Semin Neurol 2023; 43(01): 065-080
DOI: 10.1055/s-0043-1763506
Review Article

Diagnostic Uncertainties: Chorea

Molly C. Cincotta
1   Department of Neurology, Temple University, Philadelphia, Pennsylvania
,
Ruth H. Walker
2   Department of Neurology, James J. Peters Veterans Affairs Medical Center and Mount Sinai School of Medicine, Bronx, New York
› Author Affiliations

Abstract

Chorea is a hyperkinetic movement disorder with a multitude of potential etiologies, both acquired and inherited. Although the differential diagnosis for new-onset chorea is extensive, there are often clues in the history, exam, and basic testing that can help to narrow the options. Evaluation for treatable or reversible causes should take priority, as rapid diagnosis can lead to more favorable outcomes. While Huntington's disease is most common genetic cause of chorea, multiple phenocopies also exist and should be considered if Huntington gene testing is negative. The decision of what additional genetic testing to pursue should be based on both clinical and epidemiological factors. The following review provides an overview of the many possible etiologies as well as a practical approach for a patient presenting with new-onset chorea.



Publication History

Article published online:
07 March 2023

© 2023. Thieme. All rights reserved.

Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA

 
  • References

  • 1 Termsarasab P. Chorea. Continuum (Minneap Minn) 2019; 25 (04) 1001-1035
  • 2 Bhidayasiri R, Truong DD. Chorea and related disorders. Postgrad Med J 2004; 80 (947) 527-534
  • 3 Walker RH. Differential diagnosis of chorea. Curr Neurol Neurosci Rep 2011; 11 (04) 385-395
  • 4 Piccolo I, Defanti CA, Soliveri P, Volontè MA, Cislaghi G, Girotti F. Cause and course in a series of patients with sporadic chorea. J Neurol 2003; 250 (04) 429-435
  • 5 Bovenzi R, Conti M, Cerroni R. et al. Adult-onset sporadic chorea: real-world data from a single-centre retrospective study. Neurol Sci 2022; 43 (01) 387-392
  • 6 Ghika-Schmid F, Ghika J, Regli F, Bogousslavsky J. Hyperkinetic movement disorders during and after acute stroke: the Lausanne Stroke Registry. J Neurol Sci 1997; 146 (02) 109-116
  • 7 Alarcón F, Zijlmans JCM, Dueñas G, Cevallos N. Post-stroke movement disorders: report of 56 patients. J Neurol Neurosurg Psychiatry 2004; 75 (11) 1568-1574
  • 8 Zijlmans JCM. Vascular Chorea in Adults and Children. Vol 100. 1st ed.. Amsterdan: Elsevier B.V.; 2011
  • 9 Chung SJ, Im JH, Lee MC, Kim JS. Hemichorea after stroke: clinical-radiological correlation. J Neurol 2004; 251 (06) 725-729
  • 10 Strauss S, Rafie D, Nimma A, Romero R, Hanna PA. Pure cortical stroke causing hemichorea-hemiballismus. J Stroke Cerebrovasc Dis 2019; 28 (10) 104287
  • 11 Laganiere S, Boes AD, Fox MD. Network localization of hemichorea-hemiballismus. Neurology 2016; 86 (23) 2187-2195
  • 12 Demartini Jr Z, Teixeira BCA, Cardoso-Demartini AA. Choreoathetosis in moyamoya disease. World Neurosurg 2021; 156: 103-104
  • 13 Cincotta M, Walker RH. One side of the story; clues to etiology in patients with asymmetric chorea. Tremor Other Hyperkinet Mov (N Y) 2022; 12 (01) 3
  • 14 Medlock MD, Cruse RS, Winek SJ. et al. A 10-year experience with postpump chorea. Ann Neurol 1993; 34 (06) 820-826
  • 15 Thobois S, Bozio A, Ninet J, Akhavi A, Broussolle E. Chorea after cardiopulmonary bypass. Eur Neurol 2004; 51 (01) 46-47
  • 16 Ahn JH, Song J, Choi I, Youn J, Cho JW. Risk factors and prognosis of adult-onset post-pump chorea. J Neurol Sci 2021; 422 (January): 117328
  • 17 Cardoso F. Autoimmune choreas. J Neurol Neurosurg Psychiatry 2017; 88 (05) 412-417
  • 18 Cardoso F, Eduardo C, Silva AP, Mota CCC. Chorea in fifty consecutive patients with rheumatic fever. Mov Disord 1997; 12 (05) 701-703
