Objective Acute necrotizing encephalopathy (ANE) of childhood is described in increasing numbers in the European Union, but remains a rare disease with higher prevalence in Asian countries. The etiology of ANE remains uncertain. Infectious, metabolic, immune mediated or inherited reasons for ANE are reported.
Methods We report about an infant presenting an acute necrotizing encephalopathy.
Results An 18 month old European girl, previously healthy, born and immunized in Germany, attended our hospital with a history of 24 h of high fever and seizures. At time of admission the girl was comatose (Glasgow Coma Scale 4). Laboratory findings from serum showed physiologic values and no sign of major infection. Analysis of cerebrospinal fluid (CSF) revealed discrete pleocytosis and increased IL-6. No infectious pathogens were detected. There was no evidence for metabolic disease. Repeated MRI scans showed progressive symmetric necrotic areas in the thalami and brainstem. Additionally contrast-enhancement of basal meningea was detected. Based upon these neuroradiologic findings we diagnosed acute necrotizing encephalopathy. Typical neuroradiologic characteristics are multifocal, symmetric lesions involving thalami, brainstem, tegmentum, supratentorial white matter and cerebellum. Initially we treated our patient with broad specturm antibiotics and antiviral drugs. On day three of hospital stay we administered high dose cortisone and immunoglobulins. Despite a close multifactorial therapeutic strategy no clinical improvement was detectable.
Conclusions Clinicians should be aware of ANE as an important differential diagnosis of seizures and fever. Early diagnosis of ANE seems of importance for initiation of adequate treatment. Clinical improvement of ANE is reported after immediate immune-suppressive therapy.
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