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The International League Against Epilepsy ( ILAE) Classification of the Epilepsies has been updated to reflect our gain in understanding of the epilepsies and their underlying mechanisms following the major scientific advances that have taken place since the last ratified classification in 1989. As a critical tool for the practicing clinician, epilepsy classification must be relevant and dynamic to changes in thinking, yet robust and translatable to all areas of the globe. Its primary purpose is for diagnosis of patients, but it is also critical for epilepsy research, development of antiepileptic therapies, and communication around the world. The new classification originates from a draft document submitted for public comments in 2013, which was revised to incorporate extensive feedback from the international epilepsy community over several rounds of consultation. It presents three levels, starting with seizure type, where it assumes that the patient is having epileptic seizures as defined by the new 2017 ILAE Seizure Classification. After diagnosis of the seizure type, the next step is diagnosis of epilepsy type, including focal epilepsy, generalized epilepsy, combined generalized, and focal epilepsy, and also an unknown epilepsy group. The third level is that of epilepsy syndrome, where a specific syndromic diagnosis can be made. The new classification incorporates etiology along each stage, emphasizing the need to consider etiology at each step of diagnosis, as it often carries significant treatment implications. Etiology is broken into six subgroups, selected because of their potential therapeutic consequences. New terminology is introduced such as developmental and epileptic encephalopathy. The term benign is replaced by the terms self-limited and pharmacoresponsive, to be used where appropriate. It is hoped that this new framework will assist in improving epilepsy care and research in the 21st century. [ABSTRACT FROM AUTHOR]