  • 19 Maia DP, Teixeira Jr AL, Quintão Cunningham MC, Cardoso F. Obsessive compulsive behavior, hyperactivity, and attention deficit disorder in Sydenham chorea. Neurology 2005; 64 (10) 1799-1801
  • 20 Cardoso F. Sydenham's chorea. Handb Clin Neurol 2011; 100: 221-229
  • 21 Attoni T, Beato R, Pinto S, Cardoso F. Abnormal eye movements in three types of chorea. Arq Neuropsiquiatr 2016; 74 (09) 761-766
  • 22 Teixeira Jr AL, Maia DP, Cardoso F. Treatment of acute Sydenham's chorea with methyl-prednisolone pulse-therapy. Parkinsonism Relat Disord 2005; 11 (05) 327-330
  • 23 Feinstein E, Walker R. Treatment of secondary chorea: a review of the current literature. Tremor Other Hyperkinet Mov (N Y) 2020; 10 (01) 22
  • 24 Doyle F, Cardoso F, Lopes L. et al. Infusion of Sydenham's chorea antibodies in striatum with up-regulated dopaminergic receptors: a pilot study to investigate the potential of SC antibodies to increase dopaminergic activity. Neurosci Lett 2012; 523 (02) 186-189
  • 25 Cardoso F, Vargas AP, Oliveira LD, Guerra AA, Amaral SV. Persistent Sydenham's chorea. Mov Disord 1999; 14 (05) 805-807
  • 26 Korn-Lubetzki I, Brand A, Steiner I. Recurrence of Sydenham chorea: implications for pathogenesis. Arch Neurol 2004; 61 (08) 1261-1264
  • 27 Maia DP, Fonseca PG, Camargos ST, Pfannes C, Cunningham MC, Cardoso F. Pregnancy in patients with Sydenham's chorea. Parkinsonism Relat Disord 2012; 18 (05) 458-461
  • 28 Robottom BJ, Weiner WJ. Chorea gravidarum. Handb Clin Neurol 2011; 100: 231-235
  • 29 Cervera R, Piette JC, Font J. et al; Euro-Phospholipid Project Group. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002; 46 (04) 1019-1027
  • 30 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; 76 (03) 203-212
  • 31 Harel L, Sandborg C, Lee T, von Scheven E. Neuropsychiatric manifestations in pediatric systemic lupus erythematosus and association with antiphospholipid antibodies. J Rheumatol 2006; 33 (09) 1873-1877
  • 32 Safarpour D, Buckingham S, Jabbari B. Chorea associated with high titers of antiphospholipid antibodies in the absence of antiphospholipid antibody syndrome. Tremor Other Hyperkinet Mov (N Y) 2015; 5 (00) 294
  • 33 Chapman J, Cohen-Armon M, Shoenfeld Y, Korczyn AD. Antiphospholipid antibodies permeabilize and depolarize brain synaptoneurosomes. Lupus 1999; 8 (02) 127-133
  • 34 Baizabal-Carvallo JF, Bonnet C, Jankovic J. Movement disorders in systemic lupus erythematosus and the antiphospholipid syndrome. J Neural Transm (Vienna) 2013; 120 (11) 1579-1589
  • 35 Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011; 10 (01) 63-74
  • 36 Haq AU, Nabi D, Alam M, Ullah SA. The spectrum of movement disorders in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis both in children and adults: an experience from a single tertiary care center. Cureus 2021; 13 (12) e20376
  • 37 Tremont-Lukats IW, Fuller GN, Ribalta T, Giglio P, Groves MD. Paraneoplastic chorea: case study with autopsy confirmation. Neuro-oncol 2002; 4 (03) 192-195
  • 38 Vigliani MC, Honnorat J, Antoine JC. et al; PNS EuroNetwork. Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience. J Neurol 2011; 258 (11) 2058-2068
  • 39 Goldstein L, Djaldetti R, Benninger F. Anti-Yo, chorea and hemiballismus: a case report. J Clin Neurosci 2017; 42: 113-114
  • 40 Pittock SJ, Yoshikawa H, Ahlskog JE. et al. Glutamic acid decarboxylase autoimmunity with brainstem, extrapyramidal, and spinal cord dysfunction. Mayo Clin Proc 2006; 81 (09) 1207-1214
  • 41 Tofaris GK, Irani SR, Cheeran BJ, Baker IWS, Cader ZM, Vincent A. Immunotherapy-responsive chorea as the presenting feature of LGI1-antibody encephalitis. Neurology 2012; 79 (02) 195-196
  • 42 O'Toole O, Lennon VA, Ahlskog JE. et al. Autoimmune chorea in adults. Neurology 2013; 80 (12) 1133-1144
