Recurrent Acute Necrotizing Encephalopathy in a Canadian Aboriginal Child

Acute necrotizing encephalopathy (ANE) in childhood was originally described in the Far East.1,2 Disease onset is commonly preceded by a viral illness.1,3 Neurological deterioration occurs abruptly and rapidly.Neuroimaging is characteristic.4 Steroids and less commonly intravenous immunoglobulins have been used with variable outcomes.5 More recently, cases have been reported from Europe and North America.3,6,7 Here we present the first report of recurrent ANE in a Canadian Aboriginal child. CASE REPORTA previously healthy 34-month-old aboriginal Canadian boy from northern Manitoba presented with a four day history of cough and fever, and two day history of headache and irritability.He was sleepy the day prior to his presentation.There was no history of trauma, travel, rash or toxic ingestion.He did not have seizures.He was initially seen at the local hospital.His Glasgow Coma Scale was six.He was intubated, ventilated, and transferred to the Pediatric Intensive Care Unit at our hospital where he was started empirically on intravenous Cefotaxime, Vancomycin and Acyclovir.His past medical history is unremarkable.The mother was well during pregnancy.He was born at thirty six weeks gestation following an uneventful pregnancy and spontaneous vaginal delivery.His birth weight was 2.7 kg. His development was normal prior to his presentation.The parents are of Canadian Aboriginal Cree descent and are nonconsanguineous.Serial investigations were done including, full blood count (CBC), electrolytes, ammonia, glucose, liver function tests, cortisol, ACTH, TSH, T3, T4, vitamin B 12 , lead, antithyroid antibodies, ANCA, ANA, toxicology screen, capillary lactic acid, plasma amino acids, plasma very long chain fatty acids, whole blood palmitate oxidation assay (a rapid screening test for fatty acids oxidation defects), free and total serum carnitine levels, acylcarnitine profile, lysosomal enzymes assay panel, blood cultures; urine amino and organic acids; cerebrospinal fluid (CSF) glucose, protein, cell count, lactic acid, amino acids, oligoclonal bands, bacterial, fungal, and viral cultures including human herpes viruses (lumbar puncture was done twice, four days apart); and stool for Enteroviruses.The results were all normal except for mild elevation of CSF protein on the first lumbar puncture at 0.52g/L (normal range: 0.15 -0.4).The results of the oligoclonal bands were negative; however, there was a systemic immune reaction associated with CSF abnormality.The electroencephalogram (EEG) showed slowing in background activity consistent with an encephalopathic process.