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: Dagmar H Zeef, Ali Jahanshahi, Rinske Vlamings, João Casaca-Carreira, Remco G Santegoeds, Marcus L F Janssen, Mayke Oosterloo, Yasin Temel
• Journal: Behavioural brain research (Behav Brain Res) Vol. 262 Pg. 31-4 (Apr 01 2014) ISSN: 1872-7549 [Electronic] Netherlands
• PMID: 24412685 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2014 Elsevier B.V. All rights reserved.

Chemical References

• Adrenergic Uptake Inhibitors
• Tetrabenazine

Topics

• Adrenergic Uptake Inhibitors (therapeutic use)
• Animals
• Disease Models, Animal
• Huntington Disease (complications, drug therapy)
• Hyperkinesis (complications, drug therapy)
• Male
• Rats
• Rats, Transgenic
• Tetrabenazine (therapeutic use)