Chorea, a characteristic manifestation of Huntington’s disease, refers to uncontrolled “dance-like” movements in the fingers, feet, face, or torso. These movements may worsen in times of heightened emotion or stress, or as the disease progresses. Chorea can interfere with sleep, swallowing, and other daily acts. Therefore, improving chorea can contribute to sustained quality-of-life benefits for those affected. According to a recent news release from biopharmaceutical company Neurocrine Biosciences, Inc. (“Neurocrine”), the U.S. FDA approved INGREZZA (valbenazine) for the treatment of adults with chorea due to Huntington’s disease.
What is INGREZZA for Chorea?
First approved in 2017 to treat tardive dyskinesia, INGREZZA is a selective vesicular monoamine transporter 2 (VMAT2) inhibitor. Administered via once-daily 40mg, 60mg, or 80mg capsules, INGREZZA selectively targets VMAT2 and inhibits dopamine release. Researchers believe that INGREZZA stops excess dopamine signaling and thus extra movement.
INGREZZA was approved for Huntington’s disease-associated chorea following data from the Phase 3 KINECT®-HD trial and the ongoing KINECT®-HD2 open-label extension (OLE) trial. These studies aimed to identify how safe, effective, and well-tolerated the treatment was. 128 participants between ages 18-75 enrolled in the KINECT®-HD trial. INGREZZA significantly improved chorea severity when compared to a placebo, with an average reduction of 40%. Approximately half of participants also saw even greater improvement within just 3 months of beginning treatment, and chorea improvements began as early as week 2. More than 150 participants will enroll in the OLE to further understand the long-term safety, efficacy, and tolerability.
While INGREZZA is safe and generally well-tolerated, adverse reactions have occurred. Excessive sleepiness, rashes, insomnia, hives, angioedema, and irregular heart rate have all emerged as potential side effects. Doctors caution that INGREZZA use can also increase the risk of depression and suicidal ideation. If you are using INGREZZA and experience worsening mood, please speak with your doctors. If you need assistance in acquiring or affording INGREZZA, please check here.
Understanding Huntington’s Disease (HD)
Caused by HTT gene mutations, Huntington’s disease is a rare and progressive neurodegenerative disorder. Normally, HTT encodes for the production of a protein called huntingtin; doctors believe this protein contributes to neuronal health. When the gene becomes mutated, abnormal and toxic huntingtin protein accumulates. This causes neurons to break down and die, leading to physical and mental deterioration.
An estimated 3-7 out of every 100,000 people will have Huntington’s disease. It typically manifests in the 30s and 40s, and people often live for 15-20 years following diagnosis. While Huntington’s disease can occur in children, known as juvenile HD, it is exceedingly rare – and often more severe.
Symptoms and manifestations of Huntington’s disease, outside of chorea, may include:
- Dystonia
- Tremors
- Cognitive decline such as difficulty learning new things, remembering facts, or putting thoughts into words
- Changes in mood, behavior, and personality
- This may manifest as mood swings, anxiety and depression, impulsivity, or apathy.
- Difficulty speaking, eating, or swallowing
- Slurred speech
- Problems with balance and coordination
- Difficulty walking and/or frequent tripping and falling
- Unintentional weight loss
- Chest infections
- Insomnia
- Lethargy and fatigue
- Seizures
Right now, there is no cure for Huntington’s disease and no way to reverse its symptoms. Treatments like INGREZZA work to reduce chorea and manage other symptoms. Continued research is needed to identify the best way to support people with this condition.
