimers Dement 13:325\u2013373","journal-title":"Alzheimers Dement"},{"key":"967_CR2","doi-asserted-by":"publisher","first-page":"1672","DOI":"10.1016\/S0140-6736(15)00461-4","volume":"386","author":"J Bang","year":"2015","unstructured":"Bang\u00a0J, Spina\u00a0S, Miller\u00a0BL (2015) Frontotemporal dementia. Lancet 386:1672\u20131682","journal-title":"Lancet"},{"key":"967_CR3","doi-asserted-by":"publisher","first-page":"706","DOI":"10.1093\/brain\/awm320","volume":"131","author":"J Beck","year":"2008","unstructured":"Beck\u00a0J, Rohrer\u00a0JD, Campbell\u00a0T et\u00a0al (2008) A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a\u00a0large UK series. Brain 131:706\u2013720","journal-title":"Brain"},{"key":"967_CR4","doi-asserted-by":"publisher","first-page":"1079","DOI":"10.1007\/s00115-005-1949-y","volume":"77","author":"H Bickel","year":"2006","unstructured":"Bickel\u00a0H, B\u00fcrger\u00a0K, Hampel\u00a0H et\u00a0al (2006) Presenile dementia in memory clinics\u2014Incidence rates and clinical features. Nervenarzt 77:1079\u20131085","journal-title":"Nervenarzt"},{"key":"967_CR5","doi-asserted-by":"publisher","first-page":"680","DOI":"10.1136\/jnnp-2014-309086","volume":"86","author":"J Brown","year":"2015","unstructured":"Brown\u00a0J (2015) The use and misuse of short cognitive tests in the diagnosis of dementia. J\u00a0Neurol Neurosurg Psychiatry 86:680\u2013685","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"967_CR6","doi-asserted-by":"publisher","first-page":"2219","DOI":"10.1001\/archinte.163.18.2219","volume":"163","author":"AM Clarfield","year":"2003","unstructured":"Clarfield\u00a0AM (2003) The decreasing prevalence of reversible dementias: an updated meta-analysis. Arch Intern Med 163:2219\u20132229","journal-title":"Arch Intern Med"},{"key":"967_CR7","doi-asserted-by":"publisher","first-page":"870","DOI":"10.1016\/j.jalz.2017.01.014","volume":"13","author":"SJ Crutch","year":"2017","unstructured":"Crutch\u00a0SJ, Schott\u00a0JM, Rabinovici\u00a0GD et\u00a0al (2017) Consensus classification of posterior cortical atrophy. Alzheimers Dement 13:870\u2013884","journal-title":"Alzheimers Dement"},{"key":"967_CR8","doi-asserted-by":"publisher","first-page":"1345","DOI":"10.1001\/jamaneurol.2017.1964","volume":"74","author":"CA Derby","year":"2017","unstructured":"Derby\u00a0CA, Katz\u00a0MJ, Lipton\u00a0RB, Hall\u00a0CB (2017) Trends in dementia incidence in a\u00a0birth cohort analysis of the Einstein aging study. JAMA Neurol 74:1345\u20131351","journal-title":"JAMA Neurol"},{"key":"967_CR9","series-title":"Leitlinien f\u00fcr Diagnostik und Therapie in der Neurologie","volume-title":"S3-Leitlinie Demenzen","author":"G Deuschl","year":"2016","unstructured":"Deuschl\u00a0G, Maier\u00a0W (2016) S3-Leitlinie Demenzen. Leitlinien f\u00fcr Diagnostik und Therapie in der Neurologie, Bd. 128"},{"key":"967_CR10","doi-asserted-by":"publisher","first-page":"779","DOI":"10.1111\/imj.13099","volume":"46","author":"B Draper","year":"2016","unstructured":"Draper\u00a0B, Withall\u00a0A (2016) Young onset dementia. Intern Med\u00a0J 46:779\u2013786","journal-title":"Intern Med J"},{"key":"967_CR11","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1016\/0022-3956(75)90026-6","volume":"12","author":"MF Folstein","year":"1975","unstructured":"Folstein\u00a0MF, Folstein\u00a0SE, McHugh\u00a0PR (1975) \u201cMini-mental state\u201d. A practical method for grading the cognitive state of patients for the clinician. J\u00a0Psychiatr Res 12:189\u2013198","journal-title":"J