  • 43 Aslam S, Shill H. Chorea in IgLON5-mediated autoimmune encephalitis. Mov Disord Clin Pract (Hoboken) 2020; 7 (Suppl. 03) S83-S84
  • 44 Zekeridou A, Kryzer T, Guo Y. et al. Phosphodiesterase 10A IgG: a novel biomarker of paraneoplastic neurologic autoimmunity. Neurology 2019; 93 (08) e815-e822
  • 45 Kruer MC, Hoeftberger R, Lim KY. et al. Aggressive course in encephalitis with opsoclonus, ataxia, chorea, and seizures: the first pediatric case of γ-aminobutyric acid type B receptor autoimmunity. JAMA Neurol 2014; 71 (05) 620-623
  • 46 Baizabal-Carvallo JF, Jankovic J. Autoimmune and paraneoplastic movement disorders: An update. J Neurol Sci 2018; 385 (385) 175-184
  • 47 Kolesnik M, Ballout AA, Hameed N, Najjar S. Case report: HSV-2 encephalitis presenting with chorea; effects of infection alone or combination of infection and autoimmunity?. Front Neurol 2021; 12 (December): 790514
  • 48 Mancone S, Selvadurai C, Baehring J, Patel A. Choreoathetosis in the setting of human herpesvirus-6 infection in a transplant recipient. Tremor Other Hyperkinet Mov (N Y) 2021; 11 (01) 40
  • 49 Nosadini M, Mohammad SS, Corazza F. et al. Herpes simplex virus-induced anti-N-methyl-d-aspartate receptor encephalitis: a systematic literature review with analysis of 43 cases. Dev Med Child Neurol 2017; 59 (08) 796-805
  • 50 Dimal NPM, Santos NJC, Reyes NGD, Astejada MN, Jamora RDG. Hemichorea-hemiballismus as a presentation of cerebritis from intracranial toxoplasmosis and tuberculosis. Tremor Other Hyperkinet Mov (N Y) 2021; 11 (01) 2
  • 51 Tse W, Cersosimo MG, Gracies JM, Morgello S, Olanow CW, Koller W. Movement disorders and AIDS: a review. Parkinsonism Relat Disord 2004; 10 (06) 323-334
  • 52 Carroll E, Sanchez-ramos J. Hyperkinetic movement disorders associated with HIV and other viral infections. Handb Clin Neurol 2011; 100: 323-334
  • 53 Achenbach J, Faissner S, Saft C. Differential diagnosis of chorea-HIV infection delays diagnosis of Huntington's disease by years. Brain Sci 2021; 11 (06) 1-14
  • 54 Eggers C, Arendt G, Hahn K. et al; German Association of Neuro-AIDS und Neuro-Infectiology (DGNANI). HIV-1-associated neurocognitive disorder: epidemiology, pathogenesis, diagnosis, and treatment. J Neurol 2017; 264 (08) 1715-1727
  • 55 Nath A. Human immunodeficiency virus (HIV) proteins in neuropathogenesis of HIV dementia. J Infect Dis 2002; 186 (Suppl. 02) S193-S198
  • 56 Ozben S, Erol C, Ozer F, Tiras R. Chorea as the presenting feature of neurosyphilis. Neurol India 2009; 57 (03) 347-349
  • 57 Sawczyńska K, Wężyk K, Bosak M. et al. Acute-onset chorea and confusional state in 77-year-old COVID-19 patient: a case report. Neurol Neurochir Pol 2022; 56 (01) 106-110
  • 58 Batot C, Chea M, Zeidan S. et al. Clinical and radiological follow-up of a Pfizer-BioNTech COVID-19 vaccine-induced hemichorea-hemiballismus. Tremor Other Hyperkinet Mov (N Y) 2022; 12 (01) 16
  • 59 Schneider SA, Hennig A, Martino D. Relationship between COVID-19 and movement disorders: a narrative review. Eur J Neurol 2022; 29 (04) 1243-1253
  • 60 Cosentino C, Torres L, Nuñez Y, Suarez R, Velez M, Flores M. Hemichorea/hemiballism associated with hyperglycemia: report of 20 cases. Tremor Other Hyperkinet Mov (N Y) 2016; 6 (00) 402
  • 61 Chua CB, Sun CK, Hsu CW, Tai YC, Liang CY, Tsai IT. “Diabetic striatopathy”: clinical presentations, controversy, pathogenesis, treatments, and outcomes. Sci Rep 2020; 10 (01) 1594
  • 62 Ryan C, Ahlskog JE, Savica R. Hyperglycemic chorea/ballism ascertained over 15 years at a referral medical center. Parkinsonism Relat Disord 2018; 48: 97-100
  • 63 Lucassen EB, Delfyett WT, Stahl MC. Persistent hemichorea and caudate atrophy in untreated diabetic striatopathy: a case report. Case Rep Neurol 2017; 9 (03) 299-303
  • 64 Wang L, Song CL. Chorea associated with nonketotic hyperglycemia: an uncommon patient with bilateral movements. J Clin Neurosci 2015; 22 (06) 1068-1069
