title: [The CADASIL syndrome: a model of subcortical-cortical disconnection].
abstract: BACKGROUND
  CADASIL syndrome (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarts and Leukoencephalopathy) includes some neurological signs and symptoms (gait disturbances, epileptic seizures, pseudobulbar palsy, migraines, etc.), as well as neuropsychological dysfunctions (cognitive and executive impairment, emotional disorders and, frequently, dementia). This syndrome is a good model of white matter damage and potential disconnection syndromes.
  
  
  METHODS
  In this article, the neuropsychological profile of a 47 year-old woman diagnosed of CADASIL is investigated and thoroughly discussed. Results show the presence of a moderate cognitive and executive impairment, specially of memory, psychomotor abilities, executive functions and verbal fluency, but not dementia, overall suggesting the presence of a temporal-frontal-subcortical disfunction.
  
  
  CONCLUSIONS
  This clinical pattern is an illuminating example of the neuropsychological consequences of the partial disconnection of prefrontal cortex from the thalamus and basal ganglia.
authors.affiliation: Unidad de Neuropsicología, Hospital Central de Asturias, Oviedo, 33006, Spain.
authors.forename: R
authors.initials: R
authors.lastname: Blanco Menéndez
authors.forename: A M
authors.initials: AM
authors.lastname: Aguado Balsas
authors.forename: E
authors.initials: E
authors.lastname: Blanco
authors.forename: B
authors.initials: B
authors.lastname: Lobo Rodríguez
authors.forename: E
authors.initials: E
authors.lastname: Vera De La Puente
mesh_terms: D001921:Brain / Q000473:pathology / Q000502:physiology; D046589:CADASIL / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology*; D003072:Cognition Disorders / Q000235:genetics / Q000503:physiopathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D009483:Neuropsychological Tests; D011596:Psychomotor Disorders / Q000235:genetics / Q000503:physiopathology