Nekrotizan Ensefalopati: Bir olgu sunumu

Akut Nekrotizan Ensefalit, akut ateşli hastalıkların sıklıkla da viral enfeksiyonların tetiklediği nadir görülen bir ensefalopati formudur. Bu sendrom özellikle talamus, beyin sapı, beyincik ve beyaz maddeyi içeren multifokal simetrik beyin lezyonların varlığı ile karakterizedir. En yaygın anormallikler artmış serum aminotransferaz aktivitesi ve artmış beyin omurilik sıvısı proteini düzeyidir. Hastalığın etyolojisi ve patogenezisi tam olarak bilinememektedir ve spesifik bir tedavisi ve korunma yöntemi yoktur. Prognoz genellikle kötüdür ve hastaların %10'undan azı tamamen iyileşir. Tanı genellikle karakteristik nörögörüntüleme bulguları ile konulur. Biz İnfluenza A enfeksiyonun neden olduğu ve Akut Nekrotizan Ensefalitin tipik klinik ve radyolojik özelliklerini taşıyan 8 yaşındaki olguyu sunarak, influenza enfeksiyonlarının bu nadir ve ciddi formuna dikkat çekmek istedik. Anahtar kelimeler: H1N1; Akut Ensefalopati; Çocuk.

Acute Necrotizing Encephalopathy of Childhood Associated With A Novel Influenza Type A Virus: A Case Report

Acute necrotizing encephalopathy of childhood is a rare, clinically distinct entity of acute encephalopathy triggered by acute febrile diseases, mostly viral infections. This syndrome is characterized by the presence of multifocal symmetrical brain lesions involving mainly thalami, brainstem, cerebellum and white matter. The most common abnormalities are an increased level of serum aminotransferase activity and cerebrospinal fluid protein. The etiology and pathogenesis remain unknown and there is no specific therapy or prevention. The prognosis is usually poor and less than 10% of patients recover completely. Diagnosis is made mainly by the characteristic findings of neuroimaging. We describe a case of a young child who was infected with a novel influenza A virus and displayed the characteristic clinical features and neuroimaging findings of acute necrotizing encephalopathy. Influenza is generally considered as a benign illness, but phsysicians should be aware of this unusual presentation of influenza infection. Key words: H1N1; Acute Encephalopathy; Child.

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  • Mizuguchi M (1997) Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain Dev 1997;19:81-92.
  • Voudris KA, Skaardoutsou A, Haronitis I, et al. Brain MRI findings in influenza A-associated acute necrotizing encephalopathy of childhood. Eur J Paediatr Neurol 2001;5:199-202.
  • Okumura A, Mizuguchi M, Kidokoro H, et al. Outcome of acute necrotizing encephalopathy in relation to treatment with corticosteroids and gammaglobulin. Brain Dev 2009;31:221-7.
  • Yoshikawa H, Watanabe T, Abe T, Oda Y. Clinical diversity in acute necrotizing encephalopathy. J Child Neurol 1999;14:249-55.
  • Weitkamp JH, Spring MD, Brogan T, Moses H, Bloch KC, Wright PF. Influenza A virus-associated acute necrotizing encephalopathy in the United States. Pediatr Infect Dis J 2004;23:259-63.
  • Seo HE, Hwang SK, Choe BH, Cho MH, Park SP, Kwon S. Clinical spectrum and prognostic factors of acute necrotizing encephalopathy in children. J Korean Med Sci 2010;25:449-53.
  • Tabarki B, Thabet F, Al Shafi S, Al Adwani N, Chehab M, Al Shahwan S. Acute necrotizing encephalopathy associated with enterovirus infection. Brain Dev 2012;23.
  • T. Ichiyama, H. Isumi, H. Ozawa, T. Matsubara, T. Morishima, S. Furukawa. Cerebrospinal fluid and serum levels of cytokines and soluble tumor necrosis factor receptor in influenza virus-associated encephalopathy. Scand J Infect Dis 2003;35: 59-61.
  • Okumura A, Mizuguchi M, Kidokoro H, Tanaka M, Abe S, Hosoya M, et al. Outcome of acute necrotizing encephalopathy in relation to treatment with corticosteroids and gammaglobulin. Brain Dev 2009;31:221-7.
  • Kansagra SM, Gallentine WB. Cytokine storm of acute necrotizing encephalopathy. Pediatr Neurol 2011;45:400-2.
Turgut Özal Tıp Merkezi Dergisi-Cover
  • ISSN: 1300-1744
  • Başlangıç: 1994
  • Yayıncı: -