A Case of Chromosomal 15q Proximal Tetrasomy Associated with Infantile Spasms and Partial Seizures and Review of Literature.Background:Infantile spasms are an age-dependent epileptic syndrome occurring during infancy, showing repetitive clusters of brief tonic spasms and hypsarrythmia on EEG, as well as psychomotor developmental arrest or deterioration. Numerous studies have been made concerning the etiology of this syndrome. Many causes are known, such as hypoxic encephalopathy during the perinatal period, tuberous sclerosis, and Down syndrome, although the precise underlying mechanism is unknown. Recently a few cases of infantile spasms associated with a chromosomal abnormality, 15q proximal tetrasomy, were reported. Here we present a similar case but with neurologically mild symptoms.Case Report:A 6-month-old girl with 15q proximal tetrasomy had infantile spasms (ISs) and cerebral cortical atrophy. She was born of healthy parents, after 39 weeks of gestation, with birth weight of 2,358 g. The perinatal period was uneventful. Developmental milestones were delayed, with head control at 5 months and rolling at 6 months. When she was age 6 months, her parents brought her to our clinic because of several series of tonic spasms. On physical examination, she showed no neurologically abnormal signs, except general muscle hypotonia. EEG examination showed hypsarrythmia. The brain magnetic resonance imaging and computed tomography showed diffuse cortical atrophy. No abnormal findings were observed in blood examination, CSF, and serologic tests for congenital viral infection of the CNS. A high-resolution analysis of chromosome 15 revealed 15q proximal tetrasomy, and 47,XX,+idic (15)(q13). Before treatment, the DQ score was 62. Tonic spasms subsided after zonisamide monotherapy without adrenocorticotropic hormone (ACTH) treatment. However, soon after the cessation of tonic spasms, she showed partial seizures, which was easily controlled with carbamazepine. Since then, no seizure recurrence was observed during 2 years of follow-up. The total DQ score is 52.Discussion:The supernumerary chromosome may occur in∼0.05% of live births (Buckton, 1980). As many as 50% of supernumerary chromosomes are derived from chromosome 15, and almost 80% of them are inv dup (15). The inv dup (15) individuals are known to manifest mental retardation, psychomotor developmental delay, hypotonia, behavioral abnormality, dysmorphy, and also seizure disorder, with various degrees from normal to severe. Chromosome 15 includes the Prader–Willi syndrome/Angelman syndrome (PW/AS) chromosomal region and also contains some neurotransmitter-related regions such asγ-aminobutyric acid (GABA) receptor subunitsβ3,α5, andγ3, and nicotinic receptorα7. Longer duplication is said to be associated with severe symptoms. However, some cases have large duplications with mild symptoms. The mechanism is unknown. Previously, three cases of 15q proximal tetrasomy with ISs were reported (Kobayashi, 1994; Peter, 1996; Cabrera, 1998). Among cases with inverted duplication of chromosome 15, the duplication occurred at 15pter-q13. In all cases, the ISs were easily controlled with ACTH or ZNS. However, the severity of the subsequent epilepsy varied among cases. Such variable severity is considered to arise from markers related to epileptogenesis present in the regions of duplication, although it is still controversial. Several epilepsy cases were found to be associated with inverted duplication of chromosome 15, but at different regions (i.e., 15q12, 15q14, pter-q11::q13-pter, and direct duplication of 15q15-q24). No ISs cases were among them. One case of ISs with chromosome 15 abnormalities; ring 15, was reported in Japan (Ishii et al., 1988).Conclusions:Chromosome analysis should be included to find out the etiology of West syndrome. In addition, in the case of supernumerary chromosomes, especially in inv dup (15) cases, precise analysis of markers around the PW/AS region provides more information concerning the basic mechanisms of epilepsy. Reciprocal Release Profile Between Hippocampal Monoamine and Acetylcholine Releases in a Novel Spontaneous Epilepsy Model,μ3b-Deficient Mice.Purpose:A novel adaptor protein (AP) complex type 3 (AP-3) was recently identified in mammalian cells. AP-3 is composed of two large subunits (δ andβ3A orβ3B), a medium-sizedμ subunit (μ3A orμ3B), and a smallσ subunit (σ3A orσ3B). Specific expression ofβ3B andμ3B subunits in neurons and neuroendocrine cells has been identified, suggesting that AP-3B, which is composed ofδ,β3B,μ3B, andδ3A/B subunits, has a specific role to play in neurons and may be involved in de novo synaptic vesicle biogenesis in endosomes. Recently we established theμ3B gene knockout mice (μ3B knockout mice), which have lost neuron-specific AP-3B and manifest spontaneous epileptic seizures. To understand the mechanisms of seizures inμ3B knockout mice, we determined the releases of dopamine (DA), serotonin (5-HT), and acetylcholine (ACh) inμ3B knockout mice and wild-type mice, by using hippocampal minislices in vitro.Methods:All of the experiments described here were performed in accordance with the specifications of the Animal Research Committee of Hirosaki University and met the Guidelines for Animal Experimentation of Hirosaki University.μ3B knockout mice were generated essentially as described by Nakatsu et al. (2003) and maintained the C57BL(6J background. Eight-week-old wild-type andμ3B knockout mice were used, and the hippocampal tissue was dissected according to the method of Glowinski and Iversen (1966). The 350× 350-μm thick minislices were prepared on a chopper and were incubated in 0.5 ml artificial cerebrospinal fluid (aCSF) at 35°C for 5 min. The levels of DA and 5-HT in aCSF were determined by high-performance liquid chromatography (HPLC) equipped with electrochemical detection (Eicom, Kyoto, Japan), and the level of ACh was determined by ECD-HPLC (Eicom) equipped with a postcolumn enzymatic reactor containing acetylcholinesterase and cholineoxidase (Eicom). To study the effects of an increase in extracellular K+ level on hippocampal extracellular levels of DA, 5-HT, and ACh, 50 mMpotassium–evoked stimulation was performed for 5 min after confirming stabilization of basal releases.Results:No significant difference was found in the basal releases of DA and 5-HT betweenμ3B knockout mice and wild-type mice in the hippocampus, whereas the basal hippocampal ACh release was higher inμ3B knockout mice than that in wild-type mice (p<0.05). In contrast, 50 mMpotassium–evoked hippocampal releases of DA and 5-HT inμ3B knockout mice were lower than those in wild-type mice (p<0.01), whereas potassium-evoked ACh release was significantly higher inμ3B knockout mice than in wild-type mice (p<0.01).Conclusions:In the present study, we determined the differences in neurotransmitter releases betweenμ3B knockout mice and wild-type mice, by using hippocampal minislices in vitro. This study demonstrated an imbalanced relation between hippocampal monoaminergic and acetylcholinergic transmission inμ3B knockout mice, a reduction of DA and 5-HT release and an increase of ACh release. Therefore these findings indicate that the imbalance between these neurotransmission systems may be, at least partially, involved in the mechanisms of spontaneous seizures inμ3B knockout mice. AP-3B plays an important role in the exocytosis of neurotransmitters by regulating the intracellular secretory and endocytic pathways. Monoaminergic and acetylcholinergic neurons may differentially depend on the molecules of AP complex, especially AP-3B, to exert their functions. [ABSTRACT FROM AUTHOR]

