Sydenham’s chorea: an update on pathophysiology, clinical features and management

References

• Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation. 2015;131:1806–1818.
   PubMed Web of Science ®Google Scholar
• Karthikeyan G, Guilherme L. Acute rheumatic fever. Lancet. 2018;392:161–174.
   PubMed Web of Science ®Google Scholar
• Cardoso F. Sydenham’s Chorea. Curr Treat Options Neurol. 2008;10:230–235.
   PubMed Web of Science ®Google Scholar
• Cardoso F. Sydenham’s chorea. Handb Clin Neurol. 2011;100:221–229.
   PubMedGoogle Scholar
• Church AJ, Dale RC, Cardoso F, et al. CSF and serum immune parameters in Sydenham’s chorea: evidence of an autoimmune syndrome? J Neuroimmunol. 2003;136:149–153.
   PubMed Web of Science ®Google Scholar
• Dale RC, Merheb V, Pillai S, et al. Antibodies to surface dopamine-2 receptor in autoimmune movement and psychiatric disorders. Brain. 2012;135:3453–3468.
   PubMed Web of Science ®Google Scholar
• Seckeler MD, Hoke TR. The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease. Clin Epidemiol. 2011;22:67–84.
   Google Scholar
• Cardoso F, Eduardo C, Silva AP, et al. Chorea in fifty consecutive patients with rheumatic fever. Mov Disord. 1997;12:701–703.
   PubMed Web of Science ®Google Scholar
• Carapetis JR, Currie BJ. Rheumatic chorea in northern Australia: a clinical and epidemiological study. Arch Dis Child. 1999;80:353–358.
   PubMed Web of Science ®Google Scholar
• Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet. 2005;366:155–168.
   PubMed Web of Science ®Google Scholar
• Gimeno H, Barry S, Lin JP, et al. Functional impact of Sydenham’s chorea: a case report. Tremor Other Hyperkinet Mov (N Y). 2013;3:pii: tre-03-128-3381–1.
   PubMedGoogle Scholar
• Kirvan CA, Swedo SE, Heuser JS, et al. Mimicry and autoantibody-mediated neuronal cell signaling in Sydenham chorea. Nat Med. 2003;9:914–920.
   PubMed Web of Science ®Google Scholar
• Kirvan CA, Cox CJ, Swedo SE, et al. Tubulin is a neuronal target of autoantibodies in Sydenham’s chorea. J Immunol. 2007;178:7412–7421.
   PubMed Web of Science ®Google Scholar
• Cox CJ, Sharma M, Leckman JF, et al. Brain human monoclonal autoantibody from Sydenham chorea targets dopaminergic neurons in transgenic mice and signals dopamine D2 receptor: implications in human disease. J Immunol. 2013;191:5524–5541.
   PubMed Web of Science ®Google Scholar
• Brimberg L, Benhar I, Mascaro-Blanco A, et al. Behavioral, pharmacological, and immunological abnormalities after streptococcal exposure: a novel rat model of Sydenham chorea and related neuropsychiatric disorders. Neuropsychopharmacology. 2012;37:2076–2087.
   PubMed Web of Science ®Google Scholar
• Brilot F, Merheb V, Ding A, et al. Antibody binding to neuronal surface in Sydenham chorea, but not in PANDAS or Tourette syndrome. Neurology. 2011;76:1508–1513.
   PubMed Web of Science ®Google Scholar
• Ben-Pazi H, Stoner JA, Cunningham MW. Dopamine receptor autoantibodies correlate with symptoms in Sydenham’s chorea. PLoS One. 2013;8:e73516.
   PubMed Web of Science ®Google Scholar
• Lotan D, Benhar I, Alvarez K, et al. Behavioral and neural effects of intra-striatal infusion of anti-streptococcal antibodies in rats. Brain Behav Immun. 2014;38:249–262.
   PubMed Web of Science ®Google Scholar
• Dileepan T, Smith ED, Knowland D, et al. Group A Streptococcus intranasal infection promotes CNS infiltration by streptococcal-specific Th17 cells. J Clin Invest. 2016;126:303–317.
   PubMed Web of Science ®Google Scholar
• Ehrlich DJ, Walker RH. Functional neuroimaging and chorea: a systematic review. J Clin Mov Disord. 2017;4:8.
   PubMedGoogle Scholar
• Giorgio SMDA, Caprio MG, Galante F, et al. Clinical value of perfusion abnormalities of brain on technetium-99m HMPAO single-photon emission computed tomography in children with Sydenham chorea. J Child Neurol. 2017;32:316–321.
