Intelligence impairment, personality features and psychopathology disturbances in a family affected with CADASIL

  1. Domínguez Sánchez, Francisco Javier 1
  2. Lasa Aristu, Amaia 1
  3. Goñi Imízcoz, Miguel 2
  1. 1 Universidad Nacional de Educación a Distancia
    info

    Universidad Nacional de Educación a Distancia

    Madrid, España

    ROR https://ror.org/02msb5n36

  2. 2 Complejo Asistencial Universitario de Burgos
    info

    Complejo Asistencial Universitario de Burgos

    Burgos, España

    ROR https://ror.org/01j5v0d02

Journal:
The Spanish Journal of Psychology

ISSN: 1138-7416

Year of publication: 2011

Volume: 14

Issue: 2

Pages: 936-943

Type: Article

DOI: 10.5209/REV_SJOP.2011.V14.N2.39 DIALNET GOOGLE SCHOLAR lock_openOpen access editor

More publications in: The Spanish Journal of Psychology

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Abstract

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a small-vessel disease of the brain that is characterized by headache, recurring lacunar strokes, mood changes and progressive cognitive deterioration. The disease is transmitted with an autosomal dominant pattern and usually starts during midadulthood (at 30–50 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.

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Funding information

Support for this work was provided by the Research Promotion Plan of the Universidad Nacional de Educación a Distancia (UNED, 2011). We thank Carmen Callejas Hervás for her comments and advice on genetic issues.

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