  • 65 Faundez T, Klee P, Hanquinet S, Schwitzgebel V, Burkhard PR, Korff CM. Diabetic striatopathy in childhood. Pediatrics 2016; 137 (04) 4-7
  • 66 Yassin AM, Shroff S, Patel SD, Paker AM, Berman MA, Jackson GR. Hemichorea in a patient with diabetic ketoacidosis. J Neurol Sci 2014; 342 (1–2): 189-191
  • 67 Das L, Pal R, Dutta P, Bhansali A. “Diabetic striatopathy” and ketoacidosis: report of two cases and review of literature. Diabetes Res Clin Pract 2017; 128: 1-5
  • 68 Dharsono F, Thompson A, van Heerden J, Cheung A. Susceptibility weighted imaging as a useful imaging adjunct in hemichorea hyperglycaemia. Case Rep Radiol 2013; 2013: 456156
  • 69 Cherian A, Thomas B, Baheti NN, Chemmanam T, Kesavadas C. Concepts and controversies in nonketotic hyperglycemia-induced hemichorea: further evidence from susceptibility-weighted MR imaging. J Magn Reson Imaging 2009; 29 (03) 699-703
  • 70 Arifi B, Gupta S, Sharma S, Daraboina A, Ahuja S. A case report of chorea associated with hyperthyroidism. J Clin Diagn Res 2016; 10 (02) PL01
  • 71 Docherty MJ, Burn DJ. Hyperthyroid chorea. Handb Clin Neurol 2011; 100: 279-286
  • 72 Leblicq C, Duval M, Carmant L, Van Vliet G, Alos N. Rising serum thyroxine levels and chorea in graves' disease. Pediatrics 2013; 131 (02) e616-e619
  • 73 Kondziella D, Brederlau A, Asztely F. Choreathetosis due to abuse of levothyroxine. J Neurol 2009; 256 (12) 2106-2108
  • 74 Yu CHY, Stovel R, Fox S. Chorea–an unusual manifestation in a woman recovering from myxedema coma. Endocr Pract 2012; 18 (03) e43-e48
  • 75 Baba M, Terada A, Hishida R, Matsunaga M, Kawabe Y, Takebe K. Persistent hemichorea associated with thyrotoxicosis. Intern Med 1992; 31 (09) 1144-1146
  • 76 Miao J, Liu R, Li J, Du Y, Zhang W, Li Z. Meige's syndrome and hemichorea associated with hyperthyroidism. J Neurol Sci 2010; 288 (1–2): 175-177
  • 77 Wang HC, Cheng SJ. The syndrome of acute bilateral basal ganglia lesions in diabetic uremic patients. J Neurol 2003; 250 (08) 948-955
  • 78 Hamed S, Mohamed K, Abd Elhameed S. et al. Movement disorders due to selective basal ganglia lesions with uremia. Can J Neurol Sci 2020; 47 (03) 350-365
  • 79 Kim YJ, Kim SJ, Kim J. et al. Chorea due to diabetic hyperglycemia and uremia: distinct clinical and imaging features. Mov Disord 2015; 30 (03) 419-422
  • 80 Rao AR, Kumar P, Gunasekaran V, Dey AB. Reversible chorea secondary to uremia in an older adult. Aging Med (Milton) 2019; 2 (02) 118-120
  • 81 Hans SK, Levine SN. Hypoparathyroidism. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2022
  • 82 Kalampokini S, Georgouli D, Dadouli K. et al. Fahr's syndrome due to hypoparathyroidism revisited: a case of parkinsonism and a review of all published cases. Clin Neurol Neurosurg 2021; 202: 106514
  • 83 Howdle PD, Bone I, Losowsky MS. Hypocalcaemic chorea secondary to malabsorption. Postgrad Med J 1979; 55 (646) 560-563
  • 84 Topakian R, Stieglbauer K, Rotaru J, Haring HP, Aichner FT, Pichler R. Hypocalcemic choreoathetosis and tetany after bisphosphonate treatment. Mov Disord 2006; 21 (11) 2026-2027
  • 85 Fernandez R, Ashraf A, Dure LS. Nutritional vitamin D deficiency presenting as hemichorea. J Child Neurol 2007; 22 (01) 74-76
  • 86 Hrastelj J, McLauchlan D, Rosser A, Clenaghan C. Hypercalcaemia mimicking Huntington's disease: lessons learned from delayed diagnosis. J R Coll Physicians Edinb 2014; 44 (04) 286-288
  • 87 Saltí I, Faris A, Tannir N, Khouri K. Rapid correction by 1-α-hydroxycholecalciferol of hemichorea in surgical hypoparathyroidism. J Neurol Neurosurg Psychiatry 1982; 45 (01) 89-90
  • 88 Paraskevas GP, Vlachos GS, Vassilopoulou S, Anagnostou E, Spengos K, Zis V. Hypoglycemia-induced hemichorea in a patient with Fahr's syndrome. Neurol Sci 2012; 33 (06) 1397-1399
