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A 23-month-old Japanese girl with a severe form of congenital fiber type disproportion myopathy under mechanical ventilation suffered from respiratory syncytial virus (RSV) bronchiolitis, complicated with acute encephalopathy. She showed consciousness disturbance and convulsions followed by severe brain damage, a rare complication in RSV infection. Patients with severe neuromuscular diseases are vulnerable to RSV infection. Prophylactic interventions should be recommended., (© 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.)

Background: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized clinically by biphasic seizures and late magnetic resonance imaging abnormalities, such as reduced subcortical diffusion from day 3 onwards, often accompanied with some neurological sequelae. In the early stages of the disease, AESD closely resembles its far more prevalent and relatively benign counterpart, febrile seizure (FS)., Methods: We measured and compared the serum or cerebrospinal fluid (CSF) levels of S100B, neuron-specific enolase (NSE), and total tau protein in 43 patients with FS and 18 patients with AESD, at any point during the disease. To assess early diagnostic validity, we compared these biomarkers in 43 FS and eight AESD patients, with whom the day 0-2 samples were available. We used the receiver-operator characteristic curve to evaluate the diagnostic values of these markers., Results: The levels of all biomarkers were significantly higher in AESD than FS patients. When only day 0-2 samples from AESD patients were used, the levels of all the measured biomarkers, except serum NSE, were still significantly higher in patients with AESD than in FS, suggesting that AESD could damage astrocytes, neurons, and axons, even in the early stages of the disease. According to the receiver-operator characteristic curve analyses, CSF S100B (cut-off value, 100 pg/mL) and CSF total tau protein (cut-off value, 100 pg/mL) were better predictors of AESD than other biomarkers., Conclusion: The combination of CSF S100B and CSF total tau protein resulted in a positive predictive value of AESD 83.3%, which could be helpful for early diagnosis, facilitating early therapeutic interventions., (© 2011 The Authors. Pediatrics International © 2011 Japan Pediatric Society.)