Huntington Disease Questions & Answers

1. Huntington G. On chorea. Med Surg Report. 1872. 26:320.

2. Folstein SE. Huntington's Disease: A Disorder of Families. The Johns Hopkins University Press. 1989.

3. Wexler NS, Lorimer J, Porter J, Gomez F, Moskowitz C, Shackell E, et al. Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. Proc Natl Acad Sci U S A. Mar 9 2004. 101(10):3498-503. [QxMD MEDLINE Link].

4. Wexler NS, Young AB, Tanzi RE, Travers H, Starosta-Rubinstein S, Penney JB, et al. Homozygotes for Huntington's disease. Nature. Mar 12-18 1987. 326(6109):194-7. [QxMD MEDLINE Link].

5. Ho A, Hocaoglu M. Impact of Huntington's across the entire disease spectrum: the phases and stages of disease from the patient perspective. Clin Genet. Sep 2011. 80(3):235-239. [QxMD MEDLINE Link].

6. Loy CT, McCusker EA. Is a motor criterion essential for the diagnosis of clinical huntington disease?. PLoS Curr. Apr 11 2013. 5. [QxMD MEDLINE Link]. [Full Text].

7. Cleret de Langavant L, Fénelon G, Benisty S, Boissé MF, Jacquemot C, Bachoud-Lévi AC. Awareness of Memory Deficits in Early Stage Huntington's Disease. PLoS One. 2013. 8(4):e61676. [QxMD MEDLINE Link]. [Full Text].

8. Nucifora FC Jr, Sasaki M, Peters MF, et al. Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science. Mar 23 2001. 291(5512):2423-8. [QxMD MEDLINE Link].

9. Graham RK, Deng Y, Slow EJ, Haigh B, Bissada N, Lu G. Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell. Jun 16 2006. 125(6):1179-91. [QxMD MEDLINE Link].

10. Stober T, Wussow W, Schimrigk K. Bicaudate diameter--the most specific and simple CT parameter in the diagnosis of Huntington's disease. Neuroradiology. 1984. 26(1):25-8. [QxMD MEDLINE Link].

11. S. E. Folstein. Huntington's Disease. A Disorder of Families. Baltimore, MD: Johns Hopkins University Press; 1989.

12. R. Avila-Giron. Medical and Social Aspects of Huntington's Chorea in the State of Zulia, Venezuela. A. Barbeau, T.N. Chase and G.W. Paulson. Advances in Neurology. New York: Raven Press; 1973. Volume 1: 261-266.

13. Pridmore SA. The prevalence of Huntington's disease in Tasmania. Med J Aust. 1990 Aug 6. 153 (3):133-4. [QxMD MEDLINE Link].

14. Pringsheim T, Wiltshire K, Day L, Dykeman J, Steeves T, Jette N. The incidence and prevalence of Huntington's disease: a systematic review and meta-analysis. Mov Disord. 2012 Aug. 27 (9):1083-91. [QxMD MEDLINE Link].

15. Quaid KA. Presymptomatic testing for Huntington disease in the United States. Am J Hum Genet. Sep 1993. 53(3):785-7. [QxMD MEDLINE Link].

16. Furr Stimming E, Claassen DO, Kayson E, and the, Huntington Study Group KINECT-HD Collaborators. Safety and efficacy of valbenazine for the treatment of chorea associated with Huntington's disease (KINECT-HD): a phase 3, randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2023 Jun. 22 (6):494-504. [QxMD MEDLINE Link].

17. Ondo WG, Tintner R, Thomas M, Jankovic J. Tetrabenazine treatment for Huntington's disease-associated chorea. Clin Neuropharmacol. Nov-Dec 2002. 25(6):300-2. [QxMD MEDLINE Link].

18. Huntington Study Group, Frank S, Testa CM, Stamler D, Kayson E, Davis C, et al. Effect of Deutetrabenazine on Chorea Among Patients With Huntington Disease: A Randomized Clinical Trial. JAMA. 2016 Jul 5. 316 (1):40-50. [QxMD MEDLINE Link]. [Full Text].

19. Racette BA, Perlmutter JS. Levodopa responsive parkinsonism in an adult with Huntington's disease. J Neurol Neurosurg Psychiatry. Oct 1998. 65(4):577-9. [QxMD MEDLINE Link].

20. Hughes S. New Guidelines on Neuropsychiatric Symptoms in Huntington’s. Medscape Medical News. Available at https://www.medscape.com/viewarticle/908855. February 8, 2019; Accessed: February 26, 2019.

21. Anderson KE, van Duijn E, Craufurd D, Drazinic C, Edmondson M, Goodman N, et al. Clinical Management of Neuropsychiatric Symptoms of Huntington Disease: Expert-Based Consensus Guidelines on Agitation, Anxiety, Apathy, Psychosis and Sleep Disorders. J Huntingtons Dis. 2018. 7 (3):355-366. [QxMD MEDLINE Link].

Fredy J Revilla, MD, FAAN, FANA Clinical Professor, University of South Carolina School of Medicine, Greenville; Chief, Division of Neurology, UMG Neuroscience Associates, Prisma Health-Upstate

Fredy J Revilla, MD, FAAN, FANA is a member of the following medical societies: American Academy of Neurology, American Neurological Association, Huntington Study Group, International Parkinson and Movement Disorder Society, Parkinson Study Group, Society for Neuroscience

Disclosure: Nothing to disclose.

Jaime Grutzendler, MD Assistant Professor, Department of Neurology and Physiology, Northwestern University School of Medicine

Jaime Grutzendler, MD is a member of the following medical societies: American Academy of Neurology, Society for Neuroscience

Disclosure: Nothing to disclose.

Travis R Larsh Department of Neurology, University of Cincinnati College of Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Bioserenity, Ceribell, Eisai, Jazz, LivaNova, Neurelis, Neuropace, SK life science, Sunovion, Takeda, UCB<br/>Serve(d) as a speaker or a member of a speakers bureau for: Aquestive, Bioserenity, Eisai, Jazz, LivaNova, Neurelis, SK life science, Stratus, Sunovion, UCB<br/>Received research grant from: Cerevel Therapeutics, Ovid Therapeutics, Neuropace, Greenwich Jazz, SK Life Science, Xenon Pharmaceuticals, UCB, Marinus, Neuroelectrics Corporation, Longboard, Janssen, Equilibre Biopharmaceuticals.

Robert A Hauser, MD, MBA Professor of Neurology, Molecular Pharmacology and Physiology, Director, USF Parkinson's Disease and Movement Disorders Center, National Parkinson Foundation Center of Excellence, Byrd Institute, Clinical Chair, Signature Interdisciplinary Program in Neuroscience, University of South Florida College of Medicine

Robert A Hauser, MD, MBA is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Society of Neuroimaging, International Parkinson and Movement Disorder Society

Disclosure: Received consulting fee from Cerecor for consulting; Received consulting fee from L&M Healthcare for consulting; Received consulting fee from Cleveland Clinic for consulting; Received consulting fee from Heptares for consulting; Received consulting fee from Gerrson Lehrman Group for consulting; Received consulting fee from Indus for consulting; Received consulting fee from University of Houston for consulting; Received consulting fee from AbbVie for consulting; Received consulting fee from Adama.

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