[The CADASIL syndrome: a model of subcortical-cortical disconnection].
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.
Blanco Menéndez
R
R
Unidad de Neuropsicología, Hospital Central de Asturias, Oviedo, 33006, Spain.
Aguado Balsas
A M
AM
Blanco
E
E
Lobo Rodríguez
B
B
Vera De La Puente
E
E
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