   PubMed Web of Science ®Google Scholar
• Beato R, Siqueira CF, Marroni BJ, et al. Brain SPECT in Sydenham’s chorea in remission. Mov Disord. 2014;29:256–258.
   PubMed Web of Science ®Google Scholar
• Torres KCDL, Rocha NP, de Rezende VB, et al. Persistent Sydenham’s chorea is not associated with sustained lymphocyte dysfunction. Arq Neuropsiquiatr. 2016;74:5–9.
   PubMed Web of Science ®Google Scholar
• Dale RC, Brilot F. Autoimmune basal ganglia disorders. J Child Neurol. 2012;27:1470–1481.
   PubMed Web of Science ®Google Scholar
• Hounie AG, Pauls DL, Do Rosario-Campos MC, et al. Obsessive-compulsive spectrum disorders and rheumatic fever: a family study. Biol Psychiatry. 2007;61:266–272.
   PubMed Web of Science ®Google Scholar
• Bryant PA, Robins-Browne R, Carapetis JR, et al. Some of the people, some of the time: susceptibility to acute rheumatic fever. Circulation. 2009;119:742–753.
   PubMed Web of Science ®Google Scholar
• Guilherme L, Weidebach W, Kiss MH, et al. Association of human leukocyte class II antigens with rheumatic fever or rheumatic heart disease in a Brazilian population. Circulation. 1991;83:1995–1998.
   PubMed Web of Science ®Google Scholar
• Gray LA, D’Antoine HA, Tong SYC, et al. Genome-wide analysis of genetic risk factors for rheumatic heart disease in aboriginal australians provides support for pathogenic molecular mimicry. J Infect Dis. 2017;216:1460–1470.
   PubMed Web of Science ®Google Scholar
• Parks T, Mirabel MM, Kado J, et al. Association between a common immunoglobulin heavy chain allele and rheumatic heart disease risk in Oceania. Nat Commun. 2017;8:14946.
   PubMed Web of Science ®Google Scholar
• Donadi EA, Smith AG, Louzada-Junior P, et al. HLA class I and class II profiles of patients presenting with Sydenham’s chorea. J Neurol. 2000;247:122–128.
   PubMed Web of Science ®Google Scholar
• Stanevicha V, Eglite J, Zavadska D, et al. HLA class II DR and DQ genotypes and haplotypes associated with rheumatic fever among a clinically homogeneous patient population of Latvian children. Arthritis Res Ther. 2007;9:R58.
   PubMed Web of Science ®Google Scholar
• Kilic A, Unuvar E, Tatli B, et al. Neurologic and cardiac findings in children with Sydenham chorea. Pediatr Neurol. 2007;36:159–164.
   PubMed Web of Science ®Google Scholar
• Cardoso F. Autoimmune choreas. J Neurol Neurosurg Psychiatry. 2017;88:412–417.
   PubMedGoogle Scholar
• Cardoso F, Vargas AP, Oliveira LD, et al. Persistent Sydenham’s chorea. Mov Disord. 1999;14:805–807.
   PubMed Web of Science ®Google Scholar
• Walker AR, Tani LY, Thompson JA, et al. Rheumatic chorea: relationship to systemic manifestations and response to corticosteroids. J Pediatr. 2007;151:679–683.
   PubMed Web of Science ®Google Scholar
• Korn-Lubetzki I, Brand A, Steiner I. Recurrence of Sydenham chorea: implications for pathogenesis. Arch Neurol. 2004;61:1261–1264.
   PubMed Web of Science ®Google Scholar
• Gurkas E, Karalok ZS, Taskin BD, et al. Predictors of recurrence in Sydenham’s chorea: clinical observation from a single center. Brain Dev. 2016;38:827–834.
   PubMed Web of Science ®Google Scholar
• Maia DP, Fonseca PG, Camargos ST, et al. Pregnancy in patients with Sydenham’s chorea. Park Relat Disord. 2012;18:458–461.
   PubMed Web of Science ®Google Scholar
• Oliveira PM, Cardoso F, Maia DP, et al. Acoustic analysis of prosody in Sydenham’s chorea. Arq Neuropsiquiatr. 2010;68:744–748.
   PubMed Web of Science ®Google Scholar
• de Teixeira AL, Cardoso F, Maia DP, et al. Frequency and significance of vocalizations in Sydenham’s chorea. Park Relat Disord. 2009;15:62–63.
