What it is
Huntington disease is an inherited (autosomal dominant) neurodegenerative brain disease that progressively affects movement, thinking, and behaviour, with onset usually in adulthood. It is caused by an expanded CAG repeat in the HTT gene.
Signs and symptoms
Chorea
Chorea, brief involuntary jerking or writhing movements, is the most common movement-disorder feature of Huntington disease.
Depression
Depression is one of the significant behavioural features of Huntington disease and affects quality of life.
Irritability
Irritability is a common behavioural feature of Huntington disease.
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
Deutetrabenazine
Deutetrabenazine is an oral medicine that reduces chorea in Huntington disease by inhibiting the vesicular monoamine transporter 2 (VMAT2). It treats the symptom, not the underlying disease.
Used to help with: Chorea.
“Deutetrabenazine was recently approved for the treatment of chorea in Huntington's disease (HD) and is the first deuterated medication that has been US Food and Drug Administration (FDA)-approved for therapeutic use.”
Tetrabenazine
Tetrabenazine is the original vesicular monoamine transporter 2 (VMAT2) inhibitor used to reduce chorea in Huntington disease, treating the symptom rather than the disease itself.
Used to help with: Chorea.
“Deutetrabenazine is the first deuterated drug and second drug after tetrabenazine, the classic vesicular monoamine transporter type 2 (VMAT2) inhibitor, to receive approval for the treatment of chorea associated with HD.”
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How to read the evidence labels
Where this comes from
This guide is built from 3 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.