Eponyms continue to have their place in medicine but there are pitfalls associated with their use. 'Priorities' may be debatable, misattributions are not uncommon, and knowledge of the original papers is often insufficient. A. Ya. Kozhevnikov (1836-1902) is considered to be the founder of the Russian neurology, best known in the West for his work on epilepsia partialis continua (EPC), published in 1894. Kozhevnikov considered various natures for this disorder but thought chronic infectious etiology to be the most probable. Shortly the eponym Kozhevnikov epilepsy was coined and used in clinical practice and writing. Thirty-five years after Kozhevnikov's death, in 1937, a new form of viral encephalitis, Russian spring-summer tick-borne encephalitis (RTBE), was discovered, which was strongly associated with EPC and at times incorrectly attributed to Kozhevnikov by Russian (Soviet) and West-European scientists, although he never specifically identified or even could have recognized this disease entity. When, in 1958, Canadian scientists published about persisting focal epilepsy due to chronic focal encephalitis in children, a new disease was proclaimed: Rasmussen syndrome or Rasmussen chronic encephalitis. The only reference to Kozhevnikov in the Canadian papers was the incorrect suggestion that Kozhevnikov himself described EPC in RTBE. This historical error resulted in continuing misquotations of Kozhevnikov in the current literature and controversies concerning the place of Kozhevnikov epilepsy in the Classification Scheme of the International League Against Epilepsy (ILAE). The history of Kozhevnikov epilepsy thereby offers an illustrative example of the successive misunderstandings, errors, and controversies that arise due to insufficient knowledge or understanding of the original publications, questionable post hoc interpretations of earlier findings, misquoting of secondary papers, or a combination of all these. [ABSTRACT FROM AUTHOR]

This paper provides a survey of the changing concepts of the etiology of epilepsy from 1860 to 2010, focusing on the first two 50-year periods and outlining more briefly major developments in the past 50 years. Among the concepts reviewed in the first 100 years are: the division between predisposing and exciting causes, idiopathic and genuine epilepsy, organic epilepsy, the concept of 'cause' being equivalent to 'causal mechanism,' Russell Reynolds etiological classification, the neurological taint and theories of degeneration, the self-perpetuating nature of seizures, reflex theories of etiology, autointoxication, heredity and eugenics, epilepsy due to brain disorders, the role of EEG and of hippocampal sclerosis, psychological theories of causation, and the multifactorial view of epilepsy etiology. In the past 50 years, the major advances in studying causation in epilepsy have been: clinical biochemistry, neuroimaging, molecular genetics, studies of mechanisms of epilepsy, better statistical methodologies and classification. A number of general observations can be made: the identification of 'cause' is not as simple as it might at first appear; progress in the study of causation has been often erratic and travelled up many cul-de-sacs; theories of causation are heavily influenced by societal influences and fashion, and is also heavily dependent on applied methodologies; the recently explored possibility that the underlying inherited mechanisms of epilepsy are shared with other neuropsychiatric conditions is in effect a reinvention of the concept of the neurological trait, and this has ethical and social implications. Considering and classifying cause in terms of causal mechanism, as was suggested by Hughlings Jackson, is an ultimate goal. [ABSTRACT FROM AUTHOR]

Summary This paper describes the various electroencephalographic (EEG) patterns expressed by the comatose brain, starting with the sleep-like oscillations associated with light coma. Deeper coma generally displays a burst-suppression pattern characterized by alternating episodes of isoelectric (flat) EEG and bursting slow waves. The latter are the result of cortical hyperexcitability, as demonstrated by intracellular recordings in anesthetized animals. Further deepening of the coma yields to continuous isoelectric EEG and eventually results in a newly discovered type of spiky waves that have been termed ν-complexes. They originate in the hippocampus as a result of intrinsically generated oscillations (ripples) in the delta range. [ABSTRACT FROM AUTHOR]

The article presents information about abstracts of various papers that will be discussed at the Discussion Group of the 6th European Congress on Epileptology that will be held on June 2, 2004. One of the papers that will be discussed is "Temporal Lobe Epilepsies, Recent Concepts," by C. Baumgartner. Temporal lobe epilepsies-the most frequent form of focal epilepsies-can be classified according to etiology, location of the seizure-onset zone within the temporal lobe and bilaterality. Other papers that will be discussed are "Mesial Temporal Lobe Epilepsy: Seizures of Hippocampal Onset," by H.G. Wieser and "The Role of Amygdala and Temporal Polar Area," by P. Kahane.

