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Maladie de Huntington
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Définition:
A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive and in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; ; ; and . Eventually intellectual impairment; loss of fine motor control; ; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including ; ; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)
Synonyme(s):
Chorée de Huntington /
Chorée chronique progressive de Huntington /
Chorée chronique progressive héréditaire de Huntington /
Maladie de Huntington à début tardif /
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(NLM)®
de l'INSERM (version française)
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PSYCHIATRIE ET PSYCHOLOGIE
Troubles mentaux