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

Francisco Javier Domínguez-Sánchez1, Amaia Lasa-Aristu, Miguel Goñi-Imízcoz

  • 1Facultad de Psicología, Universida Nacional de Educación a Distancia (UNED), C/Juan del Rosal, 10. 28040 Madrid, Spain. franjados@psi.uned.es

The Spanish Journal of Psychology
|November 9, 2011
PubMed

Insights

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare brain disorder. This study details cognitive and mood changes in three affected family members over seven years.

Area of Science:

  • Neurology
  • Genetics
  • Neuropsychology

Background:

  • Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited small-vessel brain disease.
  • It typically manifests in mid-adulthood with symptoms like stroke, cognitive decline, and mood disturbances.

Observation:

  • This study followed three patients from a single Spanish family with CADASIL over seven years.
  • Comprehensive clinical and neuropsychological evaluations were performed.

Findings:

  • Patients exhibited significant decline in intelligence quotient (IQ) and abstract reasoning (g factor).
  • Mood disturbances, including major depression and dysthymia, were prominent.
  • Neurotic personality features were also observed.

Implications:

  • These findings highlight the progressive cognitive and psychological impact of CADASIL.
  • Understanding these deficits is crucial for patient management and therapeutic development.
  • This case series contributes to the clinical characterization of CADASIL in a familial context.

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