Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Chorea in Adults Clinical Presentation

  • Author: Stephanie M Vertrees, MD; Chief Editor: Selim R Benbadis, MD  more...
 
Updated: Oct 24, 2014
 

History

Patients with chorea may not initially be aware of the abnormal movements because they may be subtle. Patients can suppress the chorea temporarily and frequently camouflage some of the movements by incorporating them into semipurposeful activities (ie, parakinesia). The inability to maintain voluntary contraction (ie, motor impersistence), as is seen during manual grip (milkmaid grip) tests or tongue protrusion, is a characteristic feature of chorea and results in the dropping of objects and clumsiness. Muscle stretch reflexes are often hung-up and pendular. In severely affected patients, a peculiar dancelike gait may be noted. Depending on the underlying cause of the chorea, other motor symptoms include dysarthria, dysphagia, postural instability, ataxia, dystonia, and myoclonus. A brief discussion of the clinical manifestations of the most common choreatic diseases is presented.

  • Huntington disease[1, 42]
    • Penetrance of HD is 100%. Expression is highly variable, both with respect to clinical manifestations and age of onset. When the disorder emerges early, particularly in patients younger than 20 years, it is most likely to run a rapid course with grave disability due to cognitive decline.[45]
    • The Westphal variant, a rigid dystonic disorder, may be accompanied by seizures and even myoclonus. It is encountered principally among those with childhood onset. In contrast, when the disorder appears late in life, the cardinal manifestation is chorea.
    • The insidious onset of clumsiness and adventitious movements may be wrongly attributed to simple nervousness. Although chorea and other motor disabilities are the most readily recognized manifestations of HD, they may be neither the earliest to appear nor the most disabling manifestations of the disease.
    • Psychological disturbances and personality change are the initial manifestations in greater than 50% of affected persons. Symptoms consistent with a depressive state are the most frequent psychological disturbances.
    • The duration of illness from onset to death is approximately 15 years in the case of adult HD and 8-10 years for the juvenile variant.
  • Wilson disease[27, 28, 46]
    • The clinical features are age dependent. In children, the disease is manifested initially by progressive dystonia, rigidity and dysarthria, and hepatic dysfunction, whereas in adults, psychiatric symptoms, tremor, and dysarthria usually predominate.
    • Because Kayser-Fleischer rings are almost always present when neurological symptoms are present, slit-lamp examination of the cornea must be performed to be certain that Wilson disease is excluded in a patient with chorea beginning in childhood or young adulthood. In patients with chorea and negative findings from a slit-lamp examination, serum copper and ceruloplasmin analysis along with a 24-hour copper urine excretion test need to be performed.
  • Neuroacanthocytosis[1, 43, 47]
    • Symptoms usually begin with lip and tongue biting (often causing self-injury), orolingual dystonia, motor and phonic tics, generalized chorea, parkinsonism, and seizures. Patients with neuroacanthocytosis may report an inability to feed themselves because of dystonic tongue protrusion every time they try to eat.
    • Other features include cognitive and personality changes, dysphagia, dysarthria, amyotrophy, areflexia, evidence of axonal neuropathy with absent deep ankle tendon stretch reflexes, and elevated serum creatine kinase levels without evidence of myopathy.
  • Senile chorea[48, 49, 50]
    • This clinical entity is characterized by a gradual onset of generalized and symmetric chorea with slow progression and specifically excluding mental deterioration, emotional disturbances, or family history.
    • To rule out the possibility of HD, genetic testing is recommended because family history can be inaccurate and distinguishing age-related mental changes from early features of HD in an elderly person may be difficult.
  • Sydenham chorea[51, 52, 53]
    • Sydenham chorea is a major manifestation of acute rheumatic fever. With the 1992 modifications of the Jones criteria (see the Jones Criteria for Diagnosis of Rheumatic Fever calculator), it alone is sufficient to enable the physician to make the diagnosis of the first attack of acute rheumatic fever. Sydenham chorea is considered a disease of childhood; however, it also may be seen in adults. Rheumatic chorea is characterized by muscle weakness and the presence of chorea. The patients have the milkmaid grip sign, clumsy gait, and explosive bursts of dysarthric speech. Often, harlequin tongue, which pops in and out when the patient tries to hold it out, can be prominently demonstrated.
    • Psychological symptoms are equally prominent and typically precede the appearance of even the most subtle choreiform movements. Emotional lability is the most common symptom; decreased attention span, obsessive-compulsive symptoms, and separation anxiety disorder also are seen. Symptoms can lag behind the etiologic streptococcal infection by 1-6 months. In adults, generalized poststreptococcal chorea may complicate birth control or pregnancy (chorea gravidarum).
  • Benign hereditary chorea[1, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41]
    • This is a rare autosomal dominant genetic disorder characterized by nonprogressive choreiform movements that appear in childhood, without intellectual impairment. It is further distinguished clinically from juvenile HD by the absence of seizures, rigidity, or cerebellar features.
    • Benign hereditary chorea is caused by a mutation in the TITF1 gene. Interestingly, this gene contains the code for a transcription factor essential for the organogenesis of the basal ganglia, the lungs, and the thyroid.
    • It does not shorten the life span of affected patients, but severely affected patients can be markedly disabled by the chorea.
Next

