“Huntington’s disease is an inherited brain disorder that causes patients to experience uncontrollable jerky movements, as well as changes in personality, behaviour, thinking and memory. There are no FDA-approved treatments for the chorea. Anti-psychotic drugs like haloperidol are commonly used to suppress chorea, but they cause many different side effects. This study shows that tetrabenazine can decrease chorea, and the drug is well-tolerated by most research subjects,” said Dr. Kathleen M. Shannon, neurologist and Huntington’s disease specialist at the Huntington’s Disease Society of America Center of Excellence at Rush University Medical Center.
The symptom that tetrabenazine treats – involuntary, writhing movements of the limbs, face, and sometimes the entire body – is the hallmark symptom of Huntington’s disease, an inherited neurodegenerative disorder that worsens as brain cells known as medium spiny neurons are killed off by a mutant protein.
Thirteen years ago the gene that causes the disease was identified by scientists, and now a simple blood test can tell people whether they will develop the disease or not. But since there is no way known to prevent the disease or slow its progression, and for other reasons as well, many patients decline the test, instead waiting to see if they develop symptoms like the ones they witnessed in a parent.
“Tetrabenazine does not help all the features of Huntington’s disease, and other medications are commonly needed to improve mood and behaviour. For those Huntington’s disease patients whose function is impaired by chorea, however, tetrabenazine can help activities of daily living, and improve functional capacity,” Shannon said.
MEDICA.de; Source: Rush University Medical Center