Dilek ÇAVUŞOĞLU, Ayhan PEKTAS

SYDENHAM KORESİ TANISI ALAN OLGULARIN NÖROLOJİK AÇIDAN DEĞERLENDİRİLMESİ

AMAÇ: Bu çalışmada Sydenham koresi tanısı alan çocukların nörolojik açıdan değerlendirilmesi amaçlanmıştır.GEREÇ VE YÖNTEM: Tanı alan hastalar retrospektif olarak taranıp demografik, klinik, laboratuar, nörogörüntüleme, ekokardiyografi (EKO), başvuru yakınmaları ve uygulanan tedaviye yanıtları bakımından değerlendirilmiştir.BULGULAR: Çalışmaya 10 olgu dahil edildi. Hastaların sekizi kız (%80), ikisi erkek (%20), kız/erkek oranı 4, ortalama tanı yaşı 12,05±2,84 yıl olarak saptandı. Başvuru anında olguların hepsinde (%100) kardit tesbit edildi. EKO incelemesinde altı olguda (%60) sadece mitral yetmezlik, dört olguda (%40) ise mitral yetmezlik ve aort yetmezliği mevcuttu. Başvuru yakınmaları açısından değerlendirildiğinde yedi olgu (%70) koreiform hareketler; bir olgu koreiform hareketler ve yürüyüş bozukluğu; bir olgu hemiparezi, dizartri ve koreiform hareketler ve bir olgu da emosyonel instabilite ve yürüyüş bozukluğu nedeniyle başvurdu. Jeneralize kore dört olguda (%40), hemikore altı olguda (%60) izlendi. Olguların dördünde sağ (% 66,6) ve ikisinde sol (%33,3) hemikore bulgusu saptandı. Hastaların tümüne valproat tedavisi (10-15 mg/kg/gün) başlandı. Tedavi ile koreiform hareketlerin kaybolma süresi 20-90 gün (ortalama 42,50±22,39 gün) arasında değişti. Hastaların hiç birinde yan etki gözlenmedi. Yalnızca bir hastanın ANA değeri pozitif olarak saptandı. Olguların sekizinin (%80) beyin manyetik rezonans görüntüleme (MRG) tetkiki normal olup iki olguda (%20) patoloji izlendi. Beyin MRG’de bir olguda periventriküler beyaz cevherde bir olguda ise bilateral frontal lob derin beyaz cevherde gliotik lezyonlar saptandı.SONUÇ: Tanıda nörolojik muayene önemli olmakla beraber hastaların koreiform hareketlerden önce davranışsal semptomlar ile de başvurabileceği akılda tutulmalıdır. Valproatın etkin ve güvenilir bir tedavi seçeneği olduğu ve beyin MRG’de görülen spesifik olmayan hiperintens lezyonların inflamasyon ve vaskulit mekanizmalarının sonucu ortaya çıktığı ileri sürülebilir.

Anahtar Kelimeler:

Sydenham koresi, çocuk, nörolojik sonuç

NEUROLOGICAL EVALUATION OF PATIENTS WITH A DIAGNOSIS OF SYDENHAM CHOREA

OBJECTIVE: In this study, it was aimed to assess the neurological evaluation of children diagnosed with sydenham chorea.MATERIAL AND METHODS: Children diagnosed with Sydenham's chorea were retrospectively screened and evaluated for demographic, clinical, laboratory, neuroimaging, echocardiographic findings (ECHO), presenting complaints, and response to the treatment.RESULTS: Ten patients with Sydenham chorea were included in the study. Eight of the patients were female (80%), two were male (20%), the female / male ratio was 4 and the mean age at diagnosis was 12.05 ± 2.84 years. Carditis was detected in all cases (100%) at the admission. ECHO examination revealed mitral insufficiency in six cases (60%), mitral insufficiency and aortic insufficiency in four cases (40%). When the cases were evaluated in terms of presenting complaints, seven cases (70%) had choreiform movements; one case choreiform movements and gait disturbance; one patient presented with hemiparesis, dysarthria, and choreiform movements and one patient presented with emotional instability and gait disturbance. Generalized chorea was observed in four cases (40%) and hemicorea in six cases (60%). Four (66.6%) of the cases hadright hemicorea and two had left hemicorea (33.3%). Valproate treatment (10-15 mg / kg / day) was administered to all patients. The duration of the disappearance of choreiform movements ranged from 20 to 90 days (mean 42.50 ± 22.39 days). No side effects were observed in any of the patients. Only one patient had a positive ANA. When the neuroimaging was examined, brain magnetic resonance imaging (MRI) was normal in eight cases (80%) and pathology was observed in two cases (20%). Brain MRI revealed glioticlesions in the periventricular white matter in one case and in the bilateral frontal lobe deep white matter in the other case.CONCLUSIONS: Although the neurological examination is important in the diagnosis of Sydenham's chorea, it should be kept in mind that patients may present with behavioral symptoms before choreiform movements. It can be suggested that valproate is an effective and safe treatment option and non-specific hyperintense lesions seen on brain MRI are the result of inflammation and vasculitis mechanisms.