Physical

Because Huntington disease (HD) is the most clearly defined choreatic disease, its physical findings are described here.

  • Huntington disease[1, 54, 42]
    • HD is caused by an expansion repeat (CAG) mutation in the IT15 gene (which codes for the protein called huntingtin) on chromosome 4. Initial signs of chorea generally are flickers in the fingers and ticlike grimaces of the face. Over time, higher-amplitude dancelike movements disrupt voluntary actions of the extremities and interfere with gait. Speech becomes dysrhythmic.
    • Characteristically, the patient is hypotonic, although reflexes may be augmented and clonus may be noted.
    • Voluntary gaze is disturbed early. In particular, saccades may be irregular or of prolonged latency and may require an initial blink for their initiation.
    • Loss of optokinetic nystagmus is common after a decade of progressive disease.
    • Cognitive changes are manifested early as loss of recent memory and impaired judgment. Apraxia is also present. Ultimately, the patient becomes severely demented.
    • Neurobehavioral changes typically consist of personality changes, apathy, social withdrawal, agitation, impulsiveness, depression, mania, paranoia, delusions, hostility, hallucinations, or psychosis.
    • The Westphal variant is dominated by rigidity, bradykinesia, and dystonic postures. Generalized seizures and myoclonus may be seen. Ataxia and dementia are also present.
Previous
Next

Causes

See the list below:

Previous
 
 
Contributor Information and Disclosures
Author

Stephanie M Vertrees, MD Fellow in Public Health, Weill Cornell Medical College-Hospital for Special Surgery Fellowship 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 Womens Association

Disclosure: Nothing to disclose.

Coauthor(s)

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.

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 College of Medicine

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

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cyberonics; Eisai; Lundbeck; Sunovion; UCB; Upsher-Smith<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cyberonics; Eisai; Glaxo Smith Kline; Lundbeck; Sunovion; UCB<br/>Received research grant from: Cyberonics; Lundbeck; Sepracor; Sunovion; UCB; Upsher-Smith.

Additional Contributors

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.

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.

References
  1. Berman SA. Chorea. Joseph AB, Young RR, eds. Movement Disorders in Neurology and Neuropsychiatry. 2nd ed. Malden, Mass: Blackwell Science; 1999. 481-94.

  2. Dorland WA, ed. Dorlands Illustrated Medical Dictionary. 30th ed. Philadelphia, Pa: WB Saunders; 2003.

  3. 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. [Medline].

  4. Dewey RB Jr, Jankovic J. Hemiballism-hemichorea. Clinical and pharmacologic findings in 21 patients. Arch Neurol. 1989 Aug. 46(8):862-7. [Medline].

  5. Fukui T, Hasegawa Y, Seriyama S, et al. Hemiballism-hemichorea induced by subcortical ischemia. Can J Neurol Sci. 1993 Nov. 20(4):324-8. [Medline].

  6. Glass JP, Jankovic J, Borit A. Hemiballism and metastatic brain tumor. Neurology. 1984 Feb. 34(2):204-7. [Medline].

  7. 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. [Medline].

  8. Vidakovic A, Dragasevic N, Kostic VS. Hemiballism: report of 25 cases. J Neurol Neurosurg Psychiatry. 1994 Aug. 57(8):945-9. [Medline].

  9. 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. [Medline].

  10. 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. [Medline].

  11. 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. [Medline].

  12. 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. [Medline].

  13. 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. [Medline].

  14. 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. [Medline].

  15. 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. [Medline].

