Medical Care

Only symptomatic treatment is available for patients with chorea. Chorea may be a disabling symptom, leading to bruises, fractures, and falls, and may impair the ability of patients to feed themselves. In addition, patients sometimes express a desire for antichorea treatment for cosmetic reasons.

The most widely used agents in the treatment of chorea are the neuroleptics. The basis of their mechanism of action is thought to be related to blocking of dopamine receptors. Neuroleptics can be classified as typical and atypical. Typical neuroleptics include haloperidol and fluphenazine. Atypical neuroleptics include risperidone, olanzapine, clozapine, and quetiapine.

Dopamine-depleting agents (eg, reserpine, tetrabenazine, deutetrabenazine, valbenazine) represent another option in the treatment of chorea associated wtih Huntington disease.^{[13, 14, 104, 105]}

GABAergic drugs, such as clonazepam, gabapentin, and valproate,^[106] can be used as adjunctive therapy.

Coenzyme Q10 alone and in combination with minocycline have been proposed as potential therapies and have shown promise in HD rodent models. Coenzyme Q10 is thought to target mitochondrial dysfunction, which has been implicated as one of the pathologic mechanisms of mutant huntingtin. Minocycline, one of the tetracyclines, is known to have anti-apoptosis effects.^{[10, 11]}

Intravenous immunoglobulin and plasmapheresis may shorten the course of the illness and decrease symptom severity in patients with Sydenham chorea.

Chorea following cardiac transplantation has been reported to be responsive to steroid treatment.^[73]

Reports of drug treatment for hemiballism must take into account the high spontaneous remission rate for the disorder. Anecdotal reports must be viewed with caution, unless they can demonstrate that the response is due to the agent (by recurrence of the movements with drug withdrawal). The rarity of this disorder and the severity of its manifestations have precluded placebo-controlled drug trials. Pharmacologic treatment is the same as that prescribed for other choreatic disorders.^{[22, 24, 107, 8]}

Deep brain stimulation

Deep brain stimulation r(DBS) is an emerging technique that may benefit patients, at least in certain cases.

In 2000, Thompson et al reported a reduction in choreiform movements in 2 pediatric cases of chorea. One patient had cerebral palsy from birth secondary to brain hemorrhage. The other, an 11-year-old child, developed chorea subsequent to a thalamic hemorrhage 4 years before. Both children improved after the procedure.^[108]

Reported in 2003, Krauss et al tested globus pallidus stimulation on 2 patients with dystonia (one adult and one child) and 4 adult patients with essentially static (ie, nonchanging) chorea secondary to cerebral palsy. The dystonia patients markedly improved. Two of the 4 chorea patients showed no improvement, but 2 showed mild improvement.^[109]

In 2004, Moro et al reported on bilateral globus pallidus internus stimulation on a patient with Huntington disease (HD). Stimulation at 130 and 40 Hz improved the chorea, but the stimulation at 130 Hz worsened the bradykinesia. Stimulation of 40 Hz had little effect on the bradykinesia and appeared to increase blood flow (assessed by positron emission tomography scanning) in areas associated with executive functions and judgment.^[110]

Although DBS is not yet used routinely for chorea, as it is for PD, exciting progress has been made with this modality. Prior to DBS, surgeries like thalamotomy and pallidotomy were found to be effective in some cases.^{[111, 112]} However, due to the invasive nature of the surgeries and the complications due to lesional surgery, they are not used in routine clinical practice.

Cell transplantation

Cell transplantation is controversial and in early stages of research. It has shown variable results for HD patient participants.