Objective: To investigate the effectiveness and safety of the ketogenic diet (KD) in drug‐resistant epilepsy in childhood in relation to the new 2017 International League Against Epilepsy (ILAE) classification of etiology. Methods: A consecutive cohort of patients treated with the KD were categorized according to the ILAE classification into known (structural, genetic, metabolic, infectious, and immune‐mediated) and unknown etiology. Primary outcome was the frequency of patients achieving seizure freedom with the KD at 3 months, secondary outcomes were seizure reduction >50% at 3 months, and both seizure freedom and seizure reduction >50% at 6, 12 months, and at last follow‐up (LFU), and adverse effects. Outcomes were compared between etiology groups. Results: Etiology was known in 70% (129/183). Outcomes did not differ at 3 months (known vs unknown: seizure freedom 28% vs 33%, seizure reduction 62 vs 67%), but seizure freedom was significantly less frequent in known etiology at 6 months (26% vs 43%) and beyond (22% vs 37%). Logistic regression identified duration of epilepsy, number of previous antiseizure medications (ASMs), and age‐appropriate psychomotor development as positive determinants of outcome. Among individual etiology groups, the effectiveness of KD was relatively best for genetic (33% at LFU) and poorest for metabolic etiology (8% at LFU). The small number of patients with infectious and immune‐mediated etiology requires larger numbers in each etiology group to corroborate our results. No differences in type and frequency of adverse effects (in 71%) between etiology groups were observed, requiring medical intervention in 21%. Significance: The KD was most effective in genetic and unknown etiology, many unknowns probably represent yet unidentified genetic causes. We recommend consequent diagnostic and genetic work‐up to identify etiologies that respond best to the KD. The KD should be offered early to infants with genetic epilepsy before deterioration of epileptic symptoms and of psychomotor development. [ABSTRACT FROM AUTHOR]

The article introduces a series of articles on the papers presented at the Innsbruck Colloquium on Status Epilepticus that took place in Innsbruck, Austria in 2007. They include articles on basic research and clinical neurophysiology of status epilepticus (SE), with special consideration to the developing brain, etiology of SE with emphasis on infection and immunology, as well as emergency treatment of SE and its medical and legal aspects.

The article presents information about abstracts of various papers that will be discussed at the Platform Session of the 6th European Congress on Epileptology that will be held on May 31, 2004. One of the papers that will be discussed is "Long-Term Seizure Control in Children With Malformations of Cortical Development," by N.J. Jovic and V. Brankovic-Sreckovie. Malformations of cortical development are often recognised as underlying aetiology for intractable seizures, mental retardation and neurological deficits in children. Other papers that will be discussed are "The Spectrum of Epilepsy in Children With Hippocampal Malrotation," by G. Buyse and J. Pauwels and "Do We Need to Measure Quality of Life in Children With Epilepsy?" by M.B. Mazurkiewicz-Beldzinska and A. Matheisel.

The article presents information about abstracts of various papers that will be discussed at the Discussion Group of the 6th European Congress on Epileptology that will be held on June 3, 2004. One of the papers that will be discussed is "Etiology and Treatment of Refractory Convulsive SE in Adults," by M. Walker. Refractory convulsive status epilepticus may occur in up to 30 % of adults who present with convulsive status epilepticus, and there is good experimental evidence that the longer that status epilepticus continues the more resistant it becomes to treatment. Other papers that will be discussed are "Etiology and Treatment of Non-Convulsive SE," by K. Malmgren and "Etiology and Treatment of Refractory Convulsive SE in Children," by R. Appleton.

The article presents information about abstracts of various papers that will be discussed at the Discussion Group of the 6th European Congress on Epileptology that will be held on June 2, 2004. One of the papers that will be discussed is "Clinical Evolution and Symptomatology of Stroke and Head Trauma-Induced Epilepsy-Similar Rather Than Different?" by M.I. Lossius, O.M. Ronning, K. Nestvold, and L. Gjerstad. A broad spectre of brain injuries and brain lesions increase the risk for development of seizures and epilepsy and account for 30-49% of all cases. Other papers that will be discussed are "Does the Hippocampus Tell Us What Caused the Epilepsy-Human Data?" by I. Blumcke and "The Same AED for All Etiologies?" by G.J. Sills.

