Huntington's Disease (HD)

  1. what are the goal of treatment for HD?

    1. symptomatic control

  2. What are the 2 FDA-approved VMAT-2i medications to treat Chorea in patients with HD?

    1. Tetrabenazine (TBZ) & Deutetrabenazine (dTBZ)

  3. What are the first-line treatment options for chorea in a patient with HD?

    1. Deutetrabenazine, Tetrabenazine, SGAs

  4. What are the first-line treatment options for Dystonia in a patient with HD?

    1. Decrease dose of SGA and/or VMAT-2 inhibitor

  5. What are the first-line treatment options for Myoclonus in a patient with HD?

    1. Sodium valproate & Clonazepam

  6. Which VMAT-2i has an FDA indication for both Tardive dyskinesia & Chorea (Huntington’s Disease)?

    1. Deutetrabenazine

  7. Deutetrabenazine is contraindicated in patients who are______.

    1. hepatically impaired

  8. if a patient’s therapy is interrupted for more than 1 week while on Deutetrabenazine, what do you do?

    1. re-titrate therapy

  9. if a patient is taking Tetrabenazine and their therapy is interupted when would they need to re-titrate therapy?

    1. if interuption last more than 5 days

  10. What are common VMAT2-i ADEs?

    1. n/v, dry mouth, Sedation/somnolence, fatigue, insomnia, and anxiety

  11. Which VMAT-2i has more ADEs?

    1. Tetrabenazine

  12. What are serious ADEs?

    1. Orthostatic hypotension

      • Prolonged QT interval

      • Syncope

      • Hyperprolactinemia

      • Dysphagia

      • Neuroleptic malignant

      syndrome

      • Akathisia* (up to 20%)

      • Parkinsonism*

  13. What is the BBW for VMAT2-is?

    1. depression and suicidal thoughts

  14. What are the extrapyramidal ADEs?

    1. dyskinesias, tardive dyskinesia, akathisia, dystonia and parksonism

  15. What is Dyskinesia?

    1. Repetitive, involuntary, purposeless body or facial movements

  16. A patient repeatedly Lip smacking or sticking out their tongue indicates while on a VMAT2i:

    1. dyskinesia

  17. what is Akathisia?

    1. Extreme form of internal or external restlessness, inability to sit still, urge to move constantly

  18. what is Dystonia?

    1. Muscle tension disorder → strong muscle contractions, unusual twisting of parts of body especially neck

  19. What is parkinsonism?

    1. Mask-like facies, resting tremor, cogwheel rigidity, shuffling gait, bradykinesia

  20. What are common DDIs with VMAT inhibitors?

    1. Strong CYP2D6 inhibitors, QT-prolonging medications, and SGAs

  21. What are the names of the SGAs

    1. Aripiprazole, Clozapine, Olanzapine, Quetiapine, Risperidone, Lurasidone, etc

  22. What is the MOA for SGAs?

    1. causes DA receptor antagonism which attenuates the hypersensitive dopamine receptors in HD

  23. A patient experiencing acute agitation should take_____ or_____.

    1. Antipsychotic or Benzodiazepine

  24. A patient experiencing chronic agitation should take_____ or_____.

    1. Antipsychotic or mood stablizer

  25. A patient experiencing anxiety should take_____ as a first line treatment or ______if they have comorbid insomnia.

    1. SSRI or Mirtazepine

  26. A patient experiencing apathy should take_____ as a first line treatment.

    1. antidepressant

  27. A patient experiencing depression should take_____,_____, or_____ if they have comorbid insomnia

    1. SSRI, SNRI, or mirtazapine

  28. A patient experiencing psychosis should take an_____ .

    1. Antipsychotic