Huntington Disease Dementia Treatment & Management

  • Author: Idan Sharon, MD; Chief Editor: David Bienenfeld, MD  more...
Updated: Apr 18, 2014

Medical Care

Acetylcholinesterase inhibitors (eg, rivastigmine, memantine) may have positive effects on cognition, although no treatment halts the progression of this illness. A recent review detailing all studies investigating the effectiveness of acetylcholinesterase inhibitors revealed that there is little evidence of the benefit of such medications.[11]

Symptomatic treatment is aimed at minimizing the distressing movements. Pharmacological intervention is available for the behavior and/or psychologic disturbances, chorea, and weight loss. Psychologic symptoms may require major antipsychotic drugs for control. Treatment for patients with depression is used to improve mood, functional status, and quality of life. Research has shed greater understanding on the disease mechanism; however, promising avenues in gene therapy and neurotransplantation are still only in their incipient stages.

  • Cognitive impairment
    • Patients who are cognitively impaired require a multidisciplinary treatment approach, which must be based on a solid alliance with the patient and family.
    • Ongoing assessment should include periodic monitoring of the development and evolution of cognitive and noncognitive psychiatric symptoms and their response to intervention.
    • Safety measures include (1) evaluation of suicidal tendency and the potential for violence; (2) recommendations regarding providing adequate supervision, preventing falls, and limiting the hazards of wandering; (3) vigilance regarding neglect or abuse; and (4) restrictions on driving and the use of other dangerous equipment.
    • Also, helping patients and their families plan for financial and legal issues is important.
  • Psychosis
    • Intervention should be guided by the patient's level of distress and risk to the patient or caregivers.
    • In addition to distress, if agitation, combativeness, or violent behavior is causing danger to the patient or others, psychopharmacologic treatment is indicated with atypical neuroleptics and mood stabilizers (anticonvulsants known as GABA agents).

Surgical Care

One experimental strategy that may offer hope in the neurodegenerative disorder of HD has been neural transplantation. Fetal human striatal implants to replace lost neurons and/or prevent the degeneration of neurons destined to die most likely will be the first transplantation strategy attempted in clinical trials.

  • A study conducted in France examined whether grafts of human fetal striatal tissue could survive and have detectable effects in 5 patients with mild-to-moderate HD.[12]
    • After 2 years of preoperative assessment, patients were grafted with human fetal neuroblasts into the right striatum then, after a year, the left striatum.
    • Final results were assessed 1 year later on the basis of neurological, neuropsychological, neurophysiological, and psychiatric test results.
    • The final position emission tomography scan assessment showed increased metabolic activity in various subnuclei of the striatum in 3 of 5 patients, contrasting with the progressive decline recorded in the other 2 patients in the series, as seen in patients with untreated HD. Motor and cognitive functions were improved or maintained within the normal range, and functional benefits were seen in activities of daily living in these 3 patients but not in the other 2.
    • Fetal neural allografts could be associated with functional, motor, and cognitive improvements in patients with HD.


Consultation with social service agency personnel is warranted. As the patient's dependency increases, caregivers may begin to feel more burdened. Families should be counseled regarding when to consider and plan for additional support at home or for possible transfer to a long-term care facility. A referral for some form of respite care (eg, home health aid, daycare, brief nursing home stay) with the help of social service agency personnel may be helpful.

Contributor Information and Disclosures

Idan Sharon, MD Consulting Staff, Departments of Neurology and Psychiatry, Cornell New York Methodist Hospital; Private Practice

Idan Sharon, MD is a member of the following medical societies: American Academy of Neurology, Medical Society of the State of New York

Disclosure: Nothing to disclose.


Tulay Ersan, MD Chief of Geriatrics, Department of Internal Medicine, Division of Geriatrics, Monmouth Medical Center

Tulay Ersan, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Geriatrics Society, American Medical Association

Disclosure: Nothing to disclose.