In 2006, Bachoud-L é vi et al reported that fetal neural cell transplantation into host striatum resulted in stabilization or improvement in chorea, oculomotor dysfunction, gait, tapping, and cognition, but dystonia progressed at the same rate as nongrafted patients. However, these results persisted for up to 6 years only, and then patients' disease continued to progress at pretransplantation rates.^[113]

In 2008, Keene et al demonstrated on autopsy that fetal neural cell grafts in 2 patients had shown neuronal differentiation and survival, but they had poor integration with host striatum, likely explaining the lack of clinical improvement in these patients.^[114]

Medication

Barbeau A, Duvoisin RC, Gerstenbrand F, Lakke JP, Marsden CD, Stern G. Classification of extrapyramidal disorders. Proposal for an international classification and glossary of terms. J Neurol Sci. 1981 Aug. 51(2):311-27. [QxMD MEDLINE Link].
Berman SA. Chorea. Joseph AB, Young RR, eds. Movement Disorders in Neurology and Neuropsychiatry. 2nd ed. Malden, Mass: Blackwell Science; 1999. 481-94.
Dorland WA, ed. Dorlands Illustrated Medical Dictionary. 30th ed. Philadelphia, Pa: WB Saunders; 2003.
Dewey RB Jr, Jankovic J. Hemiballism-hemichorea. Clinical and pharmacologic findings in 21 patients. Arch Neurol. 1989 Aug. 46(8):862-7. [QxMD MEDLINE Link].
Fukui T, Hasegawa Y, Seriyama S, et al. Hemiballism-hemichorea induced by subcortical ischemia. Can J Neurol Sci. 1993 Nov. 20(4):324-8. [QxMD MEDLINE Link].
Glass JP, Jankovic J, Borit A. Hemiballism and metastatic brain tumor. Neurology. 1984 Feb. 34(2):204-7. [QxMD MEDLINE Link].
Sanchez-Ramos JR, Factor SA, Weiner WJ, Marquez J. Hemichorea-hemiballismus associated with acquired immune deficiency syndrome and cerebral toxoplasmosis. Mov Disord. 1989. 4(3):266-73. [QxMD MEDLINE Link].
Vidakovic A, Dragasevic N, Kostic VS. Hemiballism: report of 25 cases. J Neurol Neurosurg Psychiatry. 1994 Aug. 57(8):945-9. [QxMD MEDLINE Link].
Chen-Plotkin AS, Sadri-Vakili G, Yohrling GJ, Braveman MW, Benn CL, Glajch KE, et al. Decreased association of the transcription factor Sp1 with genes downregulated in Huntingtons disease. Neurobiol Dis. 2006 May. 22(2):233-41. [QxMD MEDLINE Link].
Smith KM, Matson S, Matson WR, Cormier K, Del Signore SJ, Hagerty SW. Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntingtons disease mice. Biochim Biophys Acta. 2006 Jun. 1762(6):616-26. [QxMD MEDLINE Link].
Stack EC, Smith KM, Ryu H, Cormier K, Chen M, Hagerty SW, et al. Combination therapy using minocycline and coenzyme Q10 in R6/2 transgenic Huntingtons disease mice. Biochim Biophys Acta. 2006 Mar. 1762(3):373-80. [QxMD MEDLINE Link].
Zuccato C, Belyaev N, Conforti P, Ooi L, Tartari M, Papadimou E, et al. Widespread disruption of repressor element-1 silencing transcription factor/neuron-restrictive silencer factor occupancy at its target genes in Huntingtons disease. J Neurosci. 2007 Jun 27. 27(26):6972-83. [QxMD MEDLINE Link].
Leavitt BR, Hayden MR. Is tetrabenazine safe and effective for suppressing chorea in Huntingtons disease?. Nat Clin Pract Neurol. 2006 Oct. 2(10):536-7. [QxMD MEDLINE Link].
Savani AA, Login IS. Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial. Neurology. 2007 Mar 6. 68(10):797; author reply 797. [QxMD MEDLINE Link].
Gomez-Anson B, Alegret M, Munoz E, Sainz A, Monte GC, Tolosa E. Decreased frontal choline and neuropsychological performance in preclinical Huntington disease. Neurology. 2007 Mar 20. 68(12):906-10. [QxMD MEDLINE Link].
