[Executive dysfunction in Huntington's disease].
BACKGROUND
In the early stages of Huntington s disease (HD), there is selective vulnerability of the medium-sized spinous cells of the striate nucleus. These cells are connected with the dorsolateral prefrontal cortex, which is an area involved in cognitive processes concerning attention and executive function, forming part of one of the cortico-striatal circuits.


OBJECTIVE
To analyze and interpret the executive dysfunction of a group of patients with HD at a moderately advanced stage.


METHODS
We evaluated 16 patients and controls. Three clinical scales were used: the motor section of the UHDRS, the independence scale and the total functional capacity scale. For cognitive evaluation we used the study protocol of the Core Assessment Program for Intracerebral Transplantation for HD (CAPIT-HD).


RESULTS
The patients with EH showed significant deterioration on the tests to evaluate cognitive function, attention and working memory. There were only two exceptions, the WEST and the direct order digit span.


CONCLUSIONS
Patients with HD have executive dysfunction, probably due to dysfunction of the cortico-striatal circuits. We therefore agree with the proposal that fronto-striatal cognitive dysfunction is the most suitable name for the cognitive deterioration seen in this condition.
Redondo Vergé
L
L
Servicio de Neurología; Hospital Virgen de la Macarena, Sevilla, España. lredondov@meditex.es
Brown
R G
RG
Chacón
J
J
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D005625:Frontal Lobe / Q000473:pathology; D006801:Humans; D006816:Huntington Disease / Q000150:complications* / Q000473:pathology; D008569:Memory Disorders / Q000209:etiology; D008875:Middle Aged; D009483:Neuropsychological Tests; D012686:Septal Nuclei / Q000473:pathology; D012720:Severity of Illness Index