  16. 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. [Medline].

  17. 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. [Medline].

  18. 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. [Medline].

  19. 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. [Medline].

  20. 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. [Medline].

  21. Klawans HL. Chorea. Can J Neurol Sci. 1987 Aug. 14(3 Suppl):536-40. [Medline].

  22. Evidente VG, Gwinn-Hardy K, Caviness JN, Alder CH. Risperidone is effective in severe hemichorea/hemiballismus. Mov Disord. 1999 Mar. 14(2):377-9. [Medline].

  23. Inzelberg R, Korczyn AD. Persistent hemiballism in Parkinsons disease. J Neurol Neurosurg Psychiatry. 1994 Aug. 57(8):1013-4. [Medline].

  24. Johnson WG, Fahn S. Treatment of vascular hemiballism and hemichorea. Neurology. 1977 Jul. 27(7):634-6. [Medline].

  25. Martinez-Martin P. Hemichorea-hemiballism in AIDS. Mov Disord. 1990. 5(2):180. [Medline].

  26. Riley D, Lang AE. Hemiballism in multiple sclerosis. Mov Disord. 1988. 3(1):88-94. [Medline].

  27. 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.

  28. 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. [Medline].

  29. Bird TD, Carlson CB, Hall JG. Familial essential (benign) chorea. J Med Genet. 1976 Oct. 13(5):357-62. [Medline].

  30. 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. [Medline].

  31. 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. [Medline].

  32. Chun RW, Daly RF, Mansheim BJ Jr, Wolcott GJ. Benign familial chorea with onset in childhood. JAMA. 1973 Sep 24. 225(13):1603-7. [Medline].

  33. Damasio H, Antunes L, Damasio AR. Familial nonprogressive involuntary movements of childhood. Ann Neurol. 1977 Jun. 1(6):602-3. [Medline].

  34. Haerer AF, Currier RD, Jackson JF. Hereditary nonprogressive chorea of early onset. N Engl J Med. 1967 Jun 1. 276(22):1220-4. [Medline].

  35. 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. [Medline].

  36. 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. [Medline].

  37. Rice E, Terrence C. Computerized tomography in hereditary nonprogressive chorea. Arch Neurol. 1979 Apr. 36(4):249-50. [Medline].

  38. Robinson RO, Thornett CE. Benign hereditary chorea--response to steroids. Dev Med Child Neurol. 1985 Dec. 27(6):814-6. [Medline].

  39. Suchowersky O, Hayden MR, Martin WR, et al. Cerebral metabolism of glucose in benign hereditary chorea. Mov Disord. 1986. 1(1):33-44. [Medline].

  40. Wheeler PG, Weaver DD, Dobyns WB. Benign hereditary chorea. Pediatr Neurol. 1993 Sep-Oct. 9(5):337-40. [Medline].

  41. 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. [Medline].

  42. 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.

  43. 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.

  44. 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.

  45. 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. [Medline].

  46. Klein C. The Wilson films--Huntington's chorea. Mov Disord. 2011 Dec. 26(14):2464-6. [Medline].

  47. 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. [Medline].

  48. Alcock, NS. A note of the pathology of senile chorea (non-hereditary). Brain. 1936. 59:376-87.

  49. Friedman JH, Ambler M. A case of senile chorea. Mov Disord. 1990. 5(3):251-3. [Medline].

  50. Galvez-Jimenez N, Friedman J, Lang A. A consistent MRI pattern in three cases of senile chorea. Neurology. 1995. 45 (Supplement 4):A185.

  51. 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. [Medline].

  52. Swedo SE. Sydenhams chorea. A model for childhood autoimmune neuropsychiatric disorders. JAMA. 1994 Dec 14. 272(22):1788-91. [Medline].

  53. 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. [Medline].

  54. Gusella JF, MacDonald ME. Huntingtons disease: seeing the pathogenic process through a genetic lens. Trends Biochem Sci. July 2006. 31(pt 9):533-40.

  55. Nutting PA, Cole BR, Schimke RN. Benign, recessively inherited choreo-athetosis of early onset. J Med Genet. 1969 Dec. 6(4):408-10. [Medline].

  56. Fisher M, Sargent J, Drachman D. Familial inverted choreoathetosis. Neurology. 1979 Dec. 29(12):1627-31. [Medline].

  57. 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. [Medline].

