title: Current Pharmacological Approaches to Reduce Chorea in Huntington's Disease.
abstract: There are currently no effective pharmacological agents available to stop or prevent the progression of Huntington's disease (HD), a rare hereditary neurodegenerative disorder. In addition to psychiatric symptoms and cognitive impairments, HD causes progressive motor disturbances, in particular choreiform movements, which are characterized by unwanted contractions of the facial muscles, trunk and extremities. Management of choreiform movements is usually advised if chorea interferes with daily functioning, causes social isolation, gait instability, falls, or physical injury. Although drugs to reduce chorea are available, only few randomized controlled studies have assessed the efficacy of these drugs, resulting in a high variety of prescribed drugs in clinical practice. The current pharmacological treatment options to reduce chorea in HD are outlined in this review, including the latest results on deutetrabenazine, a newly developed pharmacological agent similar to tetrabenazine, but with suggested less peak dose side effects. A review of the existing literature was conducted using the PubMed, Cochrane and Medline databases. In conclusion, mainly tetrabenazine, tiapride (in European countries), olanzapine, and risperidone are the preferred first choice drugs to reduce chorea among HD experts. In the existing literature, these drugs also show a beneficial effect on motor symptom severity and improvement of psychiatric symptoms. Generally, it is recommended to start with a low dose and increase the dose with close monitoring of any adverse effects. New interesting agents, such as deutetrabenazine and pridopidine, are currently under development and more randomized controlled trials are warranted to assess the efficacy on chorea severity in HD.
authors.affiliation: Department of Neurology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
authors.forename: Emma M
authors.initials: EM
authors.lastname: Coppen
authors.affiliation: Department of Neurology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands. r.a.c.roos@lumc.nl.
authors.forename: Raymund A C
authors.initials: RA
authors.lastname: Roos
mesh_terms: D014150:Antipsychotic Agents / Q000627:therapeutic use*; D001569:Benzodiazepines / Q000627:therapeutic use; D006801:Humans; D006816:Huntington Disease / Q000188:drug therapy* / Q000503:physiopathology*; D000077152:Olanzapine; D018967:Risperidone / Q000627:therapeutic use; D013747:Tetrabenazine / Q000627:therapeutic use; D063325:Tiapride Hydrochloride / Q000627:therapeutic use
chemical_list: D014150:Antipsychotic Agents; D001569:Benzodiazepines; D063325:Tiapride Hydrochloride; D018967:Risperidone; D000077152:Olanzapine; D013747:Tetrabenazine