Glass M, Dragunow M, Faull RL. The pattern of neurodegeneration in Huntingtons disease: a comparative study of cannabinoid, dopamine, adenosine and GABA(A) receptor alterations in the human basal ganglia in Huntingtons disease. Neuroscience. 2000. 97(3):505-19. [QxMD MEDLINE Link].
Saft C, Lauter T, Kraus PH, Przuntek H, Andrich JE. Dose-dependent improvement of myoclonic hyperkinesia due to Valproic acid in eight Huntingtons Disease patients: a case series. BMC Neurol. 2006 Feb 28. 6:11. [QxMD MEDLINE Link].
Curtis MA, Faull RL, Glass M. A novel population of progenitor cells expressing cannabinoid receptors in the subependymal layer of the adult normal and Huntingtons disease human brain. J Chem Neuroanat. 2006 Apr. 31(3):210-5. [QxMD MEDLINE Link].
de Lago E, Fernandez-Ruiz J, Ortega-Gutierrez S, Cabranes A, Pryce G, Baker D, et al. UCM707, an inhibitor of the anandamide uptake, behaves as a symptom control agent in models of Huntingtons disease and multiple sclerosis, but fails to delay/arrest the progression of different motor-related disorders. Eur Neuropsychopharmacol. 2006 Jan. 16(1):7-18. [QxMD MEDLINE Link].
Dubinsky RM, Greenberg M, Di Chiro G, et al. Hemiballismus: study of a case using positron emission tomography with 18fluoro-2-deoxyglucose. Mov Disord. 1989. 4(4):310-9. [QxMD MEDLINE Link].
Klawans HL. Chorea. Can J Neurol Sci. 1987 Aug. 14(3 Suppl):536-40. [QxMD MEDLINE Link].
Evidente VG, Gwinn-Hardy K, Caviness JN, Alder CH. Risperidone is effective in severe hemichorea/hemiballismus. Mov Disord. 1999 Mar. 14(2):377-9. [QxMD MEDLINE Link].
Inzelberg R, Korczyn AD. Persistent hemiballism in Parkinsons disease. J Neurol Neurosurg Psychiatry. 1994 Aug. 57(8):1013-4. [QxMD MEDLINE Link].
Johnson WG, Fahn S. Treatment of vascular hemiballism and hemichorea. Neurology. 1977 Jul. 27(7):634-6. [QxMD MEDLINE Link].
Martinez-Martin P. Hemichorea-hemiballism in AIDS. Mov Disord. 1990. 5(2):180. [QxMD MEDLINE Link].
Riley D, Lang AE. Hemiballism in multiple sclerosis. Mov Disord. 1988. 3(1):88-94. [QxMD MEDLINE Link].
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].
Jankovic J. Huntingtons disease, Wilsons disease, and neuroacanthocytosis. A Comprehensive Review of Movement Disorders for the Clinical Practitioner. 2nd Annual Course. New York, NY: Columbia University; 1992. 261-78.
Petrukhin K, Lutsenko S, Chernov I, et al. Characterization of the Wilson disease gene encoding a P-type copper transporting ATPase: genomic organization, alternative splicing, and structure/function predictions. Hum Mol Genet. 1994 Sep. 3(9):1647-56. [QxMD MEDLINE Link].
Bird TD, Carlson CB, Hall JG. Familial essential (benign) chorea. J Med Genet. 1976 Oct. 13(5):357-62. [QxMD MEDLINE Link].
Breedveld GJ, van Dongen JW, Danesino C, et al. Mutations in TITF-1 are associated with benign hereditary chorea. Hum Mol Genet. 2002 Apr 15. 11(8):971-9. [QxMD MEDLINE Link].
Burns J, Neuhauser G, Tomasi L. Benign hereditary non-progressive chorea of early onset. Clinical genetics of the syndrome and report of a new family. Neuropadiatrie. 1976 Nov. 7(4):431-8. [QxMD MEDLINE Link].
Chun RW, Daly RF, Mansheim BJ Jr, Wolcott GJ. Benign familial chorea with onset in childhood. JAMA. 1973 Sep 24. 225(13):1603-7. [QxMD MEDLINE Link].
