Psychiatr. pro Praxi, 2009; 10(5): 205-208

Symptomatic treatment in Huntington’s disease

doc. MUDr. Jan Roth CSc¹, MUDr. Jiří Klempíř, Ph.D¹, MUDr. Tereza Uhrová²

¹ Neurologická klinika 1. LF UK a VFN, Praha

² Psychiatrická klinika 1. LF UK a VFN Praha

Huntington´s disease (HD) is an autosomal dominantly inherited fatal neurodegenerative disease. HD symptoms comprise a variety of

movements disorders, behavioral and other neuropsychiatric changes including severe dementia. The onset of HD is usually in the 4th

decade. The diagnosis is confirmed by the genetical testing. The therapy of HD is purely symptomatic. Choreatic dyskinesias can be

ameliorated by antipsychotics (tiapride, risperidone etc), GABAergics (clonazepame) or depletors of dopamine (tetrabenazine). Dystonic

posturing could be improved by amantadine. In rare juvenile cases with dominant rigidity and akinesia levodopa could be effective.

Irritability, agressivity in HD could be ameliorated by antipsychotics or valproate. Antipsychotics are used in HD patients with hallucinations

and delusions. Dementia is unfortunately fully untreatable. The essential role in HD plays the appropriate nutrition. During the

progresson of HD specific symptoms appear and multidisciplinary highly specialized approach is needed.

Keywords: Huntington´s disease, antipsychotics, antidepressants, amantadine, clonazepam, sipping, percutaneuous gastrostomy.

Published: November 1, 2009  Show citation