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This month, the US Food and Drug Administration approved the first drug (Xenazine; tetrabenazine) to treat people with Huntington’s disease (HD). Xenazine is used to reduce the jerky, involuntary movements in people with this disease.
HD is a relatively rare disease, affecting approximately 30,000 people in the US. HD is an inherited neurological disorder: if either parent has it, his or her child has a 50-50 chance of getting it. Symptoms, which usually appear after age 30, include dementia (poor judgment, memory problems, personality changes), chorea (jerky, random movements of the body), poor coordination, depression, and mood swings. Patients often die within 15 years of the onset of symptoms. HD damages neurons in the area of the brain called the basal ganglia, especially the caudate nucleus and globus pallidus. People with HD have overactive dopamine neurotransmitter systems.
Xenazine works by blocking the uptake of the neurotransmitter dopamine into vesicles. Therefore, there is a decrease in the amount of dopamine available to be released into synapses. Side effects of Xenazine include insomnia, depression, drowsiness, restlessness and nausea.
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