Background: Perampanel is a novel drug recently approved as adjunctive therapy in epileptic patients.

A 3.5-year-old child with influenza B virus pneumonia developed pneumomediastinum and subcutaneous emphysema on the 3rd day of illness. Bronchoscopy demonstrated obstruction of the left main bronchus by mucopurulent sputum. Culture of the broncho-alveolar lavage yielded Stenotrophomonas maltophilia. After the respiratory complications resolved (11 days), the patient developed neurological symptoms and was diagnosed as acute disseminated encephalomyelitis (ADEM). Stenotrophomonas maltophilia was probably a factor in the development of pneumomediastinum. To our knowledge, this is the first case report of influenza virus infection with Stenotrophomonas maltophilia co-infection associated with spontaneous pneumomediastinum.

We retrospectively analyzed the patients with mitochondrial disorder based on muscle biopsy with ragged-red fibers by Gomori-trichrome stain, increased activity in NADH and SDH stains, and absent COX activity from January 1, 2000 to December 31, 2005. There were 76% of thirty-nine patients with male predominance who were diagnosed before the age of 5 (figure 1). The majority of patients were presented with developmental delay where the gut is the most frequently involved organ system (figure 2). This study describes clinical manifestations, laboratory findings, and prognosis of mitochondria myopathy in childhood living in Taiwan.

Objectives: Mitochondrial diseases represent a heterogeneous group of disorders with widely varying clinical features caused by structural and functional abnormalities in mitochondria. Our purpose was to evaluate the clinical characteristics, laboratory investigation of pediatric group with mitochondrial myopathies and cytopathies.

Objectives: There is a high fatality and morbidity rate of enterovirus 71 (EV71) infection. Extracorporeal membrane oxygenation (ECMO) could be a life saving facility in such patients; however, it takes the risk of cerebrovascular complications. We conduct this study to test the correlation of serum neuron specific enolase (sNSE) value and neurological outcomes in children with severe cardiorespiratory failure with ECMO treatment.

Aim: To study the difference between pyridoxine (PN) and its active form, pyridoxal phosphate, (PLP) in control of idiopathic intractable epilepsy in children. Methods: Among 574 children with active epilepsy, 94 (aged 8 months to 15 years) were diagnosed with idiopathic intractable epilepsy for more than six months. All received intravenous PLP 10 mg/kg, then 10 mg/kg/day in four divided doses. If seizures recurred within 24 hours, another dose of 40 mg/kg was given, followed by 50 mg/kg/day in four divided doses. For those patients whose seizures were totally controlled, PLP was replaced by the same dose of oral PN. If the seizure recurred, intravenous PLP was infused followed by oral PLP 50 mg/kg/day. Results: Fifty seven patients had generalised seizures (of whom 13 had infantile spasms) and 37 had focal seizure. Eleven had dramatic and sustained responses to PLP; of these, five also responded to PN. Within six months of treatment with PLP or PN, five of the 11 patients were seizure free and had their previous antiepileptic medicine tapered off gradually. Two were controlled with pyridoxine and the other three needed PLP to maintain seizure freedom. The remaining six responders needed PLP exclusively for seizure control. Six of the 11 responders to PLP had infantile spasms (46%); four of them needed PLP exclusively. The other five responders were in the remaining 81 patients with other seizure type. Conclusions: PLP could replace PN in the treatment of intractable childhood epilepsy, particularly in the treatment of infantile spasms.

Hemimegalencephaly is a rare neuronal migration disorder characterized by overgrowth of part or all of 1 cerebral hemisphere. Lissencephaly, pachygyria, polymicrogyria, heterotopia, and glial abnormalities are usually noted pathologically in the enlarged hemisphere. We report the sonographic changes associated with hemimegalencephaly in 2 neonates presenting with seizures and correlate these changes with the magnetic resonance imaging findings. © 2005 Wiley Periodicals, Inc. J Clin Ultrasound 33:243–247, 2005