Document Type : Original Article(s)
Authors
1 Pediatric Health Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
2 Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran
3 Infectious and Tropical Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
Abstract
Background: Although infrequent, Sydenham’s chorea (SC) may occur as a result of injury to the basal ganglia in children with acute rheumatic fever (ARF) secondary to group A Streptococcal infection. Certain hallmarks of SC, such as movement disorders, could be utilized as a predictive marker for carditis. The present study aimed to investigate neurologic and cardiologic symptoms in children with suspected SC after ARF.
Methods: All children aged 5-16 who were admitted at Shahid Madani Pediatric Hospital (Tabriz, Iran), with an initial diagnosis of ARF and SC between 2009 and 2022 were included for echocardiographic assessment and prospective follow-up within 6 and 12 months after the start point. The pattern and severity of valvulopathy, as well as the prevalence of Jones criteria for rheumatic fever, were used to assess the effect. The collected data were analyzed using SPSS Statistics software (version 22.0) using Chi square and Fisher’s exact tests. P<0.05 was considered statistically significant.
Results: The study enrolled 85 children, 36 girls and 49 boys, with a mean age of 9.7±2.7. On the first echocardiography, 42.4% of patients had mitral valve regurgitation (MR), with a predominance of female patients (P=0.04). Of those diagnosed with SC (12 girls and 6 boys), 66.7% showed cardiac involvement, with a higher prevalence of MR in both sexes (P=0.04). The pattern of cardiac involvement after 6 months was significantly different between the groups (P=0.04). However, no such difference was observed during the one-year follow-up (P=0.07). Female sex was found to have a significant relationship with SC localization (P=0.01).
Conclusion: In addition to its neurological manifestations, SC can be associated with clinical or subclinical cardiac valve dysfunction that might last for more than a year. In addition to attempting early detection and appropriate management, a precise cardiac and neurologic assessment during admission and follow-up is recommended.
Keywords
- Auala T, Zavale BG, Mbakwem AC, Mocumbi AO. Acute Rheumatic Fever and Rheumatic Heart Disease: Highlighting the Role of Group A Streptococcus in the Global Burden of Cardiovascular Disease. Pathogens. 2022;11. doi: 10.3390/pathogens11050496. PubMed PMID: 35631018; PubMed Central PMCID: PMCPMC9145486.
- Zimmerman M, Sable C. Acute Rheumatic Fever and Rheumatic Heart Disease. In: Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult. 3th Ed. New Jersey: Wiley-Blackwell; 2021. p. 856-69. doi: 10.1002/9781119612858.ch40.
- Orsini A, Foiadelli T, Sica A, Santangelo A, Carli N, Bonuccelli A, et al. Psychopathological Impact in Patients with History of Rheumatic Fever with or without Sydenham’s Chorea: A Multicenter Prospective Study. Int J Environ Res Public Health. 2022;19. doi: 10.3390/ijerph191710586. PubMed PMID: 36078300; PubMed Central PMCID: PMCPMC9517806.
- Vreeland A, Thienemann M, Cunningham M, Muscal E, Pittenger C, Frankovich J. Neuroinflammation in Obsessive-Compulsive Disorder: Sydenham Chorea, Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections, and Pediatric Acute Onset Neuropsychiatric Syndrome. Psychiatr Clin North Am. 2023;46:69-88. doi: 10.1016/j.psc.2022.11.004. PubMed PMID: 36740356.
- Chain JL, Alvarez K, Mascaro-Blanco A, Reim S, Bentley R, Hommer R, et al. Autoantibody Biomarkers for Basal Ganglia Encephalitis in Sydenham Chorea and Pediatric Autoimmune Neuropsychiatric Disorder Associated With Streptococcal Infections. Front Psychiatry. 2020;11:564. doi: 10.3389/fpsyt.2020.00564. PubMed PMID: 32670106; PubMed Central PMCID: PMCPMC7328706.
- Tanz RR, Heaberlin LE, Harvey E, Katsogridakis YL, Burns RR, Rippe J, et al. Performance of a Molecular Test for Group A Streptococcus Pharyngitis. J Pediatric Infect Dis Soc. 2023;12:56-9. doi: 10.1093/jpids/piac115. PubMed PMID: 36322677.
- Risavi BL, Iszkula E, Yost B. Sydenham’s Chorea. J Emerg Med. 2019;56:e119-e21. doi: 10.1016/j.jemermed.2019.02.012. PubMed PMID: 31003820.
- Hawkes MA, Ameriso SF. Neurologic complications of rheumatic fever. Handb Clin Neurol. 2021;177:23-31. doi: 10.1016/B978-0-12-819814-8.00002-0. PubMed PMID: 33632442.
- Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. 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. JAMA. 1992;268:2069-73. PubMed PMID: 1404745.
- Çiftlik SS. Rheumatic Heart Diseases. Chronic Disease Follow-Ups for Adults in Primary Care. New York: Nova Science; 2022. p. 257-66.
- Vasconcelos LPB, da Silva Bastos Vasconcelos MC, Di Flora F, de Oliveira FAP, Lima PD, Silva L, et al. Neurological and Psychiatric Disorders in Patients with Rheumatic Heart Disease: Unveiling what is Beyond Cardiac Manifestations. Glob Heart. 2022;17:62. doi: 10.5334/gh.1149. PubMed PMID: 36199561; PubMed Central PMCID: PMCPMC9438462.
