Acute Necrotizing Encephalopathy of Childhood: A Turkish Case

Çocukluk çağının akut nekrotizan ensefalopatisi, talamusta tipik, simetrik lezyonlar ve ak madde, beyin sapı ve serebellumun değişken tutulumu ile nitelenen, etiyolojisi bilinmeyen, nadir bir akut ensefalittir. Klinikte, gastrointestinal veya solunum sistemini etkileyen nonspesifik viral enfeksiyonlar sonrasında hızlı nörolojik bozulma izlenir. Asya kökenli çocuklarda daha sık görülür. Üç yaşında bir erkek olgu bilinç bulanıklığı, ve ateş nedeniyle hastanemize başvurdu. Nazofarenks sürüntüsünde polimeraz zincir reaksiyonu ile insan parainfluenza virüs tip 2 saptandı. Beyin manyetik rezonans incelemesinde bilateral talamusta simetrik yayılan hiperintensiteler saptanan hastaya akut nekrotizan ensefalopati tanısı kondu.

Çocukluk Çağının Akut Nekrotizan Ensefalopatisi: Türkiye'den Bir Olgu

Acute necrotizing encephalopathy of childhood is a rare form of acute encephalopathy of unknown etiology characterized by typical symmetrical lesions in the thalami, with variable involvement of the white matter, brainstem and cerebellum. Clinically there is a rapid neurologic deterioration after a short period of a nonspecific viral-like illness associated with gastrointestinal or respiratory signs. Asian children are especially affected. Here we present a 3-yearold boy admitted to our hospital with fever and deterioration of consciousness. His nasopharyngeal swab for polymerase chain reaction revealed positive results for human parainfluenza 2 virüs. The diagnosis of acute necrotizing encephalopathy was made by cranial magnetic resonance imaging findings with symmetrical expansile hyperintense signals in bilateral thalami.

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  • Mizuguchi M, Abe J, Mikkaichi K, Noma S, Yoshida K, Yamanaka T, Kamoshita S. Acute necrotising encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. Journal of Neurology, Neurosurgery, and Psychiatry. 1995;58:555-61.
  • San Milan B, Teijeira S, Penin C, Garcia JL, Navarro C. Acute necrotizing encephalopathy of childhood: report of a Spanish case. Pediatr Neurol. 2007;37:438-41. Klinikleri J Pediatr. 2012;21:40-4.
  • Skelton BW, Hollingshead MC, Sledd AT, Phillips D, Castillo M. Acute necrotizing encephalopathy of childhood: typical findings in an atypical disease. Pediatr Radiol. 2008;38:810-13.
  • Albayram S, Bilgi Z, Selcuk H, et al. Diffusion- weighted MR imaging findings of acute necrotizing encephalopathy. AJNR. 2004;25:792-7.
  • Mastroyianni SD, Voudris KA, Katsarou E et al. Acute necrotizing encephalopathy associated with parainfluenza virus in a caucasian child: J Child Neurol. 2003;18:570-2.
  • Ohsaka M, Houkin K, Takigami M, Koyanagi I. Acute necrotizing encephalopathy associated with human herpesvirus-6 infection. Pediatr Neurol. 2006;34:160- 3.
  • Mastroyianni SD, Gionnis D, Voudris K, Skardoutsou A, Mizuguchi M. Acute necrotizing encephalopathy of childhood in non-Asian patients: report of three cases and literature review. J Child Neurol. 2006;21:872-79.
  • Marco EJ, Anderson JE, Neilson DE, Jonathan B, Strober JB. Acute necrotizing encephalopathy in 3 brothers. Pediatrics. 2010;125:693-98.
  • Mizuguchi M. Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain & Development. 1997;19:81-92.
  • Munakata M, Kato R, Yokoyama H, et al. Combined therapy with hypotermia and anticytokine agents in influenza A encephalopathy. Brain Dev. 2000;22:373- 7.
  • Martin A, Reade EP. Acute necrotizing encephalopathy progressing to brain death in a pediatric patient with novel influenza A (H1N1) infection. Clinical Infectious Diseases. 2010;50:50-2.
  • Kömür M, Okuyaz Ç, Arslankoylu AE, Kara E, Atici A. Acute necrotizing encephalopathy associated with novel influenza A (H1N1) virus in Turkey. J Pak Med Assoc. 2011;61:1237-9.
  • Wong A, Simone E, Zimmerman R et al. Acute necrotizing encephalopathy of childhood: correlation of MR findings and clinical outcome. AJNR Am J Neuroradiol. 2006;27:1919-23.
  • Sugaya N. Influenza-associated encephalopathy in Japan. Semin Pediatr Infect Dis. 2002;13:79-84.
  • Vilensky JA, Foley P, Gilman S. Children and encephalitis lethargica: a historical review. Pediatr Neurol. 2007;37:79-84.
  • Maladrini A, Luchini D, Gambelli S, et al. A syndrome of bilateral hemorrhage of the thalamus and myocarditis with fatal course. J Clin Forensic Med. 2004;11:208-10.
  • Voudris KA, Skardoutsou 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.
  • Tran TD, Kubota M, Takeshita K, et al: Varicella- associated acute necrotizing encephalopathy with a good prognosis. Brain Dev. 2001;23:54-7.
  • Olgar S, Ertugrul T, Nisli K, Aydin K, Caliskan M. Influenza A associated encephalopathy. Neuropediatrics. 2006;37:166-8. acute necrotizing
  • Morishima T. Treatment of influenza associated encephalopathy [in Japanese]. Nippon Rinsho. 2003;61:2006-12.