Evaluation of clinical findings and treatment options of Sydenham chorea patients

Abstract

Background/Aim: Sydenham chorea is an autoimmune neurological disorder of the childhood which occurs due to cross-reaction of antibodies against group A beta-hemolytic streptococci in the basal ganglia. We evaluated patients with Sydenham’s chorea, treatment options, recovery duration, and relapses to determine whether there is any relationship between biochemical parameters such as erythrocyte sedimentation rate (ESR), serum Anti Streptolysin Antibody (ASA), and patients’ clinical course. Methods: This case series includes patients with Sydenham chorea who visited the pediatric neurology outpatient clinics between May 2013 and September 2018. Neurologic examination was performed by a pediatric neurologist, and electrocardiography and echocardiography were performed by a pediatric cardiologist. ESR and ASA levels, treatment options, and clinical course of the disease were evaluated. Results: Sixteen patients, with nine females (56.3%) and seven males (43.7%) were included in this study. The most seen chorea type was hemichorea. The median ASA and ESR values of the patients were 619 IU/ml (278.25-794.75) and 17.5 mm/h (7.25-27), respectively. Their median age and time until recurrence were 12 (9-14.25) years and 16 months (9-18), respectively. The median recovery period was 5.5 months (3-6). Diazepam and haloperidol were the most used secondary treatment options. Mitral insufficiency (MI) was the most frequent finding (56.3%). Benzathine penicillin and secondary prophylaxis, e.g., haloperidol, diazepam were our treatment agents of choice. ASA levels were lower among patients treated with steroids (U=9, z=-2.38, P=0.017). Age was moderately positively correlated with recovery period (age r (14) = 0.738, P=0.001)), while no correlation was found between age and ESR, or recurrence period (P=0.98, P=0.33, respectively). The recovery period of generalized chorea was longer than that of hemichorea (U=10.5, z=-1.96, P=0.05). Recurrence was not related to ASA levels, ESR levels, age, or recovery period (P=0.73, P=0.89, P=0.78, P=0.83 respectively). Conclusion: High ASA levels may not indicate steroid need, and the recovery period increases with age and in cases of generalized chorea. Instead of benzathine penicillin or other known secondary prophylactic agents, azithromycin and levetiracetam can be used in hypersensitive patients.

Keywords

• Sydenham chorea
• treatment
• levetracetam
• azithromycine

References

1. 1. Swedo SE, Leonard HL, Schapiro MB, Casey BJ, Mannheim GB, Lenane MC, Rettew DC: Sydenham chorea - physical and psychological symptoms of St.Vitus dance. Pediatrics. 1993;91:706–13.
2. 2. Walker KG, Wilmshurst MJ. An update on the treatment of Sydenham’s chorea: the evidence for established and evolving interventions. Ther Adv Neurol Disord. 2010;3:301-9.
3. 3. Hancı F, Hizal M, Türay S, Kalaycıoğlu O, Kabakuş N. Sydenham's Chorea; Clinical and Magnetic Resonance Imaging Findings, a Retrospective Observational Study in Children. J Pediatr Neurol.2020;18:217-22.
4. 4. Miranda M, Walker RH, Saez D, Renner V. Severe Sydenham chorea (chorea paralytica) successfully treated with plasmapheresis. J Clin Mov Disord. 2015;21:2:2.
5. 5. Gewitz MH, Baltimore RS, Tani LY, Sable CA, Shulman ST, Carapetis J, 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;131:1806–18.
6. 6. Loiselle CR, Singer HS. Genetics of childhood disorders: XXXI. Autoimmune disorders, part 4: is Sydenham chorea an autoimmune disorder? J Am Acad Child Adolesc Psychiatry. 2001;40:1234-36.
7. 7. Faustino PC, Terreri MT, da Rocha AJ, Zappitelli MC, Lederman HM, Hilaｴrio MO. Clinical, laboratory, psychiatric and magnetic resonance findings in patients with Sydenham’s chorea. Neuroradiology. 2003;45:456-62.
8. 8. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. JAMA. 1992;268:2069-73.