The article presents information about abstracts of various papers that will be discussed at the Discussion Group of the 6th European Congress on Epileptology that will be held on June 2, 2004. One of the papers that will be discussed is "Nocturnal Movement Disorders and Epilepsy: Overlap Syndromes?" by K.R. Chaudhuri. Generalized tonic clonic seizures or partial motor seizures can mimic hyperkinetic movement disorders such as myoclonus. Rarely chorea/choreoathetoid movements, tics or dystonia may also complicate disorders, which manifest with epilepsy. Other papers that will be discussed are "Basal Ganglia in Experimental Models of Epilepsy," by C. Deransart and A. Depaulis and "SPECT Imaging Basal Ganglia and Thalamic Involvement," by R. Duncan.

Objective: Population‐based data on epilepsy syndromes and etiologies in early onset epilepsy are scarce. The use of next‐generation sequencing (NGS) has hitherto not been reported in this context. The aim of this study is to describe children with epilepsy onset before 2 years of age, and to explore to what degree whole exome and whole genome sequencing (WES/WGS) can help reveal a molecular genetic diagnosis. Methods: Children presenting with a first unprovoked epileptic seizure before age 2 years and registered in the Stockholm Incidence Registry of Epilepsy (SIRE) between September 1, 2001 and December 31, 2006, were retrieved and their medical records up to age 7 years reviewed. Children who met the epilepsy criteria were included in the study cohort. WES/WGS was offered in cases of suspected genetic etiology regardless of whether a structural or metabolic diagnosis had been established. Results: One hundred sixteen children were included, of which 88 had seizure onset during the first year of life and 28 during the second, corresponding to incidences of 139 and 42/100 000 person‐years, respectively. An epilepsy syndrome could be diagnosed in 54% of cases, corresponding to a birth prevalence of 1/1100. Structural etiology was revealed in 34% of cases, a genetic cause in 20%, and altogether etiology was known in 65% of children. The highest diagnostic yield was seen in magnetic resonance imaging (MRI) with 65% revealing an etiology. WES/WGS was performed in 26/116 cases (22%), with a diagnostic yield of 58%. Significance: Epilepsy syndromes can be diagnosed and etiologies revealed in a majority of early onset cases. NGS can identify a molecular diagnosis in a substantial number of children, and should be included in the work‐up, especially in cases of epileptic encephalopathy, cerebral malformation, or metabolic disease without molecular diagnosis. A genetic diagnosis is essential to genetic counselling, prenatal diagnostics, and precision therapy. [ABSTRACT FROM AUTHOR]

Objective: Seizure recurrence following surgery for temporal lobe (TL) epilepsy may be related to extratemporal epileptogenic foci, so‐called temporal‐plus (TL+) epilepsy. Here, we sought to leverage whole brain connectomic profiling in magnetoencephalography (MEG) to identify neural networks indicative of TL+ epilepsy in children. Methods: Clinical and MEG data were analyzed for 121 children with TL and TL+ epilepsy spanning 20 years at the Hospital for Sick Children. Resting‐state connectomes were derived using the weighted phase lag index from neuromagnetic oscillations. Multidimensional associations between patient connectomes, TL versus TL+ epilepsy, seizure freedom, and clinical covariates were performed using a partial least squares (PLS) analysis. Bootstrap resampling statistics were performed to assess statistical significance. Results: A single significant latent variable representing 66% of the variance in the data was identified with significant contributions from extent of epilepsy (TL vs TL+), duration of illness, and underlying etiology. This component was associated with significant bitemporal and frontotemporal connectivity in the theta, alpha, and beta bands. By extracting a brain score, representative of the observed connectivity profile, patients with TL epilepsy were dissociated from those with TL+, independent of their postoperative seizure outcome. Significance: By analyzing 121 connectomes derived from MEG data using a PLS approach, we find that connectomic profiling could dissociate TL from TL+ epilepsy. These findings may inform patient selection for resective procedures and guide decisions surrounding invasive monitoring. [ABSTRACT FROM AUTHOR]