  • 89 Desai K, Walzade P, Ravat SH, Agarwal PA. Adult-onset isolated hemichorea revealing iatrogenic hypoparathyroidism and bilateral basal ganglia calcification. Ann Indian Acad Neurol 2019; 22 (04) 496-499
  • 90 Jin D, Yoon WT, Suh BC, Moon HS, Chung PW, Kim YB. Exacerbation of idiopathic paroxysmal kinesigenic dyskinesia in remission state caused by secondary hypoparathyroidism with hypocalcemia after thyroidectomy: evidence for ion channelopathy. Brain Dev 2012; 34 (10) 840-843
  • 91 Ba F, Miyasaki JM. Movement disorders in pregnancy. Handb Clin Neurol 2020; 172: 219-239
  • 92 Neurological disorders and pregnancy: chorea gravidarum. Continuum (Minneap Minn) 2000; 6: 128-131
  • 93 Delaruelle Z, Honoré PJ, Santens P. Adult-onset Sydenham's chorea or drug-induced movement disorder? A case report. Acta Neurol Belg 2016; 116 (03) 399-400
  • 94 Sharmila V, Babu TA. Oral contraceptive pills induced hemichorea in an adolescent female with polycystic ovarian disease. Indian J Pharmacol 2015; 47 (02) 232-233
  • 95 Smith KM, Dahodwala N. Sex differences in Parkinson's disease and other movement disorders. Exp Neurol 2014; 259: 44-56
  • 96 Baraka AM, Korish AA, Soliman GA, Kamal H. The possible role of estrogen and selective estrogen receptor modulators in a rat model of Parkinson's disease. Life Sci 2011; 88 (19–20): 879-885
  • 97 Marvi MM, Lew MF. Polycythemia and chorea. Handb Clin Neurol 2011; 100: 271-276
  • 98 Kim W, Kim JS, Lee KS, Kim YI, Park CW, Chung YA. No evidence of perfusion abnormalities in the basal ganglia of a patient with generalized chorea-ballism and polycythaemia vera: analysis using subtraction SPECT co-registered to MRI. Neurol Sci 2008; 29 (05) 351-354
  • 99 Miyasaki JM. Chorea caused by toxins. Handb Clin Neurol 2011; 100: 335-346
  • 100 Narula N, Krishnan N, Siddiqui F, Chalhoub M. Cracking the crack dance: a case report on cocane-induced choreoathetosis. Chest 2017; 152 (04) A355
  • 101 Martínez-Dubarbie F, Ricart-Colome C, Manzanedo-Terán B, Infante J. Amphetamine-induced hemichorea. Neurol Sci 2021; 42 (06) 2587-2588
  • 102 Lussier D, Cruciani RA. Choreiform movements after a single dose of methadone. J Pain Symptom Manage 2003; 26 (02) 688-691
  • 103 Verdugo RJ, Ochoa JL. Abnormal movements in complex regional pain syndrome: assessment of their nature. Muscle Nerve 2000; 23 (02) 198-205
  • 104 Bonnet U, Banger M, Wolstein J, Gastpar M. Choreoathetoid movements associated with rapid adjustment to methadone. Pharmacopsychiatry 1998; 31 (04) 143-145
  • 105 Zaatreh M, Tennison M, D'Cruz O, Beach RL. Anticonvulsants-induced chorea: a role for pharmacodynamic drug interaction?. Seizure 2001; 10 (08) 596-599
  • 106 Yim SH, Choi YH, Heo K, Cho KH. A case of dyskinesia after levetiracetam administration. BMC Neurol 2019; 19 (01) 292
  • 107 Attupurath R, Aziz R, Wollman D, Muralee S, Tampi RR. Chorea associated with gabapentin use in an elderly man. Am J Geriatr Pharmacother 2009; 7 (04) 220-224
  • 108 Miller MA, Levsky ME. Choreiform dyskinesia following isolated lamotrigine overdose. J Child Neurol 2008; 23 (02) 243-243
  • 109 Lai MH, Wang TY, Chang CC, Tsai KC, Chang ST. Hemichorea associated with gabapentin therapy with hypoperfusion in contralateral basal ganglion - a case of a paraplegic patient with neuropathic pain. J Clin Pharm Ther 2008; 33 (01) 83-86
  • 110 Patel DM, Gurumukhani JK, Patel MV, Patel GR. Phenytoin induced chorea: a rare adverse effect of the drug. Curr Drug Saf 2019; 14 (01) 51-52
  • 111 Srinivasan S, Lok AW. Valproate-induced reversible hemichorea. Mov Disord 2010; 25 (10) 1511-1512
  • 112 Gatto EM, Aldinio V, Parisi V. et al. Sertraline-induced hemichorea. Tremor Other Hyperkinet Mov (N Y) 2017; 7: 518
