Pre-surgical mapping of eloquent cortex for paediatric epilepsy surgery candidates: Evidence from a review of advanced functional neuroimaging.

Purpose: A review of all published evidence for mapping eloquent (motor, language and memory) cortex using advanced functional neuroimaging (functional magnetic resonance imaging [fMRI] and magnetoencephalography [MEG]) for paediatric epilepsy surgery candidates has not been conducted previously. Research in this area has predominantly been in adult populations and applicability of these techniques to paediatric populations is less established.

Methods: A review was performed using an advanced systematic search and retrieval of all published papers examining the use of functional neuroimaging for paediatric epilepsy surgery candidates.

Corticosteroids including ACTH for childhood epilepsy other than epileptic spasms.

Background: This is an updated version of the original Cochrane review published in Issue 1, 2007.Epilepsy is a disorder with recurrent epileptic seizures. Corticosteroids have been used in the treatment of children with epilepsy and have significant adverse effects.

Antiepileptic drug treatment of rolandic epilepsy and Panayiotopoulos syndrome: clinical practice survey and clinical trial feasibility.

Background: The evidence base for management of childhood epilepsy is poor, especially for the most common specific syndromes such as rolandic epilepsy (RE) and Panayiotopoulos syndrome (PS). Considerable international variation in management and controversy about non-treatment indicate the need for high quality randomised controlled trials (RCT). The aim of this study is, therefore, to describe current UK practice and explore the feasibility of different RCT designs for RE and PS.

Syncope-like epileptic seizures in Panayiotopoulos syndrome.

Objective: To describe the clinical features of syncope-like epileptic seizures (SLES) and their frequency in Panayiotopoulos syndrome (PS).

Methods: This was a 6-year prospective study of all children aged 1-15 years referred for an EEG. PS was defined by the occurrence of at least one autonomic seizure (AS) in a neurodevelopmentally normal child and at least one EEG with focal spikes.

Recovery of consciousness following acute symptomatic seizures due to central nervous system infections in children.

Aim: The aim of this study was to assess whether acute symptomatic epileptic seizures associated with central nervous system infections (AS(inf) ) have a different ictal and postictal course to seizures of other aetiologies.

Method: A case note analysis of 81 children (47 males; 34 females; age range 1mo-15y 6mo; median age 12mo) with central nervous system infections was undertaken. Seizure type, duration, aetiology, and timing were recorded.

Rufinamide: a new antiepileptic drug treatment for Lennox-Gastaut syndrome.

Lennox-Gastaut syndrome is a relatively rare epilepsy syndrome that usually begins in early-mid childhood and is characterized by multiple seizure types, particularly generalized seizures, which are often resistant to antiepileptic drug medication. Rufinamide is a new antiepileptic drug approved as adjunctive therapy to treat seizures in Lennox-Gastaut syndrome in those 4 years of age and older. In this article, the putative mechanism of action is described, along with data relating to its pharmacokinetics and metabolism.

Cogan's syndrome: a rare cause of meningoencephalitis.

We report a 14-year-old boy who presented with meningoencephalitis. Other features particularly auditory, vestibular, and ocular lead to the diagnosis of Cogan's syndrome. Treatment with prednisolone resulted in a rapid improvement and recovery of his hearing.

Panayiotopoulos syndrome: an important childhood autonomic epilepsy to be differentiated from occipital epilepsy and acute non-epileptic disorders.

Panayiotopoulos syndrome is a common multifocal autonomic childhood epileptic disorder with significant clinical, pathophysiological and management implications. It affects otherwise normal children with onset at around 3-6 years. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms and mainly ictal vomiting.

Treatment of Lennox-Gastaut Syndrome (LGS).

Lennox-Gastaut Syndrome (LGS) is a severe form of epilepsy that usually starts in early to mid childhood and is characterized by multiple seizure types, abnormal electroencephalogram with slow spike-and-wave discharges and cognitive problems. Numerous approaches are currently used to treat LGS, including use of conventional antiepileptic drugs (most commonly sodium valproate, lamotrigine and topiramate), other drug interventions (corticosteroids and intravenous immunoglobulin) and nonpharmacologic treatments (ketogenic diet, corpus callosotomy and vagus nerve stimulation). Rufinamide is the most recent antiepileptic drug to have shown efficacy in the treatment of LGS.

Clinical and molecular phenotype of Aicardi-Goutieres syndrome.

Aicardi-Goutieres syndrome (AGS) is a genetic encephalopathy whose clinical features mimic those of acquired in utero viral infection. AGS exhibits locus heterogeneity, with mutations identified in genes encoding the 3'-->5' exonuclease TREX1 and the three subunits of the RNASEH2 endonuclease complex. To define the molecular spectrum of AGS, we performed mutation screening in patients, from 127 pedigrees, with a clinical diagnosis of the disease.

Ethnic differences in the incidence of seizure disorders in children from Bradford, United kingdom.

We sought to determine if there are differences in the incidence of seizure disorders between the children of the indigenous and immigrant (predominantly Pakistani) populations of Bradford, United Kingdom. Annual incidence rates per 100,000 for new onset seizures were calculated along with Townsend deprivation scores. The incidence of seizures (including febrile and single) was 153 (95%CI 104-139).

Autonomic status epilepticus in Panayiotopoulos syndrome and other childhood and adult epilepsies: a consensus view.

Purpose: To discuss and propose a definition of autonomic status epilepticus (SE), describe its clinical and EEG features, and review what is known about its epidemiology, pathophysiology, differential diagnosis, and management.

Methods: An international consortium of established researchers in the field was identified from their published work, agreed the purpose of the project, searched the literature, and, by use of e-mail communication, agreed the consensus document.

Results: Autonomic SE is a condition lasting at least 30 min and characterized by epileptic activity causing altered autonomic function of any type at seizure onset or in which manifestations consistent with altered autonomic function are prominent (quantitatively dominant or clinically important) even if not present at seizure onset.

Idiopathic generalized epilepsies imitating focal epilepsies.

Classification of epileptic seizures and epilepsy syndromes as either focal or generalized is a fundamental and early part in the diagnostic process and is generally fairly easily accomplished. However, in patients with idiopathic generalized epilepsies, seizure and EEG features may suggest, particularly to the unwary, the occurrence of focal rather than generalized seizures. Misinterpretation of typical absence seizures as focal seizures, especially as temporal lobe seizures and of myoclonic seizures as focal clonic seizures, is a relatively common error and focal features during generalized tonic-clonic seizures may also be quite common.

The contribution of the EEG technologists in the diagnosis of Panayiotopoulos syndrome (susceptibility to early onset benign childhood autonomic seizures).

Purpose: To assess the contribution of the EEG technologists in the diagnosis of children with epileptic seizures.

Methods: We analysed the clinical information obtained by the EEG technologists from children with epileptic seizures and their parents, and assessed its value for the generation of a clinically useful EEG report and a plausible electroclinical diagnosis. Interviews were based on a qualitative questionnaire, and were videotaped.

Atypical evolution of Panayiotopoulos syndrome: a case report.

Panayiotopoulos syndrome is a relatively common condition with susceptibility to early onset benign childhood seizures, which manifests primarily with autonomic and mainly emetic symptoms. It predominantly affects children of 3-6 years of age (13% of those with one or more non-febrile seizures). EEG shows great variability, with occipital, extra-occipital spikes or brief generalised discharges alone or in combination; it may also be consistently normal.