Objective: To determine the underlying etiologies in a contemporary cohort of infants with infantile spasms and to examine response to treatment. Methods: Identification of the underlying etiology and response to treatment in 377 infants enrolled in a clinical trial of the treatment of infantile spasms between 2007 and 2014 using a systematic review of history, examination, and investigations. They were classified using the pediatric adaptation of International Classification of Diseases, Tenth Revision (ICD‐10). Results: A total of 219 of 377 (58%) had a proven etiology, of whom 128 (58%) responded, 58 of 108 (54%) were allocated hormonal treatment, and 70 of 111 (63%) had combination therapy. Fourteen of 17 (82%, 95% confidence interval [CI] 59% to 94%) infants with stroke and infarct responded (compared to 114 of 202 for the rest of the proven etiology group (56%, 95% CI 48% to 62%, chi‐square 4.3, P = .037): the better response remains when treatment allocation and lead time are taken into account (odds ratio 5.1, 95% CI 1.1 to 23.6, P = .037). Twenty of 37 (54%, 95% CI 38% to 70%) infants with Down syndrome had cessation of spasms compared to 108 of 182 (59%, 95% CI 52% to 66%, chi‐square 0.35, P = .55) for the rest of the proven etiology group. The lack of a significant difference remains after taking treatment modality and lead‐time into account (odds ratio 0.8, 95% CI 0.4 to 1.7, P = .62). In Down syndrome infants, treatment modality did not appear to affect response: 11 of 20 (55%) allocated hormonal therapy responded, compared to 9 of 17 (53%) allocated combination therapy. Significance: This classification allows easy comparison with other classifications and with our earlier reports. Stroke and infarct have a better outcome than other etiologies, whereas Down syndrome might not respond to the addition of vigabatrin to hormonal treatment. [ABSTRACT FROM AUTHOR]

Objective: To determine the incidence, etiology, and outcome of status epilepticus (SE) in Auckland, New Zealand, using the latest International League Against Epilepsy (ILAE) SE semiological classification. Methods: We prospectively identified patients presenting to the public or major private hospitals in Auckland (population = 1.61 million) between April 6, 2015 and April 5, 2016 with a seizure lasting 10 minutes or longer, with retrospective review to confirm completeness of data capture. Information was recorded in the EpiNet database. Results: A total of 477 episodes of SE occurred in 367 patients. Fifty‐one percent of patients were aged <15 years. SE with prominent motor symptoms comprised 81% of episodes (387/477). Eighty‐four episodes (18%) were nonconvulsive SE. Four hundred fifty episodes occurred in 345 patients who were resident in Auckland. The age‐adjusted incidence of 10‐minute SE episodes and patients was 29.25 (95% confidence interval [CI] = 27.34‐31.27) and 22.22 (95% CI = 20.57‐23.99)/100 000/year, respectively. SE lasted 30 minutes or longer in 250 (56%) episodes; age‐adjusted incidence was 15.95 (95% CI = 14.56‐17.45) SE episodes/100 000/year and 12.92 (95% CI = 11.67‐14.27) patients/100 000/year. Age‐adjusted incidence (10‐minute SE) was 25.54 (95% CI = 23.06‐28.24) patients/100 000/year for males and 19.07 (95% CI = 16.91‐21.46) patients/100 000/year for females. The age‐adjusted incidence of 10‐minute SE was higher in Māori (29.31 [95% CI = 23.52‐37.14]/100 000/year) and Pacific Islanders (26.55 [95% CI = 22.05‐31.99]/100 000/year) than in patients of European (19.13 [95% CI = 17.09‐21.37]/100 000/year) or Asian/other descent (17.76 [95% CI = 14.73‐21.38]/100 000/year). Seventeen of 367 patients in the study died within 30 days of the episode of SE; 30‐day mortality was 4.6%. Significance: In this population‐based study, incidence and mortality of SE in Auckland lie in the lower range when compared to North America and Europe. For pragmatic reasons, we only included convulsive SE if episodes lasted 10 minutes or longer, although the 2015 ILAE SE classification was otherwise practical and easy to use. [ABSTRACT FROM AUTHOR]

The article presents information about abstracts of various papers that will be discussed at the Discussion Group of the 6th European Congress on Epileptology that will be held on June 2, 2004. One of the papers that will be discussed is "Pharmacokinetic-Based Design of New Antiepileptics and CNS Drugs," by M. Bialer. Several of the new antiepileptic drugs (AEDs) are second generation or follow-up compounds of existing AEDs that their development was pharmacokinetic- (PK)-based design in order to overcome the PK drawbacks possessed by the parent compound. Other papers that will be discussed are "How to Design New CNS Drugs That Will Possess Antiepileptogenic Activity?" by A. Nehlig and "Mechanism-Based Design of New Antiepileptics and CNS Drugs," by C.E. Elger.