   PubMed Web of Science ®Google Scholar
• Cairney S, Maruff P, Currie J, et al. Increased anti-saccade latency is an isolated lingering abnormality in Sydenham chorea. J Neuroophthalmol. 2009;29:143–145.
   PubMed Web of Science ®Google Scholar
• Ridel KR, Lipps TD, Gilbert DL. The prevalence of neuropsychiatric disorders in Sydenham’s chorea. Pediatr Neurol. 2010;42:243–248.
   PubMed Web of Science ®Google Scholar
• Swedo SE, Rapoport JL, Cheslow DL, et al. High prevalence of obsessive-compulsive symptoms in patients with Sydenham’s chorea. Am J Psychiatry. 1989;146:246–249.
   PubMed Web of Science ®Google Scholar
• Asbahr FR, Garvey MA, Snider LA, et al. Obsessive-compulsive symptoms among patients with Sydenham chorea. Biol Psychiatry. 2005;57:1073–1076.
   PubMed Web of Science ®Google Scholar
• Moreira J, Kummer A, Harsanyi E, et al. Psychiatric disorders in persistent and remitted Sydenham’s chorea. Park Relat Disord. 2014;20:233–236.
   PubMed Web of Science ®Google Scholar
• Mercadante MT, Busatto GF, Lombroso PJ, et al. The psychiatric symptoms of rheumatic fever. Am J Psychiatry. 2000;157:2036–2038.
   PubMed Web of Science ®Google Scholar
• de Alvarenga PG, Floresi AC, Torres AR, et al. Higher prevalence of obsessive-compulsive spectrum disorders in rheumatic fever. Gen Hosp Psychiatry. 2009;31:178–180.
   PubMed Web of Science ®Google Scholar
• Hounie AG, Pauls DL, Mercadante MT, et al. Obsessive-compulsive spectrum disorders in rheumatic fever with and without Sydenham’s chorea. J Clin Psychiatry. 2004;65:994–999.
   PubMed Web of Science ®Google Scholar
• Maia DP, Teixeira AL Jr., Quintao Cunningham MC, et al. Obsessive compulsive behavior, hyperactivity, and attention deficit disorder in Sydenham chorea. Neurology. 2005;64:1799–1801.
   PubMed Web of Science ®Google Scholar
• Cavalcanti A, Hilário MOE, Dos Santos FH, et al. Subtle cognitive deficits in adults with a previous history of Sydenham’s chorea during childhood. Arthritis Care Res. 2010;62:1065–1071.
   PubMed Web of Science ®Google Scholar
• Beato R, Maia DP, Teixeira AL Jr., et al. Executive functioning in adult patients with Sydenham’s chorea. Mov Disord. 2010;25:853–857.
   PubMed Web of Science ®Google Scholar
• Cunningham MC, Maia DP, Teixeira AL Jr., et al. Sydenham’s chorea is associated with decreased verbal fluency. Park Relat Disord. 2006;12:165–167.
   PubMed Web of Science ®Google Scholar
• Harsanyi E, Moreira J, Kummer A, et al. Language impairment in adolescents with Sydenham chorea. Pediatr Neurol. 2015;53:412–416.
   PubMed Web of Science ®Google Scholar
• Teixeira AL Jr., Meira FC, Maia DP, et al. Migraine headache in patients with Sydenham’s chorea. Cephalalgia. 2005;25:542–544.
   PubMedGoogle Scholar
• Beier K, Pratt DP. Sydenham Chorea. StatPearls [Internet]. Treasure Island (FL); 2019. Available from: https://www.ncbi.nlm.nih.gov/pubmed/28613588
   Google Scholar
• Marijon E, Mirabel M, Celermajer DS, et al. Rheumatic heart disease. Lancet. 2012;379(9819):953–964.
   PubMed Web of Science ®Google Scholar
• Emery ES, Vieco PT. Sydenham chorea: magnetic resonance imaging reveals permanent basal ganglia injury. Neurology. 1997;48:531–533.
   PubMed Web of Science ®Google Scholar
• Faustino PC, Terreri MT, da Rocha AJ, et al. Clinical, laboratory, psychiatric and magnetic resonance findings in patients with Sydenham chorea. Neuroradiology. 2003;45:456–462.
   PubMed Web of Science ®Google Scholar
• Teixeira AL, Maia DP, Cardoso F. UFMG Sydenham’s Chorea Rating Scale (USCRS): reliability and consistency. Mov Disord. 2005;20:585–591.
