OBJECTIVE To describe the features of adult patients with benign, unilateral cerebral cortical encephalitis positive for the myelin oligodendrocyte glycoprotein (MOG) antibody. METHODS In this retrospective, cross-sectional study, after we encountered an index case of MOG antibody-positive unilateral cortical encephalitis with epileptic seizure, we tested for MOG antibody using our in-house, ...

We studied six patients suffering from benign rolandic epilepsy of childhood with central temporal spikes who presented so-called 'extreme somatosensory evoked potentials (SEPs)' following peripheral somatosensory stimulation. Stimuli were delivered to the fingers of one hand using both a triggered tendon hammer and low-intensity electrical stimulation. The electrical stimulation was delivered ...

Magnetic resonance imaging (MRI)-negative epilepsy may be successfully solved with a multidisciplinary approach using invasive recordings, image and signal analysis. The whole methodology used by the epilepsy surgery team is systematically described based on an resistant epilepsy case with all steps and rationale of choosing different investigation methods from surface electroencephalography (E...

There is currently increasing interest in identifying and classifying pediatric benign epilepsy syndromes and recently several new syndromes have been recognized. Benign epilepsy syndromes, by definition, occur in children with normal developmental history, respond well to therapy, and remit without sequelae. The large majority of children with benign epilepsy syndromes follow a truly benign co...

Benign adult familial myoclonic epilepsy is an inherited epileptic syndrome characterized by cortical hand tremors, myoclonic jerks, and seizures with no signs of early dementia. Worldwide prevalence is unknown, but is estimated to be less than 1/35,000. It is transmitted autosomal dominantly, and penetrance is high. This is a well-delineated disease with remarkable features that clearly distin...

BACKGROUND Over 60 Asian and European families with cortical myoclonic tremor and epilepsy have been reported under various names. Cerebellar changes may be part of the syndrome. In this study, we report the neuropathology findings in a new Dutch familial cortical myoclonic tremor with epilepsy case and review the literature on this syndrome. METHODS Neuropathological investigations were perf...

Background: Over 60 Asian and European families with cortical myoclonic tremor and epilepsy have been reported under various names. Cerebellar changes may be part of the syndrome. In this study, we report the neuropathology findings in a new Dutch familial cortical myoclonic tremor with epilepsy case and review the literature on this syndrome. Methods: Neuropathological investigations were perf...

Background Autosomal dominant familial cortical myoclonic tremor and epilepsy (FCMTE) is characterized by distal tremulous myoclonus, generalized seizures, and signs of cortical reflex myoclonus. FCMTE has been described in over 100 pedigrees worldwide, under several different names and acronyms. Pathological changes have been located in the cerebellum. This systematic review discusses the clin...

This article presents the updated, modified classification and definitions of epileptic syndromes, proposed by consensus International League Against Epilepsy (ILAE) task force. The ILAE report suggests a nosological revision epilepsy syndromes with onset in childhood, which are subdivided into three categories: (1) self-limited focal epilepsies; (2) genetic generalized (3) developmental and/or...