The article presents information about abstracts of various papers that will be discussed at the Discussion Group of the 6th European Congress on Epileptology that will be held on June 2, 2004. One of the papers that will be discussed is "Seizure Semiology and Ictal SPECT," by S. Asenbaum. One of the main purposes of nuclear medicine in neurology is the investigation of the epileptogenic zone (EZ) in the presurgical evaluation of patients with therapy refractory focal seizures. Other papers that will be discussed are "Ictal SPECT in Extratemporal Lobe Epilepsy: Contribution or Confusion?" by K. Tatsch and "Is There a Role for Benzodiazepine Receptor SPECT in Focal Epilepsy?" by J.O. Rinne.

The article presents information about abstracts of various papers that will be discussed at the Discussion Group of the 6th European Congress on Epileptology that will be held on June 2, 2004. One of the papers that will be discussed is "Tuberous Sclerosis," by N. Migone, L. Longa, A. Allavena, M. Barberis, S. Padovan, I. Borelli, M. Ruggieri and E. Grosso. Tuberous sclerosis complex is an autosomal dominant, genetically heterogeneous disease, characterised by the growth of multiple hamartomas in almost every organs and by a dramatic phenotypic variability. Other papers that will be discussed are "Neuronal Migration Disorders," by R. Guerrini and "Progressive Myoclonus Epilepsies," by A.E. Lehesjoki.

Summary: Children and the elderly (≥60 years of age) have the highest incidence of status epilepticus (SE). Because of their general health, elderly individuals are much more likely than younger (<60 years of age) persons to have more severe consequences from seizures. The incidence of SE is 15.5/100 000 in the 60‐69 age group, 21.5/100 000 in the 70‐79 age group and 25.9/100 000 in persons 80 and older. The most common cause in the elderly is acute symptomatic, with stroke and hypoxia the most frequent. The overall mortality of SE is quite high and occurs early, often within the first few days, and is related to the cause, with mortality of more than 80% in persons with anoxia. Although the cause of SE is an important factor in mortality, the aging body and brain may contribute to an unfavorable outcome. Treatment in the elderly is essentially the same as in younger adults with benzodiazepines (lorazepam, diazepam, clonazepam) and longer acting antiseizure drugs (phenytoin, fosphenytoin, valproate, levetiracetam, and lacosamide. At this time there are no evidence‐based studies regarding Axis 2 (etiology) and Axis 4 (age). All current interventions for SE involve antiseizure drugs that were developed for treatment of chronic epilepsy. Treatments should be developed that are more specific for the various etiologies and involve drugs that work on the underlying cause of the SE. [ABSTRACT FROM AUTHOR]

Summary: Objective: Patients with genetic generalized epilepsy (GGE) have subtle morphologic abnormalities of the brain revealed with magnetic resonance imaging (MRI), particularly in the thalamus. However, it is unclear whether morphologic abnormalities of the brain in GGE are a consequence of repeated seizures over the duration of the disease, or are a consequence of treatment with antiepileptic drugs (AEDs), or are independent of these factors. Therefore, we measured brain morphometry in a cohort of AED‐naive patients with GGE at disease onset. We hypothesize that drug‐naive patients at disease onset have gray matter changes compared to age‐matched healthy controls. Methods: We performed quantitative measures of gray matter volume in the thalamus, putamen, caudate, pallidum, hippocampus, precuneus, prefrontal cortex, precentral cortex, and cingulate in 29 AED‐naive patients with new‐onset GGE and compared them to 32 age‐matched healthy controls. We subsequently compared the shape of any brain structures found to differ in gray matter volume between the groups. Results: The thalamus was the only structure to show reduced gray matter volume in AED‐naive patients with new‐onset GGE compared to healthy controls. Shape analysis revealed that the thalamus showed deflation, which was not uniformly distributed, but particularly affected a circumferential strip involving anterior, superior, posterior, and inferior regions with sparing of medial and lateral regions. Significance: Structural abnormalities in the thalamus are present at the initial onset of GGE in AED‐naive patients, suggesting that thalamic structural abnormality is an intrinsic feature of GGE and not a consequence of AEDs or disease duration. [ABSTRACT FROM AUTHOR]

