Sydenham’s Chorea with a Pediatric Neurologist’s Point of View

Öz

Objective: The Sydenham’s chorea, which is an autoimmune and hyperkinetic movement disorder seen following pharyngitis caused by group A streptococci, is the neurological manifestation of acute rheumatoid fever. This study aims to present the demographical and clinical features in addition to patient management and outcomes in patients diagnosed with Sydenham’s chorea. Material and Methods: Thirty-three children and adolescents who were admitted to the Ankara Education and Research Hospital pediatric neurology clinic between September 2016 and September 2019 and were diagnosed with Sydenham’s chorea were included in the study. Demographic, clinical and laboratory data, treatments and responses to treatments along with outcomes were evaluated in a retrospective and cross sectional manner. Results: The average age of the patients was 119,6±29,2 months and 24,2% were male. In 30 (90,9%) of the male patients, valvulitis as reported by echocardiography accompanied Sydenham’s chorea. Hemichorea was present in 11 (30%) patients while chorea was more prominent in the extremities in 12 patients, in the trunk in 8 patients and in the face in 1 patient. Antistreptolysin-O titers were elevated in 28 (84%) patients. Twenty-seven (81,8%) patients were given haloperidol, 3 (9%) patients were given carbamazepine and 2 (%6) patients were given sodium valproate for an average of 6,8±1,1 months. Clinical remission was achieved in 6,9±1,2 months. The only adverse effect observed was parkinsonism caused by haloperidol prescribed for acute symptomatic treatment. The patients were followed up for 9,6±4,2 months after the symptoms had subsided and no recurrence was observed. Discussion: Sydenham’s chorea is one of the major criteria for acute rheumatoid fever which classically limits itself but may cause loss of function and adversely effect the quality of life. Although its diagnosis algorithm and differential diagnoses are clear, a consensus can not be reached due to conflicting data on treatment

Anahtar Kelimeler

• Chorea
• Movement disorder
• Sydenham’s chorea

Çocuk Nörolojisi Bakışı ile Syndenham Koresi

Öz

Amaç: A grubu beta hemolitik streptokokkal farenjit sonrası gelişen otoimmun, hiperkinetik bir hareket bozukluğu olan Syndenham koresi, akut romatizmal ateşin nörolojik manifestasyonudur. Bu çalışmada Syndenham koresi tanısı alan hastaların demografik ve klinik özellikleri yanısıra hasta yönetimi ve sonuçlarının literatüre sunulması amaçlanmıştır. Metod: Ankara Eğitim ve Araştırma Hastanesi, Çocuk Nörolojisi Polikliniğinde Eylül 2016 - Eylül 2019 tarihleri arasında başvuran Syndenham koresi tanısı alarak izlenen 33 çocuk ve adolesan hasta çalışmaya alınmıştır. Hastaların demografik, klinik ve laboratuvar özelliklerine ait bilgiler, verilen tedaviler ve tedavi yanıtları ile hastaların prognozları retrospektif ve kesitsel olarak değerlendirilmiştir. Sonuçlar: Yaş ortalaması 119.6 ± 29.2 ay olan %24.2’si erkek hastaların 30’unda (%90.9) ekokardiyografik valvulit syndenham koresine eşlik ediyordu. Hemikore 11 hastada (%30) izlenirken, 12 hastada ekstremitelerde, 8 hastada gövdede, 1 hastada yüzde belirgin kore izlendi. Anti-streptolizin-O yüksekliği 28 hastada (%84) saptandı. Ortalama 6.8 ± 1.1 ay süre ile Yirmi yedi hastaya (%81.8) haloperidol, 3 hastaya karbamazepin (%9), 2 hastaya sodyum valproat (%6) tedavisi verildi. Klinik remisyon ortalama 6.9 ± 1.2 ayda sağlandı. Akut semptomatik tedavi için verilen haloperidole bağlı gelişen parkinsonizm izlenen tek yan etki olarak kaydedildi. Semptomlar gerileyip tedavi sonlandırıldıktan sonra ortalama 9.6 ± 4.2 ay izlenen hastalarda rekürrens görülmedi. Tartışma: Syndenham koresi, klasik olarak kendi kendini sınırladığı bilinen ancak işlevselliği bozan, hayat kalitesini negatif yönde etkileyen akut romatizmal ateş majör kriterlerinden birisidir. Tanı ve ayırıcı tanı algoritmasında netleşen yönetime rağmen tedavi açısından çelişkili ve tartışmalı bilgiler halen bir konsensüs sağlanmasına engel olmaktadır.

Anahtar Kelimeler

• Kore
• Hareket bozukluğu
• Syndenham koresi

Kaynakça

1. 1- 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;131:1806–18. 2- Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet. 2005; 366:155-68. 3- 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;37:2076-87. 4- Kilic A, Unuvar E, Tatli B, et al. Neurologic and cardiac findings in children with Sydenham chorea. Pediatr Neurol. 2007;36:159–64. 5- Dean SL, Singer HS. Treatment of Sydenham’s Chorea: A Review of the Current Evidence. Tremor Other Hyperkinet Mov (N Y). 2017;7:456. 6- Gurkas E, Karalok ZS, Taskin BD, et al. Predictors of recurrence in Sydenham’s chorea: Clinical observation from a single center. Brain Dev. 2016;38:827–34. 7- Walker AR, Tani LY, Thompson JA, et al. Rheumatic chorea: relationship to systemic manifestations and response to corticosteroids. J Pediatr. 2007;151:679–83 8- Korn-Lubetzki I, Brand A, Steiner I. Recurrence of Sydenham chorea: implications for pathogenesis. Arch Neurol. 2004;61:1261–4. 9- Cardoso F, Vargas AP, Oliveira LD, et al. Persistent Sydenham’s chorea. Mov Disord. 1999;14:805– 7. 10- 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;60:374–7. 11- Bashir H, Jankovic J. Treatment options for chorea. Expert Rev. Neurother. 2018; 18(1):51-63 12- Daoud, AS; Zaki, M; Shakir, R; al-Saleh Q. Effectiveness of sodium valproate in the treatment of Sydenham’s chorea. Neurology. 1990;40:1140–1. 13- Harel L, Zecharia A, Straussberg R, et al. Successful treatment of rheumatic chorea with carbamazepine. Pediatr. Neurol. 2000;23:147–51. 14- Demiroren, K; Yavuz, H; Cam, L; Oran, B; Karaaslan SDS. Sydenham’s chorea: a clinical follow-up of 65 patients. J Child Neurol. 2007;22:550–4. 15- 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;74:1350-1. 16- Cardoso F. Autoimmune choreas. J Neurol Neurosurg Psychiatry. 2017;88:412–7. 17- Swedo SE, Rapoport JL, Cheslow DL, et al. High prevalence of obsessive-compulsive symptoms in patients with Sydenham’s chorea. Am J Psychiatry. 1989;146:246–9. 18- Asbahr FR, Garvey MA, Snider LA, et al. Obsessive-compulsive symptoms among patients with Sydenham chorea. Biol Psychiatry. 2005;57:1073–6. 19- 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;65:994-9 20- Teixeira Jr. AL, Meira FC, Maia DP, et al. Migraine headache in patients with Sydenham’s chorea. Cephalalgia. 2005;25:542–4.