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Updated: Feb 25 2018

Huntington Disease Drugs

  • Snapshot
    • A 53-year-old man presents with involuntary jerking movement of the arms. This is worsened with voluntary movement and appears to be nonrepetitive. He tries to incorporate these movements into voluntary movement. He states that this is negatively affecting his quality of life, as he has difficulty with activities of daily living. Family history is significant for Huntington's disease in the father. On physical examination, there is impaired fine motor movements and choreiform movement of the upper extremity. He is started on tetrabenazine.
  • Introduction
    • Huntington's disease (HD)
      • autosomal dominant neurodegenerative disorder
        • secondary to CAG repeat on chromosome 4
        • characterized by chorea, psychiatric disturbance, and cognitive decline
      • no known cure; therefore, treatment is symptomatic
      • changes in neurotransmitters with HD
        • ↑ dopamine, ↓ GABA, and ↓ACh
          • ↑ dopamine can lead to
            • hyperkinesis (via nigro-striatal pathway)
            • psychosis (via meso-limbic pathway)
    • Medications
      • treatment for chorea
        • tetrabenazine
          • vesicular monoamine transporter (VMAT) inhibitor
            • impairs monoamine uptake into vesicles (e.g., dopamine, norepinephrine, and serotonin)
      • treatment for psychiatric and behavioral symptoms
        • psychosis/agitation
          • antipsychotics
            • e.g., haloperidol (typical) and risperidone (atypical)
              • dopamine-2 receptor antagonist
        • depression
          • serotonin re-uptake inhibitors (SSRIs)
          • tricyclic antidepressants (TCAs)
            • inhibits re-uptake of norepinephrine and serotonin
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