Damasio H, Antunes L, Damasio AR. Familial nonprogressive involuntary movements of childhood. Ann Neurol. 1977 Jun. 1(6):602-3. [QxMD MEDLINE Link].
Haerer AF, Currier RD, Jackson JF. Hereditary nonprogressive chorea of early onset. N Engl J Med. 1967 Jun 1. 276(22):1220-4. [QxMD MEDLINE Link].
Kuwert T, Lange HW, Langen KJ, et al. Normal striatal glucose consumption in two patients with benign hereditary chorea as measured by positron emission tomography. J Neurol. 1990 Apr. 237(2):80-4. [QxMD MEDLINE Link].
MacMillan JC, Morrison PJ, Nevin NC, Shaw DJ, Harper PS, Quarrell OW, et al. Identification of an expanded CAG repeat in the Huntington's disease gene (IT15) in a family reported to have benign hereditary chorea. J Med Genet. 1993 Dec. 30(12):1012-3. [QxMD MEDLINE Link].
Rice E, Terrence C. Computerized tomography in hereditary nonprogressive chorea. Arch Neurol. 1979 Apr. 36(4):249-50. [QxMD MEDLINE Link].
Robinson RO, Thornett CE. Benign hereditary chorea--response to steroids. Dev Med Child Neurol. 1985 Dec. 27(6):814-6. [QxMD MEDLINE Link].
Suchowersky O, Hayden MR, Martin WR, et al. Cerebral metabolism of glucose in benign hereditary chorea. Mov Disord. 1986. 1(1):33-44. [QxMD MEDLINE Link].
Wheeler PG, Weaver DD, Dobyns WB. Benign hereditary chorea. Pediatr Neurol. 1993 Sep-Oct. 9(5):337-40. [QxMD MEDLINE Link].
Yapijakis C, Kapaki E, Zournas C, Rentzos M, Loukopoulos D, Papageorgiou C. Exclusion mapping of the benign hereditary chorea gene from the Huntingtons disease locus: report of a family. Clin Genet. 1995 Mar. 47(3):133-8. [QxMD MEDLINE Link].
Ross CA, Aylward EH, Wild EJ, Langbehn DR, Long JD, Warner JH, et al. Huntington disease: natural history, biomarkers and prospects for therapeutics. Nat Rev Neurol. 2014 Apr. 10 (4):204-16. [QxMD MEDLINE Link].
McKusick V. Huntington disease; HD. OMIM ID #143100. Online Mendelian Inheritance in Man. Available at http://www.ncbi.nlm.nih.gov/omim/143100. Accessed: March 17, 2009.
Langbehn DR, Hayden MR, Paulsen JS, and the PREDICT-HD Investigators of the Huntington Study Group. CAG-repeat length and the age of onset in Huntington disease (HD): a review and validation study of statistical approaches. Am J Med Genet B Neuropsychiatr Genet. 2010 Mar 5. 153B (2):397-408. [QxMD MEDLINE Link].
McKusick V. Choreoacanthocytosis; CHAC. OMIM ID #200150. Online Mendelian Inheritance in Man. Available at http://www.ncbi.nlm.nih.gov/omim/200150. Accessed: March 17, 2009.
Jung HH, Danek A, Walker RH. Neuroacanthocytosis syndromes. Orphanet J Rare Dis. 2011 Oct 25. 6:68. [QxMD MEDLINE Link].
Zomorrodi A, Wald ER. Sydenham's chorea in western Pennsylvania. Pediatrics. 2006 Apr. 117 (4):e675-9. [QxMD MEDLINE Link].
Haerer AF, Currier RD, Jackson JF. Hereditary nonprogressive chorea of early onset. N Engl J Med. 1967 Jun 1. 276 (22):1220-4. [QxMD MEDLINE Link].
McKusick V. Chorea, benign hereditary; BHC. OMIM ID #118700. Online Mendelian Inheritance in Man. Available at http://www.ncbi.nlm.nih.gov/omim/118700. Accessed: March 17, 2009.
Jones R, Stout JC, Labuschagne I, Say M, Justo D, Coleman A, et al. The potential of composite cognitive scores for tracking progression in Huntington's disease. J Huntingtons Dis. 2014. 3(2):197-207. [QxMD MEDLINE Link].
Langbehn DR, Brinkman RR, Falush D, Paulsen JS, Hayden MR, International Huntington's Disease Collaborative Group. A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length. Clin Genet. 2004 Apr. 65 (4):267-77. [QxMD MEDLINE Link].
