Çocukluk çağında akut dissemine ensefalomiyelit: Tek merkez deneyimi

Amaç: Çocuklarda akut dissemine ensefalomyelitin (ADEM) epidemiyolojik, klinik, nöro-görüntüleme ve laboratuvar bulguları ile tedavi ve sonuçlarının araştırılması amaçlandı. Yöntemler: Selçuk Üniversitesi Pediatrik Nöroloji Bilim Dalında Ocak 2004-Ocak 2010 aralığındaki altı yıllık zaman diliminde ADEM tanısı ile takip edilen on dört hastanın öyküleri, klinik özellikleri, laboratuar ve radyolojik bulguları kaydedildi. Bulgular: Olgularımızın onu erkek, dördü kız idi. On hastanın başvuru mevsimi kış ya da ilkbahar ayı idi. Hastaların büyük kısmında (%64,2) geçirilmiş akut ateşli hastalık öyküsü vardı. En sık görülen başvuru semptomları yürüme bozukluğu (%71,4) ve bilinç durumunda değişiklik (%57) idi. Detaylı laboratuvar incelemelerine rağmen sadece dört hastada mikrobiyolojik ajan gösterilebildi. Kranial magnetik rezonans görüntülemede (MRG) kortikal ve subkortikal beyaz cevher tutulumu özellikle frontal (%57) ve temporal (%42) tutulum saptandı. On hasta kortikosteroid, 4 hasta ise intravenöz immunglobülin tedavisi aldı. Ebstein-Barr virüs ve mikoplazma serolojisi pozitif olan hastalara gansiklovir ve klaritromisin tedavisi verildi. Onüç hastada tedavi ile nörolojik şikayet ve bulgular tamamen kayboldu. Sonuç: Bu seride çocuklarda ADEMin esas olarak kış ve ilkbahar aylarında ve bir üst solunum yolu enfeksiyonunu takiben ortaya çıktığı bulundu.

Acute disseminated encephalomyelitis in childhood: Single center experience

Objective: To describe the epidemiologic, clinical, neuroimaging, and laboratory features; treatment; and outcome in a cohort of children with acute disseminated encephalomyelitis (ADEM). Methods: Clinical features, laboratory and radiological findings of 14 patients with the diagnosis of ADEM were recorded in 2004-2010 in the range of 6-year follow-up time period in Pediatric Neurology Department of Selçuk University. Results: Ten patients were boys and four were girls. Ten patients presented either in winter or in spring. The majority of patients (64.2%) had as history of acute febrile illness. Patients presented most often with gait disturbances (71.4%) and secondly with altered consciousness (57%). Despite rigorous microbiologic testing, a definite microbiologic diagnosis was established only in four patients. Brain magnetic resonance imaging (MRI) identified lesions in the cortex and, or in subcortical white matter, especially in the frontal (57%) and temporal (42%) regions. Ten patients were treated with corticosteroids, and four were treated with intravenous immunoglobulin. Ganciclovir and clarithromycin were administered in patients with Epstein-Barr virus and mycoplasma according to the serology. In 13 patients, all neurologic signs and symptoms resolved after treatment. Conclusion: The present series demonstrates that acute disseminated encephalomyelitis in children occurs predominantly in winter and spring and often follows an upper respiratory tract illness.

PDF

___

1. Tardieu M, Mikaeloff Y. What is acute disseminated encephalomyelitis (ADEM)? Eur J Paediatr Neurol 2004; 8: 239-242.
2. Leake AD, Albani S, Kao SA. Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr Infect Dis J 2004; 23: 756-764.
3. Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ. Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurol 2001; 56:1308-1312.
4. Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain 2000;123:2407-2422.
5. Murtthy SN, Faden HS, Cohen ME, Bakshi R. Acute disseminated encephalomyelitis in children. Pediatrics 2002;110(2 Pt 1):e21.
6. Hung K-L, Liao H-T, Tsai M-L. Postinfectious encephalomyelitis: etiologic and diagnostic trends. J Child Neurol 2000;15:666-670.
7. Stonehouse M, Gupte G, Wassmer E, Whitehouse WP. Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians. Arch Dis Child 2003;88:122-124.
8. Tenembaum S, Chitnis T, Ness J, Hahn JS. International Pediatric MS Study Group. Acute disseminated encephalomyelitis. Neurology 2007;68:23-36.
9. Brass SD, Caramanos Z, Santos C, Dilenge ME, Lapierre Y, Rosenblatt B. Multiple sclerosis vs acute disseminated encephalomyelitis in childhood. Pediatr Neurol 2003;29:227-231.
10. Kabakuş N, Kurt ANÇ. Akut dissemine ensefalomyelit: Klinik seri. Fırat Tıp Dergisi 2005;10:127-131.
11. Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology 2002; 59: 1224-1231.
12. Erol I, Ozkale Y, Alkan O, Alehan F. Acute Disseminated encephalomyelitis in children and adolescents: A single center experience. Pediatr Neurol. 2013 Jul 9. doi:pii: S0887- 8994(13)00216-6. 10.1016/j.pediatrneurol.2013.03.021. [Epub ahead of print]
13. Rossi A. Imaging of acute disseminated encephalomyelitis. Neuroimaging Clin N Am 2008;18:149-161.
14. Caldemeyer KS, Smith RR, Harris TM, Edwards MK. MRI in acute disseminated encephalomyelitis. Neuroradiology 1994;36:216-220.
15. Kimura S, Nezu A, Ohtsuki N, Kobayashi T, Osaka H, Uehara S. Serial magnetic resonance imaging in children with postinfectious encephalitis. Brain Dev 1996;18:461-5.
16. Pohl D, Waubant E, Banwell B, et al. Treatment of pediatric multiple sclerosis and variants. Neurology 2007;68:54-65.
17. Alper G, Schor NF. Toward the definition of acute disseminated encephalitis of childhood. Curr Opin Pediatr 2004;16:637-640.