  58. Evans BK, Jankovic J. Tuberous sclerosis and chorea. Ann Neurol. 1983 Jan. 13(1):106-7. [Medline].

  59. 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. [Medline].

  60. Sethi KD, Ray R, Roesel RA, et al. Adult-onset chorea and dementia with propionic acidemia. Neurology. 1989 Oct. 39(10):1343-5. [Medline].

  61. Hefter H, Mayer P, Benecke R. Persistent chorea after recurrent hypoglycemia. A case report. Eur Neurol. 1993. 33(3):244-7. [Medline].

  62. Linazasoro G, Urtasun M, Poza JJ, et al. Generalized chorea induced by nonketotic hyperglycemia. Mov Disord. 1993. 8(1):119-20. [Medline].

  63. Toghill PJ, Johnston AW, Smith JF. Choreoathetosis in porto-systemic encephalopathy. J Neurol Neurosurg Psychiatry. 1967 Aug. 30(4):358-63. [Medline].

  64. Blunt SB, Brooks DJ, Kennard C. Steroid-responsive chorea in childhood following cardiac transplantation. Mov Disord. 1994 Jan. 9(1):112-4. [Medline].

  65. 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. [Medline].

  66. Peters AC, Vielvoye GJ, Versteeg J, et al. ECHO 25 focal encephalitis and subacute hemichorea. Neurology. 1979 May. 29(5):676-81. [Medline].

  67. 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. [Medline].

  68. 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. [Medline].

  69. Schwartz A, Hennerici M, Wegener OH. Delayed choreoathetosis following acute carbon monoxide poisoning. Neurology. 1985 Jan. 35(1):98-9. [Medline].

  70. 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. [Medline].

  71. Jones HR Jr, Baker RA, Kott HS. Hypertensive putaminal hemorrhage presenting with hemichorea. Stroke. 1985 Jan-Feb. 16(1):130-1. [Medline].

  72. Margolin DI, Marsden CD. Episodic dyskinesias and transient cerebral ischemia. Neurology. 1982 Dec. 32(12):1379-80. [Medline].

  73. Tabaton M, Mancardi G, Loeb C. Generalized chorea due to bilateral small, deep cerebral infarcts. Neurology. 1985 Apr. 35(4):588-9. [Medline].

  74. Bae SH, Vates TS Jr, Kenton EJ 3d. Generalized chorea associated with chronic subdural hematomas. Ann Neurol. 1980 Oct. 8(4):449-50. [Medline].

  75. Pavlakis SG, Schneider S, Black K, Gould RJ. Steroid-responsive chorea in moyamoya disease. Mov Disord. 1991. 6(4):347-9. [Medline].

  76. Bruyn GW, Ferrari MD. Chorea and migraine: Hemicrania choreatica?. Cephalalgia. 1984 Jun. 4(2):119-24. [Medline].

  77. Kok J, Bosseray A, Brion JP, et al. Chorea in a child with Churg-Strauss syndrome. Stroke. 1993 Aug. 24(8):1263-4. [Medline].

  78. 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. [Medline].

  79. Caviness VS Jr. Huntingtons disease. Dev Med Child Neurol. 1985 Dec. 27(6):826-9. [Medline].

  80. 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. [Medline].

  81. Walker FO, Hunt VP. Ballism: an association with ventriculoperitoneal shunting. Neurology. 1990 Jun. 40(6):1004. [Medline].

  82. 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. [Medline].

  83. 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.

  84. Rutledge JN, Hilal SK, Silver AJ, et al. Study of movement disorders and brain iron by MR. Am J Neuroradiol. 1987. 8:397-411.

  85. 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. [Medline].

  86. 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. [Medline].

  87. 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. [Medline].

  88. 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. [Medline].

  89. 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. [Medline].

  90. Shannon KM. Hemiballismus. Clin Neuropharmacol. 1990 Oct. 13(5):413-25. [Medline].

  91. 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. [Medline].

  92. 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. [Medline].

  93. Moro E, Lang AE, Strafella AP, et al. Bilateral globus pallidus stimulation for Huntingtons disease. Ann Neurol. 2004 Aug. 56(2):290-4. [Medline].

  94. 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. [Medline].

  95. 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. [Medline].

  96. Souza Ad, Moloi MW. Involuntary movements due to vitamin B12 deficiency. Neurol Res. 2014 Dec. 36(12):1121-8. [Medline].

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