Klein C. The Wilson films--Huntington's chorea. Mov Disord. 2011 Dec. 26(14):2464-6. [QxMD MEDLINE Link].
Bostantjopoulou S, Katsarou Z, Kazis A, Vadikolia C. Neuroacanthocytosis presenting as parkinsonism. Mov Disord. 2000 Nov. 15 (6):1271-3. [QxMD MEDLINE Link].
Kobal J, Dobson-Stone C, Danek A, Fidler V, Zvan B, Zaletel M. Chorea-acanthocytosis presenting as dystonia. Acta Clin Croat. 2014 Mar. 53(1):107-12. [QxMD MEDLINE Link].
Alcock, NS. A note of the pathology of senile chorea (non-hereditary). Brain. 1936. 59:376-87.
Friedman JH, Ambler M. A case of senile chorea. Mov Disord. 1990. 5(3):251-3. [QxMD MEDLINE Link].
Galvez-Jimenez N, Friedman J, Lang A. A consistent MRI pattern in three cases of senile chorea. Neurology. 1995. 45 (Supplement 4):A185.
Giedd JN, Rapoport JL, Kruesi MJ, Parker C, Schapiro MB, Allen AJ, et al. Sydenhams chorea: magnetic resonance imaging of the basal ganglia. Neurology. 1995 Dec. 45(12):2199-202. [QxMD MEDLINE Link].
Swedo SE. Sydenhams chorea. A model for childhood autoimmune neuropsychiatric disorders. JAMA. 1994 Dec 14. 272(22):1788-91. [QxMD MEDLINE Link].
Beato R, Maia DP, Teixeira AL Jr, Cardoso F. Executive functioning in adult patients with Sydenham's chorea. Mov Disord. 2010 May 15. 25(7):853-7. [QxMD MEDLINE Link].
Gusella JF, MacDonald ME. Huntingtons disease: seeing the pathogenic process through a genetic lens. Trends Biochem Sci. July 2006. 31(pt 9):533-40.
Blekher T, Johnson SA, Marshall J, White K, Hui S, Weaver M, et al. Saccades in presymptomatic and early stages of Huntington disease. Neurology. 2006 Aug 8. 67 (3):394-9. [QxMD MEDLINE Link].
Nutting PA, Cole BR, Schimke RN. Benign, recessively inherited choreo-athetosis of early onset. J Med Genet. 1969 Dec. 6(4):408-10. [QxMD MEDLINE Link].
Fisher M, Sargent J, Drachman D. Familial inverted choreoathetosis. Neurology. 1979 Dec. 29(12):1627-31. [QxMD MEDLINE Link].
Wheeler PG, Dobyns WB, Plager DA, Ellis FD. Familial remitting chorea, nystagmus, and cataracts. Am J Med Genet. 1993 Dec 1. 47(8):1215-7. [QxMD MEDLINE Link].
Evans BK, Jankovic J. Tuberous sclerosis and chorea. Ann Neurol. 1983 Jan. 13(1):106-7. [QxMD MEDLINE Link].
Ross CA, Margolis RL, Rosenblatt A, et al. Huntington disease and the related disorder, dentatorubral-pallidoluysian atrophy (DRPLA). Medicine (Baltimore). 1997 Sep. 76(5):305-38. [QxMD MEDLINE Link].
Sethi KD, Ray R, Roesel RA, et al. Adult-onset chorea and dementia with propionic acidemia. Neurology. 1989 Oct. 39(10):1343-5. [QxMD MEDLINE Link].
Hefter H, Mayer P, Benecke R. Persistent chorea after recurrent hypoglycemia. A case report. Eur Neurol. 1993. 33(3):244-7. [QxMD MEDLINE Link].
Linazasoro G, Urtasun M, Poza JJ, et al. Generalized chorea induced by nonketotic hyperglycemia. Mov Disord. 1993. 8(1):119-20. [QxMD MEDLINE Link].
Toghill PJ, Johnston AW, Smith JF. Choreoathetosis in porto-systemic encephalopathy. J Neurol Neurosurg Psychiatry. 1967 Aug. 30(4):358-63. [QxMD MEDLINE Link].
Blunt SB, Brooks DJ, Kennard C. Steroid-responsive chorea in childhood following cardiac transplantation. Mov Disord. 1994 Jan. 9(1):112-4. [QxMD MEDLINE Link].
Curless RG, Katz DA, Perryman RA, et al. Choreoathetosis after surgery for congenital heart disease. J Pediatr. 1994 May. 124(5 Pt 1):737-9. [QxMD MEDLINE Link].
Peters AC, Vielvoye GJ, Versteeg J, et al. ECHO 25 focal encephalitis and subacute hemichorea. Neurology. 1979 May. 29(5):676-81. [QxMD MEDLINE Link].
