Chorea: Treatment Update

Chorea: Treatment Update

1. Chorea: Treatment Update Ade Wijaya, MD – November 2019

2. Introduction Chorea is a hyperkinetic movement disorder consisting of rapid dance-like involuntary movements that flow from one area of the body to another Dysfunction of the basal ganglia is a core feature of the pathophysiology of chorea Hermann A, Walker RH. Diagnosis and treatment of chorea syndromes. Curr Neurol Neurosci Rep. 2015;15 Mink JW. The basal ganglia and involuntary movements: impaired inhibition of competing motor patterns. Arch Neurol. 2003;60:1365–8

3. Mink JW. The basal ganglia and involuntary movements: impaired inhibition of competing motor patterns. Arch Neurol. 2003;60:1365–8

4. Dopamine-depleting Agents Tetrabenazine Start with a daily dose of 12.5 mg, increased weekly as tolerated to three to four doses a day Adverse events: parkinsonism , weight loss, dysphagia, and depression Effective for chorea due to a variety of etiologies and can be especially useful if dystonia is an additional feature Feinstein E, Walker R. An Update on the Treatment of Chorea. Current treatment options in neurology. 2018 Oct 1;20(10):44.

5. Dopamine-depleting Agents Deutetrabenazine Initiated at a dose of 6 mg/ day and increased to approximately 40 mg/day Adverse events : sedation, dry mouth, anxiety, depression Less potential for developing the side effects related to Tetrabenazine Frank S, Testa CM, Stamler D, et al. Effect of Deutetrabenazine on chorea among patients with Huntington disease: a randomized clinical trial. JAMA. 2016;316:40–50 . Fernandez HH, Factor SA, Hauser RA, Jimenez-Shahed J, Ondo WG, Jarskog LF, et al. Randomized controlled trial of deutetrabenazine for tardive dyskinesia: the ARM-TD study. Neurology. 2017;88:2003–10 . Anderson KE, Stamler D, Davis MD, Factor SA, Hauser RA, Isojärvi J, et al. Deutetrabenazine for treatment of involuntary movements in patients with tardive dyskinesia (AIM-TD): a double-blind, randomised, placebocontrolled, phase 3 trial. The lancet Psychiatry. 2017;4:595–604.

6. Dopamine-depleting Agents Valbenazine 40-80 mg / day Adverse events: somnolence , akathisia, and dry mouth Less side effecs Grigoriadis DE, Smith E, Hoare SRJ, Madan A, Bozigian H. Pharmacologic characterization of Valbenazine (NBI-98854) and its metabolites. J Pharmacol Exp Ther. 2017;361:454–61 . Hauser RA, Factor SA, Marder SR, Knesevich MA, Ramirez PM, Jimenez R, et al. KINECT 3: a phase 3 randomized, double-blind, placebo-controlled trial of Valbenazine for tardive dyskinesia. Am J Psychiatry. 2017;174:476–84 . Factor SA, Remington G, Comella CL, Correll CU, Burke J, Jimenez R, et al. The effects of Valbenazine in participants with tardive dyskinesia. J Clin Psychiatry. 2017;78:1344–50.

7. Dopamine D2 receptor-blocking agents Neuroleptics : typical Haloperidol Adverse events: somnolence, parkinsonism, and the potential to cause tardive dyskinesia Neuroleptics : atypical Clozapine 150 mg / day Olanzapine 10-30 mg / day Feinstein E, Walker R. An Update on the Treatment of Chorea. Current treatment options in neurology. 2018 Oct 1;20(10):44.

8. Anticonvulsants Valproic acid: Sydenham’s chorea (15 to 20 mg/kg/day, divided two to three times daily) Carbamazepine: Sydenham’s chorea (15–20 mg/kg/day twice daily ) Benzodiazepines: clonazepam 2–4.5 mg significantly reduced dyskinesia in schizophrenics with tardive dyskinesia Levetiracetam: Huntington disease / tardive dyskinesia (500–3000 mg/day) Feinstein E, Walker R. An Update on the Treatment of Chorea. Current treatment options in neurology. 2018 Oct 1;20(10):44.

9. Anti-glutamatergic Agents Amantadine 100 mg / day Adverse events: hallucinations, confusion, forgetfulness, anxiety/agitation, morbid thoughts, diarrhea, and nausea Riluzole: 200 mg/day Feinstein E, Walker R. An Update on the Treatment of Chorea. Current treatment options in neurology. 2018 Oct 1;20(10):44.

10. Deep Brain Stimulation (DBS) Is a reasonable option for patients with hyperkinetic movement disorders that cannot be adequately controlled with oral medications However , the surgery can worsen some disease features, such as dysarthria, dysphagia, and gait impairment Risks and benefits should be carefully weighed in individual cases, but the procedure is most likely to benefit patients with non-degenerative disorders Feinstein E, Walker R. An Update on the Treatment of Chorea. Current treatment options in neurology. 2018 Oct 1;20(10):44.

11. Summary Symptomatic chorea from infections, structural lesions, or metabolic causes is often self-limited, may resolve with treatment of the primary condition, and does not typically require treatment . Treatment: medications and DBS Multidisciplinary approach

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