   PubMed Web of Science ®Google Scholar
• Dean SL, Singer HS. Treatment of Sydenham’s chorea: a review of the current evidence. Tremor Other Hyperkinet Mov (N Y). 2017;7:456.
   PubMed Web of Science ®Google Scholar
• Walker KG, Wilmshurst JM. An update on the treatment of Sydenham-s chorea: the evidence for established and evolving interventions. Ther Adv Neurol Disord. 2010;3:301–319.
   PubMedGoogle Scholar
• Leal M, Passos LSA, F V G, et al. Rheumatic heart disease in the modern era: recent developments and current challenges. Rev Soc Bras Med Trop. 2019;52:e20180041.
   PubMed Web of Science ®Google Scholar
• Carapetis JR, Beaton A, Cunningham M, et al. Acute rheumatic fever and rheumatic heart disease. Nat Rev Dis Prim. 2016;2:15084.
   PubMed Web of Science ®Google Scholar
• Gerber MA, Baltimore RS, Eaton CB, et al. Prevention of rheumatic fever and diagnosis and treatment of acute streptococcal pharyngitis: A scientific statement from the American Heart Association Rheumatic Fever,Endocarditis,and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young,the Interdisciplinary Council on Functional Genomics and Translational Biology,and the Interdisciplinary Council on Quality of Care. Circulation. 2009;119:1541–1551.
   PubMed Web of Science ®Google Scholar
• Cunningham MW. Rheumatic fever, autoimmunity, and molecular mimicry: the streptococcal connection. Int Rev Immunol. 2014;33:314–329.
   PubMed Web of Science ®Google Scholar
• Demiroren K, Yavuz H, Cam L, et al. Sydenham’s chorea: a clinical follow-up of 65 patients. J Child Neurol. 2007;22:550–554.
   PubMed Web of Science ®Google Scholar
• Teixeira AL, Cardoso F, Maia DP, et al. Sydenham’s chorea may be a risk factor for drug induced parkinsonism. J Neurol Neurosurg Psychiatry. 2003;74:1350–1351.
   PubMed Web of Science ®Google Scholar
• Sethi S, Setiya R, Lallar K. Successful treatment of Sydenham chorea with olanzapine. J Pediatr Neurol. 2006;4:171–174.
   Google Scholar
• Teixeira AL, Maia DP, Cardoso F. Efficacy and safety of risperidone in the treatment of Sydenham´s chorea. In: the movement disorder society’s 9th international congress of Parkinson’s disease and movement disorders, 2005, New Orleans LA USA. Mov Disord. 2005;20:S8–S8.
   Google Scholar
• Bashir H, Jankovic J. Treatment options for chorea. Expert Rev Neurother. 2018;18(1):51–63.
   PubMed Web of Science ®Google Scholar
• Singer C. Comprehensive treatment of Huntington disease and other choreic disorders. Cleve Clin J Med. 2012;79:S30–S34.
   PubMedGoogle Scholar
• Zaki S, Shanbag P, Lad V. Quadriparesis and dysarthria due to tetrabenazine therapy in a child with rheumatic chorea. Indian J Pharmacol. 2011;43:601–602.
   PubMed Web of Science ®Google Scholar
• Pena J, Mora E, Cardozo J, et al. Comparison of the efficacy of carbamazepine, haloperidol and valproic acid in the treatment of children with Sydenham’s chorea: clinical follow-up of 18 patients. Arq Neuropsiquiatr. 2002;60:374–377.
   PubMed Web of Science ®Google Scholar
• Genel F, Arslanoglu S, Uran N, et al. Sydenham’s chorea: clinical findings and comparison of the efficacies of sodium valproate and carbamazepine regimens. Brain Dev. 2002;24:73–76.
   PubMed Web of Science ®Google Scholar
• Appleton RE, Jan JE. Efficacy of valproic acid in the treatment of Sydenham’s chorea. J Child Neurol. 1988;3:147.
   PubMedGoogle Scholar
• Daoud AS, Zaki M, Shakir R, et al. Effectiveness of sodium valproate in the treatment of Sydenham’s chorea. Neurology. 1990;40:1140–1141.
   PubMed Web of Science ®Google Scholar
• Harel L, Zecharia A, Straussberg R, et al. Successful treatment of rheumatic chorea with carbamazepine. Pediatr Neurol. 2000;23:147–151.
   PubMed Web of Science ®Google Scholar
• Şahin S, Cansu A. A new alternative drug with fewer adverse effects in the treatment of Sydenham chorea: levetiracetam efficacy in a child. Clin Neuropharmacol. 2015;38:144–146.
