Acute Diseminated Encephalomyelitis: A Case Report and Review of Literature

Amaç: Akut disemine ensefalomiyelit (ADEM) otoimmün, monofazik, vaskülitik ve inflamatuvar olmayan yaygın demiyelinizasyon ile seyreden, özellikle de beyin ve spinal kordu etkileyen bir santral sinir sistemi hastalığıdır. ADEM çocukluk çağının nadir görülen bilinç bozukluğu nedenlerinden biridir. 20 yaştan önce hastalığın görülme sıklığı 1.53/100000'dir ve sıklıkla 5-8 yaş arasında görülür. Altta yatan neden tam olarak bilinmese de enfeksiyonlar ve aşılamalar hastalığın ortaya çıkmasında önemli faktörlerdir. Multifokal beyaz cevher lezyonları manyetik rezonans görüntülemede (MR) hastalığın karakteristik özelliğidir. Bizim hastamız da bilinç bulanıklığı ve nöbet sonrası yapılan beyin MR'da serebrumda beyaz cevherde hiperintens lezyonlar saptandı. Hastanın beyin omurilik sıvı incelemesinde hastalığa ait bir özellik saptanmadı. Bu yazıda yüksek doz steroid tedavisi sonrası tam düzelme gösteren, viral üst solunum yolu enfeksiyonu sonrası ADEM gelişen 3 yaşındaki erkek hasta sunulmuştur.

Akut Disemine Ensefalomiyelit; Olgu Sunumu Ve Literatür İncelemesi

Acute disseminated encephalomyelitis (ADEM) is an autoimmune and monophasic central nervous system disease that principally affects brain and spinal cord by causing non-vasculitic inflammatory diffuse demyelination. ADEM is one of the rare causes of impaired consciousness in childhood. Incidence of the disease under the age of 20 is 1.5-3 / 100000 per year and it mostly occurs in children between the ages of 5-8 years. Underlying causes aren't defined clearly yet, however, infections and vaccinations are known as predisposing factors for the disease. Multifocal white matter lesions on magnetic resonance imaging (MRI) are characteristic signs of ADEM. In our case, hyperintense lesions in cerebrum were detected on cranial MRI subsequent to impaired consciousness and convulsion. No specific feature was found in cerebrospinal fluid analysis of the patient. In this paper, we present a three-year-old boy who developed ADEM subsequent to viral upper respiratory tract infection and had full recovery after high dose steroid therapy.

___

  • Incecik F, Hergüner MO, and Altunbaşak S. Acute disseminated encephalomyelitis: an evaluation of 15 cases in childhood. Turk J Pediatr 2013;55(3):253-9.
  • Elhassanien, AF and Aziz HA. Acute demyelinating encephalomyelitis: Clinical characteristics and outcome. J Pediatr Neurosci 2013;8(1):26-30.
  • Absoud, M, Lim MJ, Chong WK, De Goede CG, Foster K, Gunny R, et al. Paediatric acquired demyelinating syndromes: incidence, clinical and magnetic resonance imaging features. Mult Scler 2013;19(1):76-86.
  • Ketelslegers IA, Catsman-Berrevoets CE, Neuteboom RF, Boon M, van Dijk KG, Eikelenboom MJ, et al. Incidence of acquired demyelinating syndromes of the CNS in Dutch children: a nationwide study. J Neurol 2012;259(9):1929-35.
  • Ichikawa K, Motoi H, Oyama Y, Watanabe Y, and Takeshita S. Fulminant form of acute disseminated encephalomyelitis in a child treated with mild hypothermia. Pediatr Int 2013;55(6):149-51.
  • Suppiej A, Vittorini R, Fontanin M, De Grandis D, Manara R, Atzori M, et al. Acute disseminated encephalomyelitis in children: focus on relapsing patients. Pediatr Neurol 2008;39(1):12-7.
  • Huhn K, Lee DH, Linker RA, Kloska S, and Huttner HB. Pneumococcal-meningitis associated acute disseminated encephalomyelitis (ADEM) - case report of effective early immunotherapy. Springerplus 2014;3:415.
  • Elias MD, Narula S, and Chu AS, Acute disseminated encephalomyelitis following meningoencephalitis: case report and literature review. Pediatr Emerg Care 2014;30(4):254-6.
  • Orhan A, Aygül R, Deniz O, Koçak N, and Ulvi H. Multiple Sklerozis' li Olgularda BAEP ve MRI Bulgularının Karşılaştırılması. Journal of Inonu University Medical Faculty 2010;13(1):13-6.
  • Langer-Gould A, Zhang JL, Chung J, Yeung Y, Waubant E, and Yao J. Incidence of acquired CNS demyelinating syndromes in a multiethnic cohort of children. Neurology 2011;77(12):1143-8.
  • Rust RS. Multiple sclerosis, acute disseminated encephalomyelitis, and related conditions. Semin Pediatr Neurol, 2000;7(2):66-90.
  • Krupp LB, Banwell B, Tenembaum S. International Pediatric, Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology 2007;68(16 Suppl 2):7-12.
  • Miller DH, Weinshenker BG, Filippi M, Banwell BL, Cohen JA, Freedman MS, et al. Differential diagnosis of suspected multiple sclerosis: a consensus approach. Mult Scler 2008;14(9):1157-74.
Turgut Özal Tıp Merkezi Dergisi-Cover
  • ISSN: 1300-1744
  • Başlangıç: 1994
  • Yayıncı: -
Sayıdaki Diğer Makaleler

