• Erhan Aksoy, Ülkü Öztoprak, Halil Çelik, Fatih Mehmet Akif Özdemir, Mehbare Özkan, Hülya Kayılıoğlu, Ayşegül Danış, Özge Kucur, Selman Kesici, Mutlu Uysal Yazıcı, Ebru Azapağası, Yasemin Taşcı Yıldız, Nesrin Ceylan, Saliha Şenel, and Deniz Yüksel.
• Department of Pediatric Neurology, Faculty of Medicine, Dr Sami Ulus Maternity Child Health and Diseases Training and Research Hospital, University of Health Sciences, Ankara, Turkey
• Turk J Med Sci. 2021 Apr 30; 51 (2): 706-715.

Background/AimAcute necrotizing encephalopathy is a rare type of acute encephalopathy characterized by multi-ocal brain lesions and associated severe neurological findings and various organ dysfunctions may accompany it.Materials And MethodsPatients with acute necrotizing encephalopathy of childhood diagnosed by pediatric neurology and pediatric intensive care at Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital between 2007 and 2020 were included in this study.ResultsNine patients (six females, three males) with a mean age of 4.05 ± 1.94 years (age range 1–6.5) were included in this study. The interval range between fever and encephalopathy in patients was 1–4 days. Influenza A (3H1N1, one untyped) was detected in four patients, influenza B in three patients, and no cause was found in two patients. Major clinical findings other than febrile encephalopathy in all patients were a hemodynamic shock in seven patients, seizures in six patients, vomiting in five patients, dystonia in three patients, and flaccid paralysis in the upper extremity in one patient. Despite all our treatment approaches, including plasmapheresis, moderate to severe neurological sequelae was observed in all of our patients, who survived even with significant radiological improvement. Three patients for whom we could not perform plasmapheresis died.ConclusionOur study revealed that thalamic involvement increased as the interval shortened, and brainstem involvement increased in patients over four years of age. The presence of persistent vomiting accompanying encephalopathy during the parainfectious period and plasmapheresis treatment being a treatment option that could prevent mortality were cautionary for our study.This work is licensed under a Creative Commons Attribution 4.0 International License.

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