   PubMed Web of Science ®Google Scholar
• Barash J, Margalith D, Matitiau A. Corticosteroid treatment in patients with Sydenham’s chorea. Pediatr Neurol. 2005;32:205–207.
   PubMed Web of Science ®Google Scholar
• Cardoso F, Maia D, Cunningham MCQS, et al. Treatment of Sydenham chorea with corticosteroids. Mov Disord. 2003;18:1374–1377.
   PubMed Web of Science ®Google Scholar
• Teixeira Jr. AL Jr., Maia DP, Cardoso F. Treatment of acute Sydenham’s chorea with methyl-prednisolone pulse-therapy. Parkinsonism Relat Disord. 2005;11:327–330.
   PubMed Web of Science ®Google Scholar
• Fusco C, Spagnoli C. Corticosteroid treatment in Sydenham’s chorea. Eur J Paediatr Neurol. 2018;22:327–331.
   PubMed Web of Science ®Google Scholar
• Fusco C, Ucchino V, Frattini D, et al. Acute and chronic corticosteroid treatment of ten patients with paralytic form of Sydenham’s chorea. Eur J Paediatr Neurol. 2012;16:373–378.
   PubMed Web of Science ®Google Scholar
• Green LN. Corticosteroids in the treatment of Sydenham’s chorea. Arch Neurol. 1978;35:53–54.
   PubMedGoogle Scholar
• Paz JA, Silva CA, Marques-Dias MJ. Randomized double-blind study with prednisone in Sydenham’s chorea. Pediatr Neurol. 2006;34:264–269.
   PubMed Web of Science ®Google Scholar
• Garvey MA, Snider LA, Leitman SF, et al. Treatment of Sydenham’s chorea with intravenous immunoglobulin, plasma exchange, or prednisone. J Child Neurol. 2005;20:424–429.
   PubMed Web of Science ®Google Scholar
• Mohammad SS, Dale RC. Principles and approaches to the treatment of immune-mediated movement disorders. Eur J Paediatr Neurol. 2018;22:292–300.
   PubMed Web of Science ®Google Scholar
• Walker K, Brink A, Lawrenson J, et al. Treatment of sydenham chorea with intravenous immunoglobulin. J Child Neurol. 2012;27:147–155.
   PubMed Web of Science ®Google Scholar
• Gregorowski C, Lochner C, Martin L, et al. Neuropsychological manifestations in children with sydenham’s chorea after adjunct intravenous immunoglobulin and standard treatment. Metab Brain Dis. 2016;31:205–212.
   PubMed Web of Science ®Google Scholar
• Boersma NA, Schippers H, Kuijpers T, et al. Successful treatment of Sydenham’s chorea with intravenous immunoglobulin. BMJ Case Rep. 2016;2016: bcr2015211673.
   Google Scholar
• Mohammad SS, Nosadini M, Grattan-Smith P, et al. Intravenous immunoglobulin in acute Sydenham’s chorea: A systematic review. J Paediatr Child Health. 2015;51:1235–1238.
   PubMed Web of Science ®Google Scholar
• van Immerzeel TD, van Gilst RM, Hartwig NG. Beneficial use of immunoglobulins in the treatment of Sydenham chorea. Eur J Pediatr. 2010;169:1151–1154.
   PubMed Web of Science ®Google Scholar
• Padilha S, Virtuoso S, Tonin FS, et al. Efficacy and safety of drugs for attention deficit hyperactivity disorder in children and adolescents: a network meta-analysis. Eur Child Adolesc Psychiatry. 2018;27:1335–1345.
   PubMed Web of Science ®Google Scholar
• Varigonda AL, Jakubovski E, Bloch MH. Systematic review and meta-analysis: early treatment responses of selective serotonin reuptake inhibitors and clomipramine in pediatric obsessive-compulsive disorder. J Am Acad Child Adolesc Psychiatry. 2016;55(851–9):e2.
   Google Scholar
• Abramowitz JS, Blakey SM, Buchholz JL, et al. Systematic review and meta-analysis: early treatment responses of selective serotonin reuptake inhibitors and clomipramine in pediatric obsessive-compulsive disorder. Eur Child Adolesc Psychiatry. 2005;11:1335–1345.
   Google Scholar
• Abramowitz JS, Blakey SM, Reuman L, et al. New directions in the cognitive-behavioral treatment of OCD: theory, research, and practice. Behav Ther. 2018;49:311–322.
   PubMed Web of Science ®Google Scholar