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Yıl:2014; Cilt: 51; Sayı: 1>> Özet
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Nöropsikiyatri Arşivi; 2014;51(1):74-78
Çocukluk Çağında İnflamatuar Demiyelinizan Hastalıklar: Olgu Sunumu ve Literatürün Gözden Geçirilmesi
E Ekizoğlu, PT Topaloğlu, Z Yapıcı, M Eraksoy
İstanbul Üniversitesi, İstanbul
Multipl skleroz (MS) ve akut dissemine ensefalomiyelitin (ADEM) benzer patofizyolojik özellikleri olduğu düşünülmesine karşın, klinik seyirlerindeki ve tedavi yaklaşımlarındaki farklılıklar nedeniyle, izleme programının ve tedavisinin planlanması açısından birbirinden ayırt edilmesi önem taşımaktadır. MS ve ADEM’de klinik bulgularda, radyolojik görüntüleme ve beyin omurilik sıvısı bulgularında ortak ve ayrılan özellikler görülür. ADEM sıklıkla çocukluk çağının monofazik bir enflamatuar demiyelinizan hastalığı olarak kabul edilmektedir. Erişkin dönemde daha sık görülen MS ise çocukluk çağında da gelişebilmektedir. Bu yazıda, 3 aylık bir süre içinde önce sol, sonrasında sağ hemiparezi, serebellar bulgular, medulla spinalis tutulumu bulguları ve ardından iki yanlı kortikal tutuluma bağlı görme kusuru gelişen 14 yaşında kız çocuğu sunulmakta ve ilgili literatür eşliğinde klinik, diğer laboratuar bulguları ve tedavi yaklaşımları tartışılmaktadır. Olgumuzun klinik ve seyir özellikleri, bu yaş grubunda gelişen MS ve ADEM’in kliniği, MR ve beyin omurilik sıvısı (BOS) bulguları göz önüne alınarak yineleyici tümefaktif MS tanısına varılmıştır. Steroid tedavisine belirgin yanıt alınmamasına karşın hasta, plazmaferezden belirgin olarak yarar görmüştür. Klinik düzelmiş, radyolojik bulgular gerilemiştir. Hasta, koruyucu tedavi altında belirli aralıklarla izleme alınmış, yeni tekrarlayan atağı olmamış, aktif yaşamını sürdürmektedir.
Inflammatory Demyelinating Diseases of Childhood: Case Report and Literature Review
Multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM) are demyelinating inflammatory diseases, considered to have a striking pathophysiological resemblance. However, due to the differences in both clinical course and clinical approaches, it is important to differentiate between the two conditions, to plan further investigations and therapy protocols. These diseases have similar but also distinct clinical, radiological and cerebrospinal fluid (CSF) findings. ADEM is typically a monophasic disease of children. MS occurs generally in adult age, but uncommonly may develop in childhood with variable features. Our case is a 14 year-old-girl, presented with a three-month history of left hemiparesis, followed by right hemiparesis, cerebellar signs, myelitis and cortical visual disturbances. Based on the clinical follow-up, MR and CSF findings, our patient was diagnosed with relapsing tumefactive multiple sclerosis. Steroid treatment was not significantly effective, however the patient has benefited from plasmapheresis clinically and radiologically. Our patient is still being followed under the disease modifying therapy without any relapse.

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