A nationwide survey of adenovirus-associated encephalitis/encephalopathy in Japan.

BACKGROUND: Adenovirus is a major pathogen causing febrile illness among children. It may also cause acute encephalitis/encephalopathy. This study aimed to elucidate the clinical features of adenovirus-associated encephalitis/encephalopathy (AdVE) among children in Japan. METHODS: A nationwide survey of children with AdVE was conducted. An initial survey was distributed among pediatricians to obtain information about children with AdVE treated between January 2014 and March 2019. A second survey was used to obtain the clinical information of children with AdVE from hospitals that responded to the initial survey and those identified from a literature search of the reported cases. We collected demographic data and information about symptoms of infection, neurological symptoms, laboratory parameters, treatment, and outcomes. Outcomes were determined using the Pediatric Cerebral Performance Category Score. RESULTS: Clinical information was available for 23 children with a median age of 39 months. Two had preexisting neurological disorders and six had a history of febrile seizures. The outcome was good in 15 patients and poor in eight patients. Serum lactate dehydrogenase, glucose, and ammonia levels were higher among children with a poor outcome compared to those with a good outcome. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion was the most common type (n = 8), followed by acute encephalopathy with biphasic seizures and late reduced diffusion (n = 7). CONCLUSION: A prior history of febrile seizures was frequent in children with AdVE. Several different subtypes of acute encephalopathy were seen in children with AdVE, and the outcome was poor in those with acute encephalopathy with biphasic seizures and late reduced diffusion and hemorrhagic shock and encephalopathy syndrome. Elevated lactate dehydrogenase, glucose, and ammonia levels on admission were found to correlate with a poor outcome.

Lateralized interictal epileptiform discharges during rapid eye movement sleep correlate with epileptogenic hemisphere in children with intractable epilepsy secondary to tuberous sclerosis complex.

PURPOSE: We assessed lateralization of interictal epileptiform discharges (IEDs) in children with intractable epilepsy secondary to tuberous sclerosis complexes (TSCs) during rapid eye movement sleep (R), compared with non-rapid eye movement sleep (NR) and wakefulness (W), to determine epileptogenicity of R-IEDs. METHODS: We retrospectively studied 23 children with TSC, who underwent prolonged scalp video-electroencephalography (EEG) and magnetic resonance imaging (MRI). We determined the lateralization of ictal EEG, clinical semiology, and the largest tuber on MRI. We analyzed a minimum of 100 IEDs during R, NR, and W to classify right/left/generalized spikes to compare the lateralization with ictal EEG, clinical semiology, and MRI. KEY FINDINGS: R-IEDs were lateralized in 22 patients (96%), W-IEDs in 19 (83%), and NR-IEDs in 16 (70%). Ictal EEGs were lateralized in 15 patients (65%) and clinical semiology in 5 (22%). Ictal EEG lateralization was concordant to R-IEDs in 14 patients (93%), W-IEDs in 11 (73%), and NR-IEDs in 10 (67%). The lateralization of clinical semiology was concordant with R-IEDs in 4 (80%), W-IEDs in 3 (60%), and NR-IEDs in 2 (40%). MRI lateralization of the largest tuber was concordant with R-IEDs in 16 (70%), W-IEDs in 12 (52%), and NR-IEDs in 11 (48%). Thirteen patients (57%) underwent resective surgery, the hemisphere of which was concordant with R-IEDs in 13 (100%), W-IEDs in 8 (62%), and NR-IEDs in 8 (62%). Nine (69%) of them achieved good seizure control after surgery. SIGNIFICANCE: R provoked the most lateralized IEDs compared to NR and W in children with TSC. Lateralization of R-IEDs corresponded with the hemisphere of ictal EEG and largest tuber, and can be used to identify surgical candidacy in TSC children with intractable epilepsy.