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Clinical care and management
J13 Prescription usage for treatment of irritability, perseverative behaviors, and chorea in huntington's disease
  1. E van Duijn1,
  2. M Groves2,
  3. D Craufurd3,
  4. K Anderson4,
  5. M Guttman5,
  6. E Wexler6,
  7. S Perlman6,
  8. A Rosenblatt7,
  9. D P van Kammen8,
  10. J Giuliano8,
  11. J-M Burgunder9,
  12. N Goodman10,11,
  13. L Goodman11
  1. 1Leiden University Medical Center, Psychiatry, Leiden, Netherlands
  2. 2Beth Israel Medical Center, Psychiatry, New York, USA
  3. 3University of Manchester, Clinical Genetics, Manchester, UK
  4. 4University of Maryland, Baltimore, Maryland, USA
  5. 5University of Toronto, Toronto, Ontario, Canada
  6. 6University of California-Los Angeles, Los Angeles, California, USA
  7. 7Johns Hopkins University, Department of Psychiatry, Baltimore, Maryland, USA
  8. 8Cure Huntington's Disease Initiative (CHDI), Princeton, New Jersey, USA
  9. 9Neurologische Klinik und Poliklinik, Universitätsspital Bern, Switzerland
  10. 10Institute for Systems Biology, Seattle, Washington, USA
  11. 11Huntington's Disease Drug Works, Seattle, Washington, USA


Background Despite large gaps in evidence-based knowledge, clinical experience supports the use of pharmacologic treatments for many symptoms of Huntington's disease (HD).

Aims The project goal is to develop consensus guidelines based on expert clinical experience to improve quality of HD care.

Methods The survey was developed by 9 international experts, and designed as a highly iterative and systematic method of soliciting expert opinions on the pharmacologic treatment of irritability, perseverative behaviors, and chorea in HD. Fifty-five experts from Australia, Europe and North America responded to at least one of the surveys.

Results For irritability, SSRI was first choice of 58%, an antipsychotic was first choice for 22%, a mood stabilizing anticonvulsant 14%, and benzodiazepine 2%. For perseverative behaviors, SSRI was first choice of 75%, an antipsychotic choice of 4%, a mood stabilizing anticonvulsant choice of 6%, and clomipramine 2%. The remaining 13% chose to qualify the response to include 2 first choices. For chorea, an antipsychotic was first choice for 56%, tetrabenazine 15%, amantadine 6%, and benzodiazepines 4%. The remaining 13% chose to qualify the response to include 2 first choices. Drug choice for use as adjunctive therapy was widely variable.

Conclusions Many areas of variability in treatment for irritability, perseverative behaviors, and chorea in HD, have been identified. Results will guide future rounds of the Delphi process to elicit rational causes for differences identified. When complete, this process will clarify useful treatment paradigms that will improve patient care and identify areas in which clinical trials would be most useful.

  • Treatment
  • guidelines
  • irritability
  • perseverative behaviors
  • chorea

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