  • 113 Bacchin R, Macchione F, Cardellini D. et al. Levofloxacin-induced hemichorea-hemiballism in a patient with previous thalamic infarction. Neurol Sci 2018; 39 (08) 1483-1485
  • 114 Azar S, Ramjiani A, Van Gerpen JA. Ciprofloxacin-induced chorea. Mov Disord 2005; 20 (04) 513-514
  • 115 Li KY, Chien CF, Wu MN, Lai CL, Liou LM. Long-term overdose of cold syrup induced hemichorea-hemiballismus. Neurol Sci 2020; 41 (10) 3007-3008
  • 116 Watari T, Tokuda Y. Drug-induced hemichorea. BMJ Case Rep 2015; 2015: 2014-2015
  • 117 Lee MS, Marsden CD. Neurological sequelae following carbon monoxide poisoning clinical course and outcome according to the clinical types and brain computed tomography scan findings. Mov Disord 1994; 9 (05) 550-558
  • 118 Hsiao CL, Kuo HC, Huang CC. Delayed encephalopathy after carbon monoxide intoxication–long-term prognosis and correlation of clinical manifestations and neuroimages. Acta Neurol Taiwan 2004; 13 (02) 64-70
  • 119 Song IU, Chung SW. Chorea as the first neurological symptom of delayed encephalopathy after carbon monoxide intoxication. Intern Med 2010; 49 (11) 1037-1039
  • 120 Patterson JF. Chronic thallitoxicosis: treatment of the choreiform sequelae. South Med J 1975; 68 (07) 923-925
  • 121 Gil R, Lefèvre JP, Neau JP, Guillard O, Hulin A. Morvan's fibrillary chorea and acrodynic syndrome following mercury treatment [in French]. Rev Neurol (Paris) 1984; 140 (12) 728-733
  • 122 Ghosh R, Dubey S, Chatterjee S, Ghosh M, Ray BK, Benito-León J. Hypermanganesemia induced chorea and cognitive decline in a tea seller. Tremor Other Hyperkinet Mov (N Y) 2020; 10 (01) 45
  • 123 de Gusmao CM, Waugh JL. Inherited and acquired choreas. Semin Pediatr Neurol 2018; 25: 42-53
  • 124 Duyao M, Ambrose C, Myers R. et al. Trinucleotide repeat length instability and age of onset in Huntington's disease. Nat Genet 1993; 4 (04) 387-392
  • 125 Tabrizi SJ, Langbehn DR, Leavitt BR. et al; TRACK-HD investigators. Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data. Lancet Neurol 2009; 8 (09) 791-801
  • 126 Tabrizi SJ, Schobel S, Gantman EC. et al. Huntington's Disease Integrated Staging System (HD-ISS): a novel evidence-based classification system for staging. medRxiv Published online 2021:2021.09.01.21262503. Accessed February 3, 2023 at: https://www.medrxiv.org/content/10.1101/2021.09.01.21262503v1%0Ahttps://www.medrxiv.org/content/10.1101/2021.09.01.21262503v1
  • 127 Gonzalez-Alegre P, Afifi AK. Clinical characteristics of childhood-onset (juvenile) Huntington disease: report of 12 patients and review of the literature. J Child Neurol 2006; 21 (03) 223-229
  • 128 Corrêa BB, Xavier M, Guimarães J. Association of Huntington's disease and schizophrenia-like psychosis in a Huntington's disease pedigree. Clin Pract Epidemiol Ment Health 2006; 2: 1-5
  • 129 Radanović-Grgurić L, Petek A, Laufer D, Koić O, Radanović B, Filaković P. Pharmacologic side effects and/or neurologic disorder: case report. Psychiatr Danub 2009; 21 (04) 575-578
  • 130 Rodrigues FB, Abreu D, Damásio J. et al; REGISTRY Investigators of the European Huntington's Disease Network. Survival, mortality, causes and places of death in a European Huntington's disease prospective cohort. Mov Disord Clin Pract (Hoboken) 2017; 4 (05) 737-742
  • 131 Renton AE, Majounie E, Waite A. et al; ITALSGEN Consortium. A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron 2011; 72 (02) 257-268
  • 132 DeJesus-Hernandez M, Mackenzie IR, Boeve BF. et al. Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 2011; 72 (02) 245-256
  • 133 Hensman Moss DJ, Poulter M, Beck J. et al. C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies. Neurology 2014; 82 (04) 292-299
  • 134 Estevez-Fraga C, Magrinelli F, Hensman Moss D. et al. Expanding the spectrum of movement disorders associated with C9orf72 hexanucleotide expansions. Neurol Genet 2021; 7 (02) e575