Roni Sharon, MD Fellow, Department of Neurology, Brigham and Women's Hospital, Harvard Medical School

Roni Sharon, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Headache Society, International Headache Society

Disclosure: Nothing to disclose.

Jaclyn P Wilkens Hofstra University

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Chief Editor

David Bienenfeld, MD Professor, Departments of Psychiatry and Geriatric Medicine, Wright State University, Boonshoft School of Medicine

David Bienenfeld, MD is a member of the following medical societies: American Medical Association, American Psychiatric Association, Association for Academic Psychiatry

Disclosure: Nothing to disclose.

Additional Contributors

Alan D Schmetzer, MD Professor Emeritus, Department of Psychiatry, Indiana University School of Medicine

Alan D Schmetzer, MD is a member of the following medical societies: American Academy of Addiction Psychiatry, American Academy of Clinical Psychiatrists, American Academy of Psychiatry and the Law, American Association for Physician Leadership, American Medical Association, American Psychiatric Association, International Society for ECT and Neurostimulation, American Neuropsychiatric Association

Disclosure: Nothing to disclose.

  1. Lipe H, Bird T. Late onset Huntington Disease: clinical and genetic characteristics of 34 cases. J Neurol Sci. 2009 Jan 15. 276(1-2):159-62. [Medline].

  2. Quintanilla RA, Johnson GV. Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease. Brain Res Bull. 2009 Oct 28. 80(4-5):242-7. [Medline]. [Full Text].

  3. Robins Wahlin TB. To know or not to know: a review of behaviour and suicidal ideation in preclinical Huntington's disease. Patient Educ Couns. 2007 Mar. 65(3):279-87. [Medline].

  4. Driver-Dunckley E, Caviness JN. Huntington's Disease. Schapira AHV. Neurology and Clinical Neuroscience. Elsevier; 2007. 67: 879-885.

  5. Jason GW, Suchowersky O, Pajurkova EM, et al. Cognitive manifestations of Huntington disease in relation to genetic structure and clinical onset. Arch Neurol. 1997 Sep. 54(9):1081-8. [Medline].

  6. van Duijn E, Reedeker N, Giltay EJ, Roos RA, van der Mast RC. Correlates of apathy in Huntington's disease. J Neuropsychiatry Clin Neurosci. 2010 Summer. 22(3):287-94. [Medline].

  7. Solomon AC, Stout JC, Johnson SA, Langbehn DR, Aylward EH, Brandt J, et al. Verbal episodic memory declines prior to diagnosis in Huntington's disease. Neuropsychologia. 2007 Apr 9. 45(8):1767-76. [Medline]. [Full Text].

  8. Peavy GM, Jacobson MW, Kane AE, Goldstein JL, Mickes L, Lessig S, et al. Proposed Criteria for the Diagnosis of Dementia Associated With Huntington's Disease (HD). Neurotherapeutics. 2008. 5:373.

  9. Stahl SM, Thiemann S, Faull KF, Barchas JD, Berger PA. Neurochemistry of dopamine in Huntington's dementia and normal aging. Arch Gen Psychiatry. 1986 Feb. 43(2):161-4. [Medline].

  10. Garcia Ruiz PJ, Mena MA, Sanchez Bernardos V, Diaz Neira W, Gimenez Roldan S, Benitez J, et al. Cerebrospinal fluid homovanillic acid is reduced in untreated Huntington's disease. Clin Neuropharmacol. 1995 Feb. 18(1):58-63. [Medline].

  11. Vattakatuchery JJ, Kurien R. Acetylcholinesterase inhibitors in cognitive impairment in Huntington's disease: A brief review. World J Psychiatry. 2013 Sep 22. 3(3):62-4. [Medline]. [Full Text].

  12. Bachoud-Levi AC, Remy P, Nguyen JP, Brugieres P, Lefaucheur JP, Bourdet C, et al. Motor and cognitive improvements in patients with Huntington's disease after neural transplantation. Lancet. 2000 Dec 9. 356(9246):1975-9. [Medline].

