title: An experimental model for Huntington's chorea?
abstract: Clinically, Huntington's disease (HD) is well known for the predominant motor symptom chorea, which is a hyperkinetic motor disorder. The only experimental model currently described in the literature, as far as we are aware of, exhibiting hyperkinetic movements is the transgenic rat model of HD. We assessed and characterized these hyperkinetic movements in detail and investigated the effect of tetrabenazine (TBZ) treatment. TBZ is an effective drug in the treatment of chorea in HD patients. Our results showed that the hyperkinetic movements fulfilled the clinical-behavioral criteria of a choreiform movement. Administration of TBZ reduced the number of these hyperkinetic movements substantially. These findings suggest that the hyperkinetic movements observed in this animal model can be considered as a choreiform movement disorder. This makes these animals unique and provides opportunities for chorea-research.
authors.affiliation: Department of Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands; Department of Neurosurgery, Maastricht University Medical Center, Maastricht, The Netherlands; European Graduate School of Neuroscience (EURON) , The Netherlands.
authors.forename: Dagmar H
authors.initials: DH
authors.lastname: Zeef
authors.affiliation: Department of Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands; Department of Neurosurgery, Maastricht University Medical Center, Maastricht, The Netherlands; European Graduate School of Neuroscience (EURON) , The Netherlands.
authors.forename: Ali
authors.initials: A
authors.lastname: Jahanshahi
authors.affiliation: Department of Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands; European Graduate School of Neuroscience (EURON) , The Netherlands.
authors.forename: Rinske
authors.initials: R
authors.lastname: Vlamings
authors.affiliation: Department of Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands; European Graduate School of Neuroscience (EURON) , The Netherlands.
authors.forename: João
authors.initials: J
authors.lastname: Casaca-Carreira
authors.affiliation: Department of Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands; European Graduate School of Neuroscience (EURON) , The Netherlands.
authors.forename: Remco G
authors.initials: RG
authors.lastname: Santegoeds
authors.affiliation: Department of Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands; Department of Neurology, Maastricht University Medical Center, Maastricht, The Netherlands; European Graduate School of Neuroscience (EURON) , The Netherlands.
authors.forename: Marcus L F
authors.initials: ML
authors.lastname: Janssen
authors.affiliation: Department of Neurology, Maastricht University Medical Center, Maastricht, The Netherlands.
authors.forename: Mayke
authors.initials: M
authors.lastname: Oosterloo
authors.affiliation: Department of Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands; Department of Neurosurgery, Maastricht University Medical Center, Maastricht, The Netherlands; European Graduate School of Neuroscience (EURON) , The Netherlands. Electronic address: y.temel@maastrichtuniversity.nl.
authors.forename: Yasin
authors.initials: Y
authors.lastname: Temel
mesh_terms: D018759:Adrenergic Uptake Inhibitors / Q000627:therapeutic use*; D000818:Animals; D004195:Disease Models, Animal; D006816:Huntington Disease / Q000150:complications / Q000188:drug therapy*; D006948:Hyperkinesis / Q000150:complications / Q000188:drug therapy*; D008297:Male; D051381:Rats; D055647:Rats, Transgenic; D013747:Tetrabenazine / Q000627:therapeutic use*
chemical_list: D018759:Adrenergic Uptake Inhibitors; D013747:Tetrabenazine
keywords: Chorea; Dopamine; Huntington's disease; Tetrabenazine; Transgenic rat model