  • 135 Anderson DG, Walker RH, Connor M, Carr J, Margolis RL, Krause A. A Systematic review of the Huntington disease-like 2 phenotype. J Huntingtons Dis 2017; 6 (01) 37-46
  • 136 Krause A, Mitchell C, Essop F. et al. Junctophilin 3 (JPH3) expansion mutations causing Huntington disease like 2 (HDL2) are common in South African patients with African ancestry and a Huntington disease phenotype. Am J Med Genet B Neuropsychiatr Genet 2015; 168 (07) 573-585
  • 137 Margolis RL, O'Hearn E, Rosenblatt A. et al. A disorder similar to Huntington's disease is associated with a novel CAG repeat expansion. Ann Neurol 2001; 50 (06) 373-380
  • 138 Margolis RL, Holmes SE, Rosenblatt A. et al. Huntington's disease-like 2 (HDL2) in North America and Japan. Ann Neurol 2004; 56 (05) 670-674
  • 139 Wild EJ, Mudanohwo EE, Sweeney MG. et al. Huntington's disease phenocopies are clinically and genetically heterogeneous. Mov Disord 2008; 23 (05) 716-720
  • 140 Toyoshima Y, Onodera O, Yamada M, Tsuji S, Takahashi H. Spinocerebellar Ataxia Type 17. In: Adam MP, Everman DB, Mirzaa GM. eds. GeneReviews® [Internet]. Seattle, WA: University of Washington, Seattle; 1993: 1993-2023 http://www.ncbi.nlm.nih.gov/pubmed/20301611
  • 141 Stevanin G, Brice A. Spinocerebellar ataxia 17 and Huntington's disease-like 4. In: Wells RD, Ashizawa T. eds. Genetic Instabilities and Neurological Diseases. 2nd ed.. Burlington, MA: Academic Press; 2006: 475-483
  • 142 Nethisinghe S, Lim WN, Ging H. et al. Complexity of the genetics and clinical presentation of spinocerebellar ataxia 17. Front Cell Neurosci 2018; 12 (November): 429
  • 143 Paucar M, Xiang F, Moore R, Walker R, Winnberg E, Svenningsson P. Genotype-phenotype analysis in inherited prion disease with eight octapeptide repeat insertional mutation. Prion 2013; 7 (06) 501-510
  • 144 Heath CA, Cooper SA, Murray K. et al. Diagnosing variant Creutzfeldt-Jakob disease: a retrospective analysis of the first 150 cases in the UK. J Neurol Neurosurg Psychiatry 2011; 82 (06) 646-651
  • 145 Zeidler M, Stewart GE, Barraclough CR. et al. New variant Creutzfeldt-Jakob disease: neurological features and diagnostic tests. Lancet 1997; 350 (9082): 903-907
  • 146 Kambouris M, Bohlega S, Al-Tahan A, Meyer BF. Localization of the gene for a novel autosomal recessive neurodegenerative Huntington-like disorder to 4p15.3. Am J Hum Genet 2000; 66 (02) 445-452
  • 147 Lesperance MM, Burmeister M. Interpretation of linkage data for a Huntington-like disorder mapping to 4p15.3. Am J Hum Genet 2000; 67 (01) 262-263
  • 148 Naito H, Oyanagi S. Familial myoclonus epilepsy and choreoathetosis: hereditary dentatorubral-pallidoluysian atrophy. Neurology 1982; 32 (08) 798-807
  • 149 Braga-Neto P, Pedroso JL, Furtado GV. et al; Rede Neurogenetica. Dentatorubro-Pallidoluysian Atrophy (DRPLA) among 700 families with ataxia in Brazil. Cerebellum 2017; 16 (04) 812-816
  • 150 Le Ber I, Camuzat A, Castelnovo G. et al. Prevalence of dentatorubral-pallidoluysian atrophy in a large series of white patients with cerebellar ataxia. Arch Neurol 2003; 60 (08) 1097-1099
  • 151 Kleiner-Fisman G. Benign hereditary chorea. Handb Clin Neurol 2011; 100: 199-212
  • 152 Breedveld GJ, van Dongen JWF, Danesino C. et al. Mutations in TITF-1 are associated with benign hereditary chorea. Hum Mol Genet 2002; 11 (08) 971-979
  • 153 Inzelberg R, Weinberger M, Gak E. Benign hereditary chorea: an update. Parkinsonism Relat Disord 2011; 17 (05) 301-307
  • 154 Milone R, Masson R, di Cosmo C. et al. A not so benign family pedigree with hereditary chorea: a broader phenotypic expression or additional picture?. Child Neurol Open 2019; 6: 2329048X1982888
  • 155 Bauer P, Kreuz FR, Bürk K. et al. Mutations in TITF1 are not relevant to sporadic and familial chorea of unknown cause. Mov Disord 2006; 21 (10) 1734-1737
