Abstract
Background: Acute necrotizing encephalopathy of childhood (ANEC) is a rapidly progressing encephalopathy characterized by fever, depressed level of consciousness, and seizures. Diagnosis depends on clinical presentation and characteristic neuroimaging findings of abnormal signal intensity involving the thalami as well as the supra and infra-tentorial areas. Treatment modalities are not well-established; empirical treatment with antibiotics and antiviral agents is the initial step, followed by steroids and immunoglobulin, as well as supportive care. Patients with ANEC have a variable prognosis, but mortality is very high.Methods: A retrospective chart review of patients diagnosed with ANEC in five tertiary centers from January 2015 to October 2018 was performed. Clinical and radiological findings, as well as the therapeutic approach and outcomes, were described.Results: Twelve children were included ranging in age from 10 months to 6 years. All patients presented with preceding febrile illness, altered level of consciousness, and seizure. Radiological features showed abnormal signals in the thalami, and five patients (41.7%) had brainstem involvement. All patients received empirical treatment with antibiotics and antiviral agents. Ten patients (83.3%) received intravenous immunoglobulin (IVIG) and IV Methylprednisolone therapy. Outcomes were variable ranging from good outcomes with minimal neurological deficits to poor outcomes and death in 25% of cases.Conclusion: ANEC is a rare fulminant disease in children. The treatment is challenging. Early interventions with the use of IVIG and IV Methylprednisolone may change the outcome; however, further studies are needed to establish a consensus guideline for the management.
Highlights
Acute necrotizing encephalopathy of childhood (ANEC) is a fulminant type of encephalopathy
We aimed to present the clinical and radiological features, as well as treatment modalities and outcomes of 12 children with ANEC following viral infection in five tertiary care centers in Saudi Arabia
We included patients who were under 18 years and presented with clinical features consistent with ANEC in the form of altered level of consciousness, and seizures, preceded by viral illness with typical brain magnetic resonance imaging (MRI) findings of ANEC which include symmetrical, multifocal brain lesions involving mainly both thalami
Summary
Acute necrotizing encephalopathy of childhood (ANEC) is a fulminant type of encephalopathy. The most common clinical presentations are fever, rapid alteration in the level of consciousness, and seizures, in addition to characteristic findings in brain imaging that include, but are not limited to, bilateral thalamic lesions with supra and infra-tentorial lesions of variable dimensions [2]. The diagnosis of ANEC was determined by specific diagnostic criteria as described by Mizuguchi [1] which consist of [1] encephalopathy preceded by viral febrile illness with rapid deterioration in the level of consciousness and convulsions. Acute necrotizing encephalopathy of childhood (ANEC) is a rapidly progressing encephalopathy characterized by fever, depressed level of consciousness, and seizures. Diagnosis depends on clinical presentation and characteristic neuroimaging findings of abnormal signal intensity involving the thalami as well as the supra and infra-tentorial areas. Patients with ANEC have a variable prognosis, but mortality is very high
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