Huntington Disease

Huntingtons sjukdom

Svensk definition

En ärftlig, autosomalt dominant sjukdom, kännetecknad av utveckling av korea (danssjuka) och demens i 40-50-årsåldern. Vanliga tidiga manifestationer är paranoia, impulsivitet, depression, hallucinationer och vanföreställningar. Senare följer mental försämring, förlorad finmotorik, atetos, diffus korea som omfattar lemmarnas muskler, och efter 10-15 år inträder ett vegetativt tillstånd. Ungdomsvarianten har ett våldsammare förlopp, med krampanfall, ataxi, demens och korea.

Engelsk definition

A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; 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 SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)

Svenska synonymer

Huntingtons korea Juvenil Huntingtons sjukdom

Engelska synonymer

Huntington Chorea Chorea, Huntington Huntington's Disease Chronic Progressive Hereditary Chorea (Huntington) Huntington Chronic Progressive Hereditary Chorea Progressive Chorea, Chronic Hereditary (Huntington) Progressive Chorea, Hereditary, Chronic (Huntington) Huntington's Chorea Chorea, Huntington's Chorea, Chronic Progressive Hereditary (Huntington) Huntington Disease, Late Onset Late-Onset Huntington Disease Huntington Disease, Late-Onset Late Onset Huntington Disease Juvenile Huntington Disease Juvenile-Onset Huntington Disease Juvenile Onset Huntington Disease Huntington Disease, Juvenile-Onset Huntington Disease, Juvenile Onset Huntington Disease, Juvenile Akinetic-Rigid Variant of Huntington Disease Akinetic Rigid Variant of Huntington Disease Huntington Disease, Akinetic-Rigid Variant Huntington Disease, Akinetic Rigid Variant