Psychiatr Res"},{"key":"967_CR12","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1007\/s40291-018-0347-7","volume":"22","author":"JS Goldman","year":"2018","unstructured":"Goldman\u00a0JS, Van Deerlin\u00a0VM (2018) Alzheimer\u2019s disease and frontotemporal dementia: the current state of genetics and genetic testing since the advent of next-generation sequencing. Mol Diagn Ther 22:505\u2013513","journal-title":"Mol Diagn Ther"},{"key":"967_CR13","doi-asserted-by":"publisher","first-page":"1006","DOI":"10.1212\/WNL.0b013e31821103e6","volume":"76","author":"ML Gorno-Tempini","year":"2011","unstructured":"Gorno-Tempini\u00a0ML, Hillis\u00a0AE, Weintraub\u00a0S et al (2011) Classification of primary progressive aphasia and its variants. Neurology 76:1006\u20131014","journal-title":"Neurology"},{"key":"967_CR14","doi-asserted-by":"publisher","first-page":"1206","DOI":"10.1136\/jnnp.74.9.1206","volume":"74","author":"RJ Harvey","year":"2003","unstructured":"Harvey\u00a0RJ, Skelton-Robinson\u00a0M, Rossor\u00a0MN (2003) The prevalence and causes of dementia in people under the age of 65 years. J\u00a0Neurol Neurosurg Psychiatry 74:1206\u20131209","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"967_CR15","doi-asserted-by":"publisher","first-page":"392","DOI":"10.1001\/archneur.1986.00520040072023","volume":"43","author":"SJ Huber","year":"1986","unstructured":"Huber\u00a0SJ, Shuttleworth\u00a0EC, Paulson\u00a0GW et\u00a0al (1986) Cortical vs subcortical dementia. Neuropsychological differences. Arch Neurol 43:392\u2013394","journal-title":"Arch Neurol"},{"key":"967_CR16","doi-asserted-by":"publisher","first-page":"2709","DOI":"10.1161\/STROKEAHA.108.542308","volume":"40","author":"C Ikejima","year":"2009","unstructured":"Ikejima\u00a0C, Yasuno\u00a0F, Mizukami\u00a0K et al (2009) Prevalence and causes of early-onset dementia in Japan: a\u00a0population-based study. Stroke 40:2709\u20132714","journal-title":"Stroke"},{"key":"967_CR17","doi-asserted-by":"publisher","first-page":"200","DOI":"10.1055\/s-0043-118778","volume":"48","author":"A Johnen","year":"2017","unstructured":"Johnen\u00a0A, Duning\u00a0T (2017) Neuropsychologische Diagnostik in der Praxis. Was ist leitliniengerecht, was ist sinnvoll, was ist machbar? Klin Neurophysiol 48:200\u2013210","journal-title":"Klin Neurophysiol"},{"key":"967_CR18","doi-asserted-by":"publisher","first-page":"1502","DOI":"10.1001\/archneur.65.11.1502","volume":"65","author":"BJ Kelley","year":"2008","unstructured":"Kelley\u00a0BJ, Boeve\u00a0BF, Josephs\u00a0KA (2008) Young-onset dementia: demographic and etiologic characteristics of 235 patients. Arch Neurol 65:1502\u20131508","journal-title":"Arch Neurol"},{"key":"967_CR19","doi-asserted-by":"publisher","first-page":"1931","DOI":"10.1017\/S1041610214001574","volume":"26","author":"R Koopmans","year":"2014","unstructured":"Koopmans\u00a0R, Rosness\u00a0T (2014) Young onset dementia\u2014What does the name imply? Int Psychogeriatr 26:1931\u20131933","journal-title":"Int Psychogeriatr"},{"key":"967_CR20","doi-asserted-by":"publisher","first-page":"479","DOI":"10.3233\/JAD-181223","volume":"69","author":"M Kvello-Alme","year":"2019","unstructured":"Kvello-Alme\u00a0M, Br\u00e5then\u00a0G, White\u00a0LR, Sando\u00a0SB (2019) The prevalence and subtypes of young onset dementia in central Norway: a population-based study. J\u00a0Alzheimers Dis 69:479\u2013487","journal-title":"J Alzheimers Dis"},{"key":"967_CR21","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1111\/ene.12325","volume":"21","author":"MA