  13. Adam OR, Jankovic J. Symptomatic treatment of Huntington disease. Neurotherapeutics. 2008 Apr. 5(2):181-97. [Medline].

  14. Ranen NG, Lipsey JR, Treisman G, Ross CA. Sertraline in the treatment of severe aggressiveness in Huntington's disease. J Neuropsychiatry Clin Neurosci. 1996 Summer. 8(3):338-40. [Medline].

  15. Robins Wahlin TB. To know or not to know: a review of behaviour and suicidal ideation in preclinical Huntington's disease. Patient Educ Couns. 2007 Mar. 65(3):279-87. [Medline].

  16. Bombard Y, Veenstra G, Friedman JM, Creighton S, Currie L, Paulsen JS, et al. Perceptions of genetic discrimination among people at risk for Huntington's disease: a cross sectional survey. BMJ. 2009 Jun 9. 338:b2175. [Medline]. [Full Text].

  17. Coulson NS, Buchanan H, Aubeeluck A. Social support in cyberspace: a content analysis of communication within a Huntington's disease online support group. Patient Educ Couns. 2007 Oct. 68(2):173-8. [Medline].

  18. [Guideline] American Psychiatric Association. Practice guideline for the treatment of patients with Alzheimer's disease and other dementias of late life. Am J Psychiatry. 1997 May. 154(5 Suppl):1-39. [Medline].

  19. Bamford KA, Caine ED, Kido DK, Cox C, Shoulson I. A prospective evaluation of cognitive decline in early Huntington's disease: functional and radiographic correlates. Neurology. 1995 Oct. 45(10):1867-73. [Medline].

  20. Barami K, Hutchins KD, Lyman WD. Neurotransmitter distribution in the second trimester fetal human corpus striatum. Neurol Res. 2001 Jan. 23(1):16-22. [Medline].

  21. Barnoy S, Tabak N. Israeli nurses and genetic information disclosure. Nurs Ethics. 2007 May. 14(3):280-94. [Medline].

  22. Biglan K, Shoulson I. Juvenile-onset huntington disease: a matter of perspective. Arch Neurol. 2007 Jun. 64(6):783-4. [Medline].

  23. Chou KL, Borek LL, Friedman JH. The management of psychosis in movement disorder patients. Expert Opin Pharmacother. 2007 May. 8(7):935-43. [Medline].

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

  25. Folstein SE. The psychopathology of Huntington's disease. Res Publ Assoc Res Nerv Ment Dis. 1991. 69:181-91. [Medline].

  26. Frank S, Biglan K. Long-term fetal cell transplant in Huntington disease: stayin' alive. Neurology. 2007 Jun 12. 68(24):2055-6. [Medline].

  27. Gilroy J. Basic Neurology. 3rd ed. New York, NY: McGraw-Hill; 2000.

  28. Gustavson AR, Cummings JL. Cholinesterase inhibitors in non-Alzheimer dementias. J Am Med Dir Assoc. 2003 Nov-Dec. 4(6 Suppl):S165-9. [Medline].

  29. Hahn-Barma V, Deweer B, Durr A, Dode C, Feingold J, Pillon B, et al. Are cognitive changes the first symptoms of Huntington's disease? A study of gene carriers. J Neurol Neurosurg Psychiatry. 1998 Feb. 64(2):172-7. [Medline].

  30. Hakimian R. Disclosure of Huntington's disease to family members: the dilemma of known but unknowing parties. Genet Test. 2000. 4(4):359-64. [Medline].

  31. Harper P. Huntington's disease. Harper P, ed. Major Problems of Neurology. 2nd ed. Philadelphia, Pa: WB Saunders; 1996.

  32. Harris GJ, Codori AM, Lewis RF, Schmidt E, Bedi A, Brandt J. Reduced basal ganglia blood flow and volume in pre-symptomatic, gene-tested persons at-risk for Huntington's disease. Brain. 1999 Sep. 122 ( Pt 9):1667-78. [Medline].