Sweeney BJ, Edgecombe J, Churchill DR, et al. Choreoathetosis/ballismus associated with pentamidine-induced hypoglycemia in a patient with the acquired immunodeficiency syndrome. Arch Neurol. 1994 Jul. 51(7):723-5. [QxMD MEDLINE Link].
Davous P, Rondot P, Marion MH, Gueguen B. Severe chorea after acute carbon monoxide poisoning. J Neurol Neurosurg Psychiatry. 1986 Feb. 49(2):206-8. [QxMD MEDLINE Link].
Schwartz A, Hennerici M, Wegener OH. Delayed choreoathetosis following acute carbon monoxide poisoning. Neurology. 1985 Jan. 35(1):98-9. [QxMD MEDLINE Link].
Abbruzzese G, Brusa G, DallAgata D, Morena M, Spadavecchia L, Favale E. Electrophysiological analysis of motor control in patients with vascular hemichorea. Ital J Neurol Sci. 1987 Aug. 8(4):357-62. [QxMD MEDLINE Link].
Jones HR Jr, Baker RA, Kott HS. Hypertensive putaminal hemorrhage presenting with hemichorea. Stroke. 1985 Jan-Feb. 16(1):130-1. [QxMD MEDLINE Link].
Margolin DI, Marsden CD. Episodic dyskinesias and transient cerebral ischemia. Neurology. 1982 Dec. 32(12):1379-80. [QxMD MEDLINE Link].
Tabaton M, Mancardi G, Loeb C. Generalized chorea due to bilateral small, deep cerebral infarcts. Neurology. 1985 Apr. 35(4):588-9. [QxMD MEDLINE Link].
Bae SH, Vates TS Jr, Kenton EJ 3d. Generalized chorea associated with chronic subdural hematomas. Ann Neurol. 1980 Oct. 8(4):449-50. [QxMD MEDLINE Link].
Pavlakis SG, Schneider S, Black K, Gould RJ. Steroid-responsive chorea in moyamoya disease. Mov Disord. 1991. 6(4):347-9. [QxMD MEDLINE Link].
Bruyn GW, Ferrari MD. Chorea and migraine: Hemicrania choreatica?. Cephalalgia. 1984 Jun. 4(2):119-24. [QxMD MEDLINE Link].
Kok J, Bosseray A, Brion JP, et al. Chorea in a child with Churg-Strauss syndrome. Stroke. 1993 Aug. 24(8):1263-4. [QxMD MEDLINE Link].
Kimura N, Sugihara R, Kimura A, Kumamoto T, Tsuda T. [A case of neuro-Behcets disease presenting with chorea]. Rinsho Shinkeigaku. 2001 Jan. 41(1):45-9. [QxMD MEDLINE Link].
Caviness VS Jr. Huntingtons disease. Dev Med Child Neurol. 1985 Dec. 27(6):826-9. [QxMD MEDLINE Link].
Cervera R, Asherson RA, Font J, et al. Chorea in the antiphospholipid syndrome. Clinical, radiologic, and immunologic characteristics of 50 patients from our clinics and the recent literature. Medicine (Baltimore). 1997 May. 76(3):203-12. [QxMD MEDLINE Link].
O'Toole O, Lennon VA, Ahlskog JE, Matsumoto JY, Pittock SJ, Bower J, et al. Autoimmune chorea in adults. Neurology. 2013 Mar 19. 80 (12):1133-44. [QxMD MEDLINE Link].
Vigliani MC, Honnorat J, Antoine JC, Vitaliani R, Giometto B, Psimaras D, et al. Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience. J Neurol. 2011 Nov. 258 (11):2058-68. [QxMD MEDLINE Link].
Kellinghaus C, Kraus J, Blaes F, Nabavi DG, Schäbitz WR. CRMP-5-autoantibodies in testicular cancer associated with limbic encephalitis and choreiform dyskinesias. Eur Neurol. 2007. 57 (4):241-3. [QxMD MEDLINE Link].
Lee EK, Maselli RA, Ellis WG, Agius MA. Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma. J Neurol Neurosurg Psychiatry. 1998 Dec. 65 (6):857-62. [QxMD MEDLINE Link].
Walker FO, Hunt VP. Ballism: an association with ventriculoperitoneal shunting. Neurology. 1990 Jun. 40(6):1004. [QxMD MEDLINE Link].