  • 156 Mencacci NE, Erro R, Wiethoff S. et al. ADCY5 mutations are another cause of benign hereditary chorea. Neurology 2015; 85 (01) 80-88
  • 157 Raj Kumar K, Fung VSC. ADCY5 identified as a novel cause of benign hereditary chorea. Mov Disord 2015; 30 (13) 1726
  • 158 Chang FCF, Westenberger A, Dale RC. et al. Phenotypic insights into ADCY5-associated disease. Mov Disord 2016; 31 (07) 1033-1040
  • 159 Peikert K, Danek A, Hermann A. Current state of knowledge in chorea-acanthocytosis as core neuroacanthocytosis syndrome. Eur J Med Genet 2018; 61 (11) 699-705
  • 160 Velayos Baeza A, Dobson-Stone C, Rampoldi L. et al. Chorea-acanthocytosis. In: Adam MP, Everman DB, Mirzaa GM. et al, eds. GeneReviews® [Internet]. Seattle, WA: University of Washington, Seattle; 1993-2023
  • 161 Malek N, Newman EJ. Hereditary chorea - what else to consider when the Huntington's disease genetics test is negative?. Acta Neurol Scand 2017; 135 (01) 25-33
  • 162 Niemelä V, Salih A, Solea D. et al. Phenotypic variability in chorea-acanthocytosis associated with novel VPS13A mutations. Neurol Genet 2020; 6 (03) e426
  • 163 Masana M, Rodriguez MJ, Alberch J. Proceedings of the Tenth International Meeting on Neuroacanthocytosis Syndromes. Tremor Other Hyperkinet Mov (N Y) 2021; 11 (01) 19
  • 164 Jung HH, Danek A, Walker RH, Frey BM, Peikert K. McLeod Neuroacanthocytosis Syndrome. In: Adam MP, Everman DB, Mirzaa GM. et al, eds. GeneReviews® [Internet]. Seattle, WA: University of Washington, Seattle; 1993-2023
  • 165 Danek A, Rubio JP, Rampoldi L. et al. McLeod neuroacanthocytosis: genotype and phenotype. Ann Neurol 2001; 50 (06) 755-764
  • 166 Kumar N, Rizek P, Jog M. Neuroferritinopathy: pathophysiology, presentation, differential diagnoses and management. Tremor Other Hyperkinet Mov (N Y) 2016; 6: 355
  • 167 Devos D, Tchofo PJ, Vuillaume I. et al. Clinical features and natural history of neuroferritinopathy caused by the 458dupA FTL mutation. Brain 2009; 132 (Pt 6): e109-e109
  • 168 Chinnery PF, Crompton DE, Birchall D. et al. Clinical features and natural history of neuroferritinopathy caused by the FTL1 460InsA mutation. Brain 2007; 130 (Pt 1): 110-119
  • 169 McNeill A, Birchall D, Hayflick SJ. et al. T2* and FSE MRI distinguishes four subtypes of neurodegeneration with brain iron accumulation. Neurology 2008; 70 (18) 1614-1619
  • 170 McNeill A, Pandolfo M, Kuhn J, Shang H, Miyajima H. The neurological presentation of ceruloplasmin gene mutations. Eur Neurol 2008; 60 (04) 200-205
  • 171 Erro R, Sheerin UM, Bhatia KP. Paroxysmal dyskinesias revisited: a review of 500 genetically proven cases and a new classification. Mov Disord 2014; 29 (09) 1108-1116
  • 172 Harvey S, King MD, Gorman KM. Paroxysmal movement disorders. Front Neurol 2021; 12: 659064
  • 173 Bakels HS, Roos RAC, van Roon-Mom WMC, de Bot ST. Juvenile-onset huntington disease pathophysiology and neurodevelopment: a review. Mov Disord 2022; 37 (01) 16-24
  • 174 Graus F, Dalmou J, Reñé R. et al. Anti-Hu antibodies in patients with small-cell lung cancer: association with complete response to therapy and improved survival. J Clin Oncol 1997; 15 (08) 2866-2872
  • 175 Darnell RB, DeAngelis LM. Regression of small-cell lung carcinoma in patients with paraneoplastic neuronal antibodies. Lancet 1993; 341 (8836): 21-22
  • 176 Criswell LA, Pfeiffer KA, Lum RF. et al. Analysis of families in the multiple autoimmune disease genetics consortium (MADGC) collection: the PTPN22 620W allele associates with multiple autoimmune phenotypes. Am J Hum Genet 2005; 76 (04) 561-571
  • 177 Gilbert DL. Acute and chronic chorea in childhood. Semin Pediatr Neurol 2009; 16 (02) 71-76
  • 178 Baizabal-Carvallo JF, Cardoso F. Chorea in children: etiology, diagnostic approach and management. J Neural Transm (Vienna) 2020; 127 (10) 1323-1342