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Factors such as family history of dementia, age at onset, presenting features such as personality change, cognitive deficits, psychiatric symptoms, and clinical course (progressive deterioration; retention of skills over time) may prove useful for directing investigations to identify underlying pathology and genetic implications. This is illustrated by two patient reports. Each patient had the onset of memory\/behavioral problems at approximately age 40 years, was initially given a psychiatric, non-dementing diagnosis, and had a positive family history for early onset behavioral and memory problems. After longitudinal assessment, the diagnosis of Alzheimer disease was confirmed at autopsy in one patient and a diagnosis of familial, adult-onset metachromatic leukodystrophy in the other.<\/jats:p>","DOI":"10.1017\/s031716710004823x","type":"journal-article","created":{"date-parts":[[2016,2,17]],"date-time":"2016-02-17T09:25:28Z","timestamp":1455701128000},"page":"312-318","source":"Crossref","is-referenced-by-count":10,"title":["The Differential Diagnosis of Adult Onset Metachromatic Leukodystrophy and Early Onset Familial Alzheimer Disease in an Alzheimer Clinic Population"],"prefix":"10.1017","volume":"20","author":[{"given":"A.D.","family":"Sadovnick","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Tuokko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.A.","family":"Applegarth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.R.","family":"Toone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Haijistavropoulos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.L.","family":"Beattie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2015,9,18]]},"reference":[{"key":"S031716710004823X_ref019","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(59)90119-6"},{"key":"S031716710004823X_ref001","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.34.7.939"},{"key":"S031716710004823X_ref020","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(87)90068-4"},{"key":"S031716710004823X_ref025","doi-asserted-by":"publisher","DOI":"10.1007\/BF00687733"},{"key":"S031716710004823X_ref003","doi-asserted-by":"publisher","DOI":"10.1038\/347194a0"},{"key":"S031716710004823X_ref045","first-page":"610","article-title":"White matter dysfunction and its neuropsychological correlates: a longitudinal study of a case pf metachromatic leukodystrophy treated with bone marrow transplant","volume":"14","author":"Schapiro","year":"1992","journal-title":"J Clin Exp Psychol"},{"key":"S031716710004823X_ref038","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1176\/ajp.146.5.577","article-title":"Overview of depression and psychosis in Alzheimer\u2019s disease","volume":"146","author":"Wragg","year":"1989","journal-title":"Am J Psychiatry"},{"key":"S031716710004823X_ref008","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199101033240104"},{"key":"S031716710004823X_ref033","first-page":"567","article-title":"Studies on adult onset metachromatic leukodystrophy: I. 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