Rosenblatt A, Liang KY, Zhou H, Abbott MH, Gourley LM, Margolis RL. The association of CAG repeat length with clinical progression in Huntington disease. Neurology. 2006 Apr 11. 66(7):1016-20. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Burton PD. Magnetic resonance imaging and brain iron: implications in the diagnosis and pathochemistry of movement disorders and dementia. Barrow Neurological Institute Quarterly. 1987. 3, No. 4:15-29.
Rutledge JN, Hilal SK, Silver AJ, et al. Study of movement disorders and brain iron by MR. Am J Neuroradiol. 1987. 8:397-411.
Montoya A, Price BH, Menear M, Lepage M. Brain imaging and cognitive dysfunctions in Huntingtons disease. J Psychiatry Neurosci. 2006 Jan. 31(1):21-9. [QxMD MEDLINE Link].
Rosas HD, Salat DH, Lee SY, Zaleta AK, Pappu V, Fischl B, et al. Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity. Brain. 2008 Apr. 131 (Pt 4):1057-68. [QxMD MEDLINE Link].
Hosokawa S, Ichiya Y, Kuwabara Y, et al. Positron emission tomography in cases of chorea with different underlying diseases. J Neurol Neurosurg Psychiatry. 1987 Oct. 50(10):1284-7. [QxMD MEDLINE Link].
Otsuka M, Ichiya Y, Kuwabara Y, et al. Cerebral glucose metabolism and striatal 18F-dopa uptake by PET in cases of chorea with or without dementia. J Neurol Sci. 1993 Apr. 115(2):153-7. [QxMD MEDLINE Link].
Tanaka M, Hirai S, Kondo S, et al. Cerebral hypoperfusion and hypometabolism with altered striatal signal intensity in chorea-acanthocytosis: a combined PET and MRI study. Mov Disord. 1998 Jan. 13(1):100-7. [QxMD MEDLINE Link].
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].
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].
Grove VE Jr, Quintanilla J, DeVaney GT. Improvement of Huntingtons disease with olanzapine and valproate. N Engl J Med. 2000 Sep 28. 343(13):973-4. [QxMD MEDLINE Link].
Shannon KM. Hemiballismus. Clin Neuropharmacol. 1990 Oct. 13(5):413-25. [QxMD MEDLINE Link].
Thompson TP, Kondziolka D, Albright AL. Thalamic stimulation for choreiform movement disorders in children. Report of two cases. J Neurosurg. 2000 Apr. 92(4):718-21. [QxMD MEDLINE Link].
Krauss JK, Loher TJ, Weigel R, et al. Chronic stimulation of the globus pallidus internus for treatment of non-dYT1 generalized dystonia and choreoathetosis: 2-year follow up. J Neurosurg. 2003 Apr. 98(4):785-92. [QxMD MEDLINE Link].
Moro E, Lang AE, Strafella AP, et al. Bilateral globus pallidus stimulation for Huntingtons disease. Ann Neurol. 2004 Aug. 56(2):290-4. [QxMD MEDLINE Link].
Goto S, Kunitoku N, Hamasaki T, Nishikawa S, Ushio Y. Abolition of postapoplectic hemichorea by Vo-complex thalamotomy: long-term follow-up study. Mov Disord. 2001 Jul. 16 (4):771-4. [QxMD MEDLINE Link].
Choi SJ, Lee SW, Kim MC, Kwon JY, Park CK, Sung JH, et al. Posteroventral pallidotomy in medically intractable postapoplectic monochorea: case report. Surg Neurol. 2003 Jun. 59 (6):486-90; discussion 490. [QxMD MEDLINE Link].
Bachoud-Levi AC, Gaura V, Brugieres P, Lefaucheur JP, Boisse MF, Maison P, et al. Effect of fetal neural transplants in patients with Huntingtons disease 6 years after surgery: a long-term follow-up study. Lancet Neurol. 2006 Apr. 5(4):303-9. [QxMD MEDLINE Link].
Keene CD, Sonnen JA, Swanson PD, Kopyov O, Leverenz JB, Bird TD, et al. Neural transplantation in Huntington disease: long-term grafts in two patients. Neurology. 2007 Jun 12. 68(24):2093-8. [QxMD MEDLINE Link].
Souza Ad, Moloi MW. Involuntary movements due to vitamin B12 deficiency. Neurol Res. 2014 Dec. 36(12):1121-8. [QxMD MEDLINE Link].