  33. Hodges JR, Salmon DP, Butters N. Differential impairment of semantic and episodic memory in Alzheimer's and Huntington's diseases: a controlled prospective study. J Neurol Neurosurg Psychiatry. 1990 Dec. 53(12):1089-95. [Medline]. [Full Text].

  34. Kallail KJ, Godfrey NE, Suter G, Anthimides L. A multidisciplinary approach to the management of Huntington's disease. Kans Med. 1989 Nov. 90(11):309-11. [Medline].

  35. Kirkwood SC, Su JL, Conneally P, Foroud T. Progression of symptoms in the early and middle stages of Huntington disease. Arch Neurol. 2001 Feb. 58(2):273-8. [Medline].

  36. Kremer B, Goldberg P, Andrew SE, Theilmann J, Telenius H, Zeisler J, et al. A worldwide study of the Huntington's disease mutation. The sensitivity and specificity of measuring CAG repeats. N Engl J Med. 1994 May 19. 330(20):1401-6. [Medline].

  37. Marder K, Zhao H, Myers RH, Cudkowicz M, Kayson E, Kieburtz K, et al. Rate of functional decline in Huntington's disease. Huntington Study Group. Neurology. 2000 Jan 25. 54(2):452-8. [Medline].

  38. Mayeux R, ed. Dementias: Advances in Neurology. New York, NY: Raven; 1983. 38 Vol:

  39. Meiser B, Dunn S. Psychological impact of genetic testing for Huntington''s disease: an update of the literature. J Neurol Neurosurg Psychiatry. 2000 Nov. 69(5):574-8. [Medline].

  40. Nance MA. Huntington disease: clinical, genetic, and social aspects. J Geriatr Psychiatry Neurol. 1998 Summer. 11(2):61-70. [Medline].

  41. Nehl C, Paulsen JS. Cognitive and psychiatric aspects of Huntington disease contribute to functional capacity. J Nerv Ment Dis. 2004 Jan. 192(1):72-4. [Medline].

  42. Ramaswamy S, Shannon KM, Kordower JH. Huntington's disease: pathological mechanisms and therapeutic strategies. Cell Transplant. 2007. 16(3):301-12. [Medline].

  43. Ribai P, Nguyen K, Hahn-Barma V, Gourfinkel-An I, Vidailhet M, Legout A, et al. Psychiatric and cognitive difficulties as indicators of juvenile huntington disease onset in 29 patients. Arch Neurol. 2007 Jun. 64(6):813-9. [Medline].

  44. Rosenstein LD. Differential diagnosis of the major progressive dementias and depression in middle and late adulthood: a summary of the literature of the early 1990s. Neuropsychol Rev. 1998 Sep. 8(3):109-67. [Medline].

  45. Sharma P, Savy L, Britton J, Taylor R, Howick A, Patton M. Huntington's disease: a molecular genetic and CT comparison. J Neurol Neurosurg Psychiatry. 1996 Feb. 60(2):206-8. [Medline].

  46. Sorensen SA, Fenger K, Olsen JH. Significantly lower incidence of cancer among patients with Huntington disease: An apoptotic effect of an expanded polyglutamine tract?. Cancer. 1999 Oct 1. 86(7):1342-6. [Medline].

  47. Starkstein SE, Brandt J, Bylsma F, Peyser C, Folstein M, Folstein SE. Neuropsychological correlates of brain atrophy in Huntington's disease: a magnetic resonance imaging study. Neuroradiology. 1992. 34(6):487-9. [Medline].

  48. Swash M. Clinical Neurology. New York, NY: Churchill Livingstone; 1991.

  49. Tian J, Herdman SJ, Zee DS, Folstein SE. Postural stability in patients with Huntington's disease. Neurology. 1992 Jun. 42(6):1232-8. [Medline].

  50. Zakzanis KK. The subcortical dementia of Huntington's disease. J Clin Exp Neuropsychol. 1998 Aug. 20(4):565-78. [Medline].

Medscape Consult
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.