Clinical characteristics of acute necrotizing encephalopathy in childhood

Objective To summary the clinical characteristics of acute necrotizing encephalopathy in childhood so as to estimate the prognosis and guide the therapy. Methods We collected and analyzed retrospectively the data of 10 acute necrotizing encephalopathy cases hospitalized in the pediatric department of our hospital from January 2014 to April 2018.The clinical manifestations, laboratory examinations and imaging features were retrospectively analyzed. Results A total of 10 cases all had fever and convulsions (100%). There was no specific clinical manifestation in the early stage, always showing the symptoms of acute respiratory infection or acute gastroenteritis.Mental malaise, lethargy, restlessness and frequent convulsions occured when the conditions aggravated.Nine patients had different degrees of consciousness disorder.It could be complicated by multiple organ dysfunction syndrome, hemophagocytic syndrome and so on.Eight patients underwent MRI.In acute phase, homogeneously prolonged T1 and T2 relaxation time of the brain lesions on MRI were found in most patients, and diffusion-weighted imaging (DWI) and FLAIR showed high signal intensity.Six cases underwent head CT examination, hypodensities were seen on 4 cases.Cerebrospinal fluid (CSF) was detected in all patients, and the protein levels in CSF increased to varying degrees, while white blood cells did not increase.Of the 10 patients, 3 died, 1 was discharged normally, 1 had hemophagocytic syndrome, transferred to pediatric hematology department, and 5 patients were transferred to rehabilitation department. Conclusion Acute necrotizing encephalopathy in children always make progress rapidly, has high mortality, many survivors have severe neurological sequelae, which is worthy of being attention. Key words: Acute necrotizing encephalopathy; Children; Clinical characteristics