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
Engelska synonymer

Huntingtons korea — Juvenil Huntingtons sjukdom — Sen debut av Huntingtons sjukdom

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