Lambert","year":"2014","unstructured":"Lambert\u00a0MA, Bickel\u00a0H, Prince\u00a0M et\u00a0al (2014) Estimating the burden of early onset dementia; systematic review of disease prevalence. Eur J Neurol 21:563\u2013569","journal-title":"Eur J Neurol"},{"key":"967_CR22","doi-asserted-by":"publisher","first-page":"1792","DOI":"10.1093\/brain\/awx101","volume":"140","author":"CJ Lansdall","year":"2017","unstructured":"Lansdall\u00a0CJ, Coyle-Gilchrist\u00a0ITS, Jones\u00a0PS et al (2017) Apathy and impulsivity in frontotemporal lobar degeneration syndromes. Brain 140:1792\u20131807","journal-title":"Brain"},{"key":"967_CR23","first-page":"34","volume":"25","author":"MF Mendez","year":"2019","unstructured":"Mendez\u00a0MF (2019) Early-onset Alzheimer disease and its variants. Continuum (N Y) 25:34\u201351","journal-title":"Continuum (N Y)"},{"key":"967_CR24","doi-asserted-by":"publisher","first-page":"709","DOI":"10.1002\/ana.21388","volume":"63","author":"M Mesulam","year":"2008","unstructured":"Mesulam\u00a0M, Wicklund\u00a0A, Johnson\u00a0N et\u00a0al (2008) Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia. Ann Neurol 63:709\u2013719","journal-title":"Ann Neurol"},{"key":"967_CR25","doi-asserted-by":"publisher","first-page":"695","DOI":"10.1111\/j.1532-5415.2005.53221.x","volume":"53","author":"ZS Nasreddine","year":"2005","unstructured":"Nasreddine\u00a0ZS, Phillips\u00a0NA, B\u00e9dirian\u00a0V et\u00a0al (2005) The Montreal Cognitive Assessment, MoCA: a\u00a0brief screening tool for mild cognitive impairment. J\u00a0Am Geriatr Soc 53:695\u2013699","journal-title":"J Am Geriatr Soc"},{"key":"967_CR26","doi-asserted-by":"publisher","first-page":"2732","DOI":"10.1093\/brain\/awv191","volume":"138","author":"R Ossenkoppele","year":"2015","unstructured":"Ossenkoppele\u00a0R, Pijnenburg\u00a0YAL, Perry\u00a0DC et\u00a0al (2015) The behavioural\/dysexecutive variant of Alzheimer\u2019s disease: clinical, neuroimaging and pathological features. Brain 138:2732\u20132749","journal-title":"Brain"},{"key":"967_CR27","doi-asserted-by":"publisher","first-page":"42","DOI":"10.3390\/diagnostics7030042","volume":"7","author":"M Pawlowski","year":"2017","unstructured":"Pawlowski\u00a0M, Meuth\u00a0SG, Duning\u00a0T (2017) Cerebrospinal fluid biomarkers in Alzheimer\u2019s disease-from brain starch to bench and bedside. Diagnostics 7:42","journal-title":"Diagnostics"},{"key":"967_CR28","doi-asserted-by":"publisher","first-page":"2456","DOI":"10.1093\/brain\/awr179","volume":"134","author":"K Rascovsky","year":"2011","unstructured":"Rascovsky\u00a0K, Hodges\u00a0JR, Knopman\u00a0D et\u00a0al (2011) Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 134:2456\u20132477","journal-title":"Brain"},{"key":"967_CR29","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1038\/nrneurol.2011.2","volume":"7","author":"C Reitz","year":"2011","unstructured":"Reitz\u00a0C, Brayne\u00a0C, Mayeux\u00a0R (2011) Epidemiology of Alzheimer disease. Nat Rev Neurol 7:137\u2013152","journal-title":"Nat Rev Neurol"},{"key":"967_CR30","doi-asserted-by":"publisher","first-page":"426","DOI":"10.1016\/S1474-4422(02)00190-4","volume":"1","author":"GC Rom\u00e1n","year":"2002","unstructured":"Rom\u00e1n\u00a0GC, Erkinjuntti\u00a0T, Wallin\u00a0A et\u00a0al (2002) Subcortical ischaemic vascular dementia. Lancet Neurol 1:426\u2013436","journal-title":"Lancet Neurol"},{"key":"967_CR31","doi-asserted-by":"publisher","first-page":"793","DOI":"10.1016\/S1474-4422(10)70159-9","volume":"9","author":"MN