Media Gallery

of 0

Author

Pradeep C Bollu, MD Associate Professor of Neurology, Vice Chair of Quality, Department of Neurology, Prisma Health, University of South Carolina School of Medicine, Columbia

Pradeep C Bollu, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, Boone County Medical Society, Indian Medical Association, Indian Red Cross Society, International Parkinson and Movement Disorder Society, Missouri State Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Tejas R Mehta, MBBS Resident Physician, Department of Neurology, University of Missouri-Columbia School of Medicine

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

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, Catalyst, Ceribell, Eisai, Jazz, LivaNova, Neurelis, Neuropace, SK Life Science Science, Sunovion, Takeda, UCB<br/>Serve(d) as a speaker or a member of a speakers bureau for: Catalyst, Jazz, LivaNova, Neurelis, SK Life Science, Stratus, UCB<br/>Received research grant from: Cerevel Therapeutics; Ovid Therapeutics; Neuropace; Jazz; SK Life Science, Xenon Pharmaceuticals, UCB, Marinus, Longboard.

Additional Contributors

Stephen A Berman, MD, PhD, MBA Professor of Neurology, University of Central Florida College of Medicine

Stephen A Berman, MD, PhD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, Phi Beta Kappa

Disclosure: Nothing to disclose.

Stephen T Gancher, MD Adjunct Associate Professor, Department of Neurology, Oregon Health Sciences University

Stephen T Gancher, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, International Parkinson and Movement Disorder Society

Disclosure: Nothing to disclose.

Stephanie M Vertrees, MD Fellow in Public Health, Weill Cornell Medical College; Fellow in Medical Ethics, Fellow in Neuromuscular Medicine, Hospital for Special Surgery

Stephanie M Vertrees, MD is a member of the following medical societies: American Academy of Neurology, American Medical Women's Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Eric Dinnerstein, MD, Maria Alejandra Herrera, MD, and Nestor Galvez-Jimenez, MD, MSc, MHA, to the development and writing of this article.