Objective The study provides updated information about the distribution of seizures, epilepsies, and etiologies of epilepsy in the general child population, and compares the old and new classification systems from the International League Against Epilepsy ( ILAE). Methods The study platform was the Norwegian Mother and Child Cohort Study. Cases of epilepsy were identified through registry linkages and sequential parental questionnaires. Epilepsy diagnoses were validated using a standardized protocol, and seizures, epilepsies, and etiologies were classified according to the old ( ILAE 1981/1989) and new ( ILAE 2017) classifications. Information was collected through medical record reviews and/or parental telephone interviews. Results The study population included 112,744 children aged 3-13 years at the end of follow-up on December 31, 2012. Of these, there were 606 children with epilepsy ( CWE). Distribution of seizure types varied by age of onset. Multiple seizure types were common with early onset. Focal epilepsies were the most common, occurring in 317 per 100,000 children in the study population and in 59% of CWE. Generalized epilepsies were found in 190 per 100,000 (35% of CWE). CWE with onset during the first 2 years of life had an even distribution of focal and generalized epilepsies, whereas focal epilepsies became dominant at later ages of onset. A definite cause of epilepsy had been demonstrated in 33% of CWE. The ILAE 1989 classification allowed for a broad syndrome category in 93% of CWE and a defined epileptic syndrome in 37%. With the ILAE 2017 classification, 41% of CWE had a defined epileptic syndrome and 63% had either a defined syndrome or structural-metabolic etiology. Significance The distribution of seizures and epilepsies is strongly dependent on age of onset. Despite diagnostic advances, the causes of epilepsy are still unknown in two-thirds of CWE. The ILAE 2017 classifications allow for a higher precision of diagnoses, but at the expense of leaving more epilepsies classifiable only at the mode of onset level. [ABSTRACT FROM AUTHOR]

Objective The increasing incidence of new-onset seizures with age is well known. Often, the etiology cannot be clarified. In the present study, patients with unprovoked late-onset seizures and without known neoplasm, who might have had paraneoplastic encephalitis, were investigated for a potentially underlying autoimmunity. Methods Sixty-six consecutive patients (36 women; aged ≥55 years) after having at least one seizure or seizures for ≤6 months were prospectively identified over a period of 4.75 years. All patients were tested for serum and cerebrospinal fluid ( CSF) antibodies (Abs) to both neural cell-surface and intracellular antigens. Forty-five (68%) underwent brain magnetic resonance imaging ( MRI). Follow-up in Ab-positive cases was ≥6 months. Results Two patients had high titers of anti- CASPR2 (contactin-associated protein-like 2) Abs in serum and CSF and fulfilled the diagnostic criteria of definite limbic encephalitis. Another two patients had bilateral encephalitic temporal MRI abnormalities. They also satisfied the criteria of definite limbic encephalitis, even though they had no Abs in serum or CSF. All four were in the age range of 55-70 years. They received immunotherapy and/or antiepileptic drug treatment and became seizure-free. Significance Our findings suggest that autoimmunity should be considered an important etiology in patients with late-onset seizures. Testing for neural antibodies and brain MRI may be worthwhile in this patient group. [ABSTRACT FROM AUTHOR]

Objective The literature is sparse on the complex interrelationships between stressors, depression, anxiety disorders, and epilepsy. We hypothesized that a relationship exists between stress and epilepsy. We evaluated whether markers of stress are associated with seizure recurrence in a low income community-based cohort of adults with single unprovoked seizure or newly diagnosed epilepsy. Methods We ascertained adult residents of Northern Manhattan and Harlem, New York City, with a first unprovoked seizure or newly diagnosed epilepsy, between December 2010 and January 2013. At enrollment, we collected information about seizure phenomenology, demographics, clinical information, and measures of stress (environmental stress, stressful life events, facets of allostatic load-i.e., the cumulative effect of adaptation to stress, psychiatric disorders, and low collective efficacy). Collective efficacy assesses neighborhood characteristics and incorporates social cohesion and informal social control. All subjects were followed for 2 years for further seizures. Cox proportional hazard regression models were used to estimate the hazard ratios of seizure recurrence during the 2 years of follow-up. Results We identified 52 subjects (64.2%) with a single unprovoked seizure and 29 (35.9%) with newly diagnosed epilepsy. Seizure recurrence was recorded in 38.5% (N = 20) of subjects with a single unprovoked seizure and in 69% of