Rossor","year":"2010","unstructured":"Rossor\u00a0MN, Fox\u00a0NC, Mummery\u00a0CJ et al (2010) The diagnosis of young-onset dementia. Lancet Neurol 9:793\u2013806","journal-title":"Lancet Neurol"},{"key":"967_CR32","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1016\/j.conb.2019.11.019","volume":"61","author":"J Simr\u00e9n","year":"2019","unstructured":"Simr\u00e9n\u00a0J, Ashton\u00a0NJ, Blennow\u00a0K, Zetterberg\u00a0H (2019) An update on fluid biomarkers for neurodegenerative diseases: recent success and challenges ahead. Curr Opin Neurobiol 61:29\u201339","journal-title":"Curr Opin Neurobiol"},{"key":"967_CR33","doi-asserted-by":"publisher","first-page":"643","DOI":"10.3233\/JAD-180080","volume":"64","author":"AE Thomann","year":"2018","unstructured":"Thomann\u00a0AE, Goettel\u00a0N, Monsch\u00a0RJ et al (2018) The montreal cognitive assessment: normative data from a\u00a0German-speaking cohort and comparison with international normative samples. J\u00a0Alzheimers Dis 64:643\u2013655","journal-title":"J Alzheimers Dis"},{"key":"967_CR34","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1038\/nrneurol.2012.135","volume":"8","author":"JD Warren","year":"2012","unstructured":"Warren\u00a0JD, Fletcher\u00a0PD, Golden\u00a0HL (2012) The paradox of syndromic diversity in Alzheimer disease. Nat Rev Neurol 8:451\u2013464","journal-title":"Nat Rev Neurol"},{"key":"967_CR35","doi-asserted-by":"publisher","first-page":"1955","DOI":"10.1017\/S1041610214001835","volume":"26","author":"A Withall","year":"2014","unstructured":"Withall\u00a0A, Draper\u00a0B, Seeher\u00a0K, Brodaty\u00a0H (2014) The prevalence and causes of younger onset dementia in Eastern Sydney, Australia. Int Psychogeriatr 26:1955\u20131965","journal-title":"Int Psychogeriatr"},{"key":"967_CR36","doi-asserted-by":"publisher","DOI":"10.1136\/practneurol-2017-001743","author":"C Zucchella","year":"2018","unstructured":"Zucchella\u00a0C, Federico\u00a0A, Martini\u00a0A et\u00a0al (2018) Neuropsychological testing. Pract Neurol. https:\/\/doi.org\/10.1136\/practneurol-2017-001743","journal-title":"Pract Neurol"}],"container-title":["Der Nervenarzt"],"original-title":[],"language":"de","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00115-020-00967-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00115-020-00967-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00115-020-00967-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,7,27]],"date-time":"2021-07-27T19:07:42Z","timestamp":1627412862000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00115-020-00967-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,7,28]]},"references-count":36,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2020,10]]}},"alternative-id":["967"],"URL":"https:\/\/doi.org\/10.1007\/s00115-020-00967-0","relation":{},"ISSN":["0028-2804","1433-0407"],"issn-type":[{"value":"0028-2804","type":"print"},{"value":"1433-0407","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,7,28]]},"assertion":[{"value":"28 July 2020","order":1,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Einhaltung ethischer Richtlinien"}},{"value":"M.\u00a0Pawlowski, A.\u00a0Johnen und T.\u00a0Duning geben an, dass kein Interessenkonflikt besteht.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Interessenkonflikt"}},{"value":"F\u00fcr diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgef\u00fchrt. F\u00fcr die aufgef\u00fchrten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Interessenkonflikt"}}]}}