Evaluation of İntra-Articular Hip Pathology: Comparison of CT Arthrography And MR Arthrography

Zeynep MARAŞ ÖZDEMİR, Ayşegül SAĞIR KAHRAMAN, Ayla ÇİMEN ÖZAYDOĞDU, İsmail Okan YILDIRIM, Cemile AYŞE GÖRMELİ, Gökay GÖRMELİ

Spontaneous Ruptured Parasellar Dermoid Tumor: CT and MRI Findings

Alper DİLLİ, Hale TURNAOĞLU, Tülin OĞUZKAN MERCİMEK, Ahmet Muhteşem AĞILDERE

Efficiency of Alvarado Score in Diagnosis of Acute Appendicitis

Ali UZUNKÖY, Uğur EKİCİ, Murat KANLIÖZ, Yusuf YÜCEL, Orhan GÖZENELİ, Abuzer DİRİCAN, Faik TATLI

Evaluating Anxiety and Stress Coping Skills and Related Variables of Mothers with Mentally Retarded Children

Mehmet KARATAŞ, Engin Burak SELCUK, Özlem ÖZCAN ÖZEL, Şenay ZIRHLI ŞELÇUK, Bora TETİK, Duygu KAYHAN

Our Initial Experiences and Outcomes of Pediatric Kidney Transplantations

Turgut PİSKİN, Yilmaz TABEL, Bulent UNAL

Side-based Activation of Sympathetic Skin Responses Recorded from the Frontal Region in Idiopathic Parkinson's Disease

Nebil YILDIZ, Serpil YILDIZ, Şule AYDIN TURKOGLU, Elif Sultan BOLAÇ

A Case of Sudden Death due to Lighter Refill Gas Inhalation

Mustafa DOĞAN, Semih PETEKKAYA, Nusret AYAZ, Mucahit ORUÇ, Adalet Eda BUDAK, Cihan GÖKTÜRK, Bedirhan Sezer ÖNER, Özcan SOYLU, Ahmet ÇELEBİ, Osman CELBİŞ

Remission of Obsessive-Compulsive Symptoms in Hypomanic Period in a Patient with Comorbid Bipolar Affective Disorder and Obsessive Compulsive Disorder: A Case Report

Şükrü KARTALCI, Serhat ŞAHİN, Gülşen ÖZTAŞ, Lale GÖNENİR ERBAY

A Case with Idiopathic Bilateral Multifocal Retinal Pigment Epithelium Detachment

Mehmet Ragıp EKMEN

Decompressive Surgery for Malignant Middle Cerebral Artery Infarctions

Suat KAMIŞLI, Mehmet TECELLİOĞLU