- Fabi M, Calicchia M, Miniaci A, Balducci A, Tronconi E, Bonetti S, et al. Carditis in Acute Rheumatic Fever in a High-Income and Moderate-Risk Country. J Pediatr. 2019;215:187-91. doi: 10.1016/j.jpeds.2019.07.072. PubMed PMID: 31587860.
- Marino A, Cimaz R, Pelagatti MA, Tattesi G, Biondi A, Menni L, et al. Acute Rheumatic Fever: Where Do We Stand? An Epidemiological Study in Northern Italy. Front Med (Lausanne). 2021;8:621668. doi: 10.3389/fmed.2021.621668. PubMed PMID: 33718402; PubMed Central PMCID: PMCPMC7943448.
- Corsenac P, Heenan RC, Roth A, Rouchon B, Guillot N, Hoy D. An epidemiological study to assess the true incidence and prevalence of rheumatic heart disease and acute rheumatic fever in New Caledonian school children. J Paediatr Child Health. 2016;52:739-44. doi: 10.1111/jpc.13185. PubMed PMID: 27203400.
- Sujhithra A, Jayanthi S, Chokkalingam M, Danisvijay D, Vidhya R, Rajaratnam SA. Echocardiographic Parameters, Clinical Profile and Presence of Streptococcus pyogenes Virulent Genes in Pharyngitis and Rheumatic Fever. Journal of Pure & Applied Microbiology. 2022;16:1028-38. doi: 10.22207/JPAM.16.2.27.
- Orsini A, Foiadelli T, Magistrali M, Carli N, Bagnasco I, Dassi P, et al. A nationwide study on Sydenham’s chorea: Clinical features, treatment and prognostic factors. Eur J Paediatr Neurol. 2022;36:1-6. doi: 10.1016/j.ejpn.2021.11.002. PubMed PMID: 34768201.
- Wang CR, Lee NY, Tsai HW, Yang CC, Lee CH. Acute rheumatic fever in adult patients. Medicine (Baltimore). 2022;101:e29833. doi: 10.1097/MD.0000000000029833. PubMed PMID: 35777053; PubMed Central PMCID: PMCPMC9239616.
- Gurses D, Kocak G, Tutar E, Ozbarlas N, Turkish ARFsg. Incidence and clinical characteristics of acute rheumatic fever in Turkey: Results of a nationwide multicentre study. J Paediatr Child Health. 2021;57:1949-54. doi: 10.1111/jpc.15619. PubMed PMID: 34227703.
- Boyarchuk O, Boytsanyuk S, Hariyan T. Acute rheumatic fever: clinical profile in children in western Ukraine. J Med Life. 2017;10:122-6. PubMed PMID: 28616087; PubMed Central PMCID: PMCPMC5467252.
- Kocevar U, Toplak N, Kosmac B, Kopac L, Vesel S, Krajnc N, et al. Acute rheumatic fever outbreak in southern central European country. Eur J Pediatr. 2017;176:23-9. doi: 10.1007/s00431-016-2801-z. PubMed PMID: 27815733.
- Joshi A, Shrestha RP, Shrestha PS, Dangol S, Shrestha NC, Poudyal P, et al. Sydenham’s Chorea as Presentation of Rheumatic Heart Disease. Kathmandu Univ Med J (KUMJ). 2015;13:271-3. doi: 10.3126/kumj.v13i3.16821. PubMed PMID: 27180377.
- Vasconcelos LPB, Vasconcelos MC, Nunes MDCP, Teixeira AL. Sydenham’s chorea: an update on pathophysiology, clinical features and management. Expert Opinion on Orphan Drugs. 2019;7:501-11. doi: 10.1080/21678707.2019.1684259.
- Rossi M, Wainsztein N, Merello M. Cardiac Involvement in Movement Disorders. Mov Disord Clin Pract. 2021;8:651-68. doi: 10.1002/mdc3.13188. PubMed PMID: 34307738; PubMed Central PMCID: PMCPMC8287161.
- Illan Ramos M, Sagastizabal Cardelus B, Garcia Ron A, Guillen Martin S, Berzosa Sanchez A, Ramos Amador JT. Chorea as the presenting feature of acute rheumatic fever in childhood; case reports from a low-prevalence European setting. BMC Infect Dis. 2021;21:322. doi: 10.1186/s12879-021-06005-x. PubMed PMID: 33827439; PubMed Central PMCID: PMCPMC8025313.
- Guilherme L, Sampaio R, de Barros SF, Köhler K, Spina G, Tarasoutchi F, et al. Rheumatic fever and rheumatic heart disease. The Heart in Rheumatic, Autoimmune and Inflammatory Diseases. Amsterdam: Elsevier; 2017. p. 529-51. doi: 10.1016/B978-0-12-803267-1.00022-3.
- Ekici F, Cetin, II, Cevik BS, Senkon OG, Alpan N, Degerliyurt A, et al. What is the outcome of rheumatic carditis in children with Sydenham’s chorea? Turk J Pediatr. 2012;54:159-67. PubMed PMID: 22734303.
- Favaretto E, Gortani G, Simonini G, Pastore S, Di Mascio A, Cimaz R, et al. Preliminary data on prednisone effectiveness in children with Sydenham chorea. Eur J Pediatr. 2020;179:993-7. doi: 10.1007/s00431-020-03574-y. PubMed PMID: 31965299.
- Dean SL, Singer HS. Treatment of Sydenham’s Chorea: A Review of the Current Evidence. Tremor Other Hyperkinet Mov (N Y). 2017;7:456. doi: 10.7916/D8W95GJ2. PubMed PMID: 28589057; PubMed Central PMCID: PMCPMC5459984.
)