Keywords:

Sydenham Chorea, Child, Neurological Outcome,

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1. Swedo SE, Leonard HL, Garvey M, et al. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases. Am J Psychiatry 1998; 155:264-71.
2. Bonthius DJ, Karacay B. Sydenham’s chorea: Not göne and not forgotten. Semin Pediatr Neurol 2003;10:11-9.
3. Faustino PC, Terreri MT, da Rocha AJ, et al. Clinical laboratory, psychiatric and magnetic resonance findings in patients with Sydenham chorea. Neuroradiology 2003;45:456-62.
4. Terreri MT, Roja SC, Len CA, et al. Sydenham’s chorea: clinical and evolutive characteristics. Sao Paulo Med J 2002;120:16-9.
5. Weiner SG, Normandin PA. Sydenham chorea: a case report and review of the literature. Pediatr Emerg Care 2007;23:20-4.
6. Sydenham T. On St Vitus dance. The works of Thomas Sydenham, MD. Translated from the Latin edition of Dr Greenhill with a life of the author by RG Latham MD. London: The Sydenham Society 1850;2: 257–9.
7. Demiroren K, Yavuz H, Cam L, et al. Sydenham's chorea: a clinical follow-up of 65 patients. J Child Neurol. 2007;22:550-4.
8. Kılıç A, Ünüvar E, Tatlı B, et al. Neurologic and cardiac findings in children with sydenham chorea. Pediatr Neurol 2007;36:159‐64.
9. Davutoğlu V, Kılınç M, Dinçkal H, et al. Sydenham’s chorea, clinic characteristics of nine patients. Int J Cardiol 2004;96:483‐4.
10. Kulkarni ML, Anees S. Sydenham’s chorea. Indian Pediatr 1996;33:112‐5.
11. Genel F, Arslanoğlu S, Uran N, et al. Sydenham’s chorea: Clinical findings and comparison of the efficacies of sodium valproate and carbamazepine regimens. Brain Dev 2002;24:73‐6.
12. Tumas V, Caldas CT, Santos AC, et al. Sydenham’s chorea: Clinical observations from a Brazilian movement disorder clinic. Parkinsonism Relat Disord 2007; 13:276‐83.
13. Kato M, Araki S. Paroxysmal kinesignenic choreoathetosis. Report of a case relieved by carbamazepine. Arch Neurol 1969;20:508‐13.
14. Shannon KM, Fenichel GM. Pimozide treatment of Sydenham’s chorea. Neurology 1990; 40:186‐7.
15. Hawkes CH, Nourse CH. Tetrabenazine in Sydenham chorea. British Med J 1977;1: 1391‐2.
16. Oulis P, Karapoulıos E, Masdrakıs VG, et al. Levetiracetam in the treatment of antipsychotics‐resistant Tourette Syndrome. World J Biol Psychiatry 2008;9:76‐7.
17. Green LN. Corticosteroids in the treatment of Sydenham’s chorea. Arch Neurol 1978;35:53‐4.
18. Pena J, Mora E, Cardozo J, et al. Comparison of the efficiency of carbamazepine, haloperidol and valproic acid in the treatment of children with Sydenham’s chorea: clinical followup of 18 patients. Arq Neuropsiquiatr 2002; 60:374-7.
19. Swedo SE, Leonard HL, Schapiro MB, et al. Sydenham’s chorea: physical and psychological symptoms of St Vitus dance. Pediatrics 1993;91:706–13.
20. Swedo SE. Sydenham’s chorea. A model for childhood autoimmune neuropsychiatric disorders. JAMA 1994;272:1788–91.
21. Lessof MH, Bywaters EGL. The duration of chorea. BMJ 1956;1:1520–3.
22. Elibol B. Pathophysiology of pediatric movement disorders. Sydenham koresi. Turkiye Klinikleri J Pediatr Sci 2006; 2: 10‐1.
23. Abraham S, Gorman MO, Shulman ST. Anti-nuclear antibodies in Sydenham’s chorea. Adv Exp Med Biol 1997;418:153–6.
24. Oosterveer DM, Overweg-Plandsoen WC, Roos RA. Sydenham's chorea: a practical overview of the current literature. Pediatr Neurol 2010;43:1-6.
25. Ekici A, Yakut A, Yimenicioglu S, et al. Clinical and Neuroimaging Findings of Sydenham's Chorea. Iran J Pediatr 2014;24:300-6