9. 9. Oosterveer DM, Overweg-Plandsoen WC, Roos RA. Sydenham's chorea: a practical overview of the current literatüre. Pediatr Neurol. 2010;43:1-6.
10. 10. Cox CJ, Sharma M, Leckman JF, Zuccolo J, Zuccolo A, Kovoor A 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;191:5524-41.
11. 11. Genel F, Arslanoglu S, Uran N, Saylan B. Sydenham’s chorea: clinical findings and comparison of the efficacies of sodium valproate and carbamazepine regimens. Brain Dev. 2002;24:73-6.
12. 12. Weiner SG, Normandin PA. Sydenham chorea: a case report and review of the literature. Pediatr Emerg Care. 2007;23:20-4.
13. 13. Öncel ED, Özsürekçi Y, Konuşkan B, Haliloğlu G, Ertuğrul İ, Alehan D, Kara A. Sydenham Koresi: Olgu Sunumu ve Literatürün Gözden Geçirilmesi. Pediatr Inf. 2012;6:54-8.
14. 14. Alrabiah H. Levetiracetam. Profiles Drug Subst Excip Relat Methodol. 2019;44:167-204.
15. 15. Striano P, Elefante A, Coppola A, Tortora F, Zara F, Minetti C, Striano S. Dramatic response to levetiracetam in post‐ischaemic Holmes' tremor. J Neurol Neurosurg Psychiatry. 2007;78:438–9.
16. 16. Vles GF, Hendriksen JG, Visschers A, Speth L, Nicolai J, Vles JS. Levetiracetam therapy for treatment of choreoathetosis in dyskinetic cerebral palsy. Dev Med Child Neurol. 2009;51:487-90.
17. 17. Sahin 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;38:144–6.
18. 18. Paz JA, Silva CAA, Marques-Dias MJ. Randomized double blind study with prednisone in Sydenham’s chorea. Pediatr Neurol. 2006;34:264-9.
19. 19. Dean SL, Singer HS. Treatment of Sydenham's Chorea: A Review of the Current Evidence. Tremor Other Hyperkinet Mov (N Y). 2017;1:7:456.
20. 20. Perlmutter SJ, Leitman SF, Garvey MA, Hamburger S, Feldman E, Leonard HL, et al. Therapeutic plasma exchange and intravenous immunoglobulin for obsessive compulsive disorder and tic disorders in childhood. Lancet. 1999;354:1153-8.
21. 21. Fusco C, Spagnoli C. Corticosteroid treatment in Sydenham's chorea. Eur J Paediatr Neurol. 2018;22:327-31.
22. 22. Cilliers AM. Rheumatic fever and its management. BMJ. 2006;333:1153-6.
23. 23. van Driel ML, De Sutter AI, Habraken H, Thorning S, Christiaens T. Different antibiotic treatments for group A streptococcal pharyngitis. Cochrane Database Syst Rev. 2016;11:CD004406.
24. 24. Working Group on Pediatric Acute Rheumatic Fever and Cardiology Chapter of Indian Academy of Pediatrics, Saxena A, Kumar RK, Gera RP, Radhakrishnan S, Mishra S, Ahmed Z. Consensus guidelines on pediatric acute rheumatic fever and rheumatic heart disease. Indian Pediatr. 2008;45:565-73.
25. 25. Gurkas E, Karalok ZS, Taskin BD, Aydogmus U, Guven A, Degerliyurt A, et al. Predictors of recurrence in Sydenham's chorea: Clinical observation from a single center. Brain Dev. 2016;38:827-34.
26. 26. Tumas V, Caldas CT, Santos AC, Nobre A, Fernandes RM. Sydenham’s chorea: clinical observations from a Brazilian movement disorder clinic. Parkinsonism Relat Disord. 2007;13:276–83.
27. 27. Shannon L. Dean, Harvey S. Singer. Treatment of Sydenham’s Chorea: A Review of the Current Evidence. Tremor Other Hyperkinet Mov (N Y). 2017;7:456.