PURPOSE To assess the frequency and clinical features of childhood-onset intractable epilepsy caused by the most common mutations in the POLG gene, which encodes the catalytic subunit of mitochondrial DNA polymerase gamma. METHODS Children presenting with nonsyndromic intractable epilepsy of unknown etiology but without documented liver dysfunction at presentation were eligible for this prosp...

Epilepsy is an important disease that affects brain function, particularly in those under 3 years old. Uncontrolled seizures can affect cognitive function and quality of life. For these reasons, many trials have been conducted to investigate treatments for pediatric epilepsy. Currently, many antiepileptic drugs are available for the treatment of epilepsy, but cases of intractable epilepsy conti...

Rasmussen encephalitis (RE) is a rare but devastating unihemispheric brain disorder that often affects children. The clinical picture is characterized by intractable focal epilepsy and progressive decline of functions associated with the affected hemisphere. Despite its known inflammatory background and T-cell involvement, immunotherapy appears to slow rather than halt disease progression, and ...

objective the lennox-gastaut syndrome (lgs: the triad of intractable seizures of various types, a slow spike-wave pattern in eeg and mental retardation) is one of the most difficult epilepsy syndromes to treat. the aim of this study was to evaluate the efficacy and safety of zonisamide (zns) as add-on therapy in seizures of children with lgs. materials & methods in a quasi- experimental study, ...

The effectiveness of resective surgery for the treatment of carefully selected patients with medically intractable, localization-related epilepsy is clear. Seizure-free rates following temporal lobectomy are consistently 65% to 70% in adults and 68% to 78% in children. Extratemporal resections less commonly lead to a seizure-free outcome, although one recent childhood series reported a seizure-...

Hemimegalencephaly, a congenital brain malformation typically characterized by enlargement of one hemisphere, is frequently associated with intractable epilepsy. The authors report a case of a 12-month-old girl with hemimegalencephaly who underwent semiurgent hemispherectomy because of rapidly escalating seizures, arrested development, and associated encephalopathy. The brain tissue was examine...

Traumatic brain injury (TBI) is a disruption in the brain functions following a head trauma. Cell therapy may provide a promising treatment for TBI. Human neural stem cells cultured in self-assembling peptide scaffolds have been proposed as a potential novel method for cell replacement treatment after TBI. In the present study, we accessed the effects of human neural stem/progenitor cells (hNS/...

BACKGROUND AND PURPOSE The aim of the current study is to review the clinical features, surgery outcomes and parental satisfaction of children with destructive encephalopathy who underwent epilepsy surgery due to medically intractable seizures. METHODS 48 patients who underwent epilepsy surgery from October 2003 to August 2011 at Severance Children's Hospital have been reviewed. The survey wa...

INTRODUCTION Epilepsy is one of the most frequently observed diseases in neurology outpatient care. METHODS We analysed our hospital's implementation of the 8 epilepsy quality measures proposed by the American Academy of Neurology: documented seizure types and seizure frequency, aetiology of epilepsy or the epilepsy syndrome, review of EEG, MRI, or CT results, counselling about antiepileptic ...

Correspondence to: Professor John S Duncan, Department of Clinical and Experimental Epilepsy, Institute of Neurology, Queen Square, University College London, London WC1N 3BG, UK; [email protected] _________________________ T he epilepsies are common serious diseases of the brain, with an age adjusted prevalence of 4–8/1000 and an annual incidence of 20–50/100 000 in developed countries. M...