{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T22:29:04Z","timestamp":1791239344233,"version":"4.1.0"},"reference-count":0,"publisher":"Cambridge University Press (CUP)","issue":"6","license":[{"start":{"date-parts":[[2001,9,25]],"date-time":"2001-09-25T00:00:00Z","timestamp":1001376000000},"content-version":"unspecified","delay-in-days":24,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Int Neuropsychol Soc"],"published-print":{"date-parts":[[2001,9]]},"abstract":"<jats:p>Cerebral autosomal dominant arteriopathy with subcortical infarcts \nand leukoencephalopathy (CADASIL) is a hereditary brain disease \nwith a variety of neurologic and psychiatric manifestations. \nWe studied 3 members of a family who each had leukoencephalopathy \non neuroimaging studies and a characteristic mutation for CADASIL \nin the notch 3 region of chromosome 19q12. In all 3 cases, \nneurobehavioral impairment dominated the clinical picture, and \na pattern of psychiatric dysfunction heralding cognitive decline \nemerged. Neuropsychological evaluation revealed diverse deficits, \nbut a profile of frontal lobe dysfunction, declarative memory \nimpairment suggestive of a retrieval deficit, and relatively \npreserved language was evident. These cases provide a \ncross-sectional study of the evolution of CADASIL, and suggest \nthat, as in other diseases characterized by white matter dementia, \npsychiatric dysfunction may occur initially, followed by pervasive \ncognitive dysfunction later in the course of the disease. CADASIL \nshould be considered in young adults with unexplained \nleukoencephalopathy on neuroimaging studies, and in those with \nneurobehavioral dysfunction and a suggestive family history. \n(<jats:italic>JINS<\/jats:italic>, 2001, <jats:italic>7<\/jats:italic>, 768\u2013774.)<\/jats:p>","DOI":"10.1017\/s135561770176612x","type":"journal-article","created":{"date-parts":[[2003,10,15]],"date-time":"2003-10-15T16:10:09Z","timestamp":1066234209000},"page":"768-774","source":"Crossref","is-referenced-by-count":21,"title":["CADASIL: Neuropsychological findings in three generations \nof an affected family"],"prefix":"10.1017","volume":"7","author":[{"given":"JOSETTE G.","family":"HARRIS","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"CHRISTOPHER M.","family":"FILLEY","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2001,9,25]]},"container-title":["Journal of the International Neuropsychological Society"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S135561770176612X","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,4,4]],"date-time":"2019-04-04T20:03:57Z","timestamp":1554408237000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S135561770176612X\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2001,9]]},"references-count":0,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2001,9]]}},"alternative-id":["S135561770176612X"],"URL":"https:\/\/doi.org\/10.1017\/s135561770176612x","relation":{},"ISSN":["1355-6177","1469-7661"],"issn-type":[{"value":"1355-6177","type":"print"},{"value":"1469-7661","type":"electronic"}],"subject":[],"published":{"date-parts":[[2001,9]]}}}