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The disease is transmitted with an autosomal dominant pattern and usually starts during midadulthood (at 30\u201350 years of age). Cognitive deficits in patients with CADASIL develop slowly. The dementia causes frontal-like symptoms and it typically develops after a history of recurrent stroke. We describe three patients from one Spanish family affected by this disease. All three cases underwent comprehensive clinical and neuropsychological examination, and were monitored for seven years. The results obtained in this study describe a) a significant loss of the intelligence quotient (IQ) and noticeable damage to abstract ability (g factor), b) mood and psychopathological disturbances (major depression and dysthymia), and c) a personality with neurotic features.<\/jats:p>","DOI":"10.5209\/rev_sjop.2011.v14.n2.39","type":"journal-article","created":{"date-parts":[[2011,11,21]],"date-time":"2011-11-21T09:41:22Z","timestamp":1321868482000},"page":"936-943","source":"Crossref","is-referenced-by-count":5,"title":["Intelligence Impairment, Personality Features and Psychopathology Disturbances in a Family Affected with CADASIL"],"prefix":"10.1017","volume":"14","author":[{"given":"Francisco Javier","family":"Dom\u00ednguez-S\u00e1nchez","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amaia","family":"Lasa-Aristu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miguel","family":"Go\u00f1i-Im\u00edzcoz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2013,1,10]]},"reference":[{"key":"S1138741600003073_ref010","first-page":"299","article-title":"Personality traits are alive and well","volume":"6","author":"Deary","year":"1993","journal-title":"The Psychologist"},{"key":"S1138741600003073_ref006","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-9822(03)00492-5"},{"key":"S1138741600003073_ref002","doi-asserted-by":"publisher","DOI":"10.1161\/01.STR.0000129787.92085.0a"},{"key":"S1138741600003073_ref026","doi-asserted-by":"publisher","DOI":"10.1161\/01.STR.0000018405.59799.D5"},{"key":"S1138741600003073_ref024","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp269"},{"key":"S1138741600003073_ref015","volume-title":"Inventario Multif\u00e1sico de Personalidad de Minnesota","author":"Hathaway","year":"1988"},{"key":"S1138741600003073_ref001","doi-asserted-by":"publisher","DOI":"10.1161\/01.STR.0000029826.41672.2E"},{"key":"S1138741600003073_ref013","volume-title":"Gu\u00eda del usuario para la Entrevista Cl\u00ednica Estructurada para los Trastornos del Eje II del DSM-IV (SCID-II)","author":"First","year":"1999"},{"key":"S1138741600003073_ref003","volume-title":"Diagnostic and Statistical Manual of Mental Disorders","year":"2000"},{"key":"S1138741600003073_ref025","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199709000-00001"},{"key":"S1138741600003073_ref005","doi-asserted-by":"publisher","DOI":"10.1016\/S0160-2896(02)00122-8"},{"key":"S1138741600003073_ref020","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000184480.07394.fb"},{"key":"S1138741600003073_ref031","volume-title":"Escala de Inteligencia de Weschler para Adultos (WAIS)","author":"Wechsler","year":"1977"},{"key":"S1138741600003073_ref012","volume-title":"Gu\u00eda del usuario para la Entrevista Cl\u00ednica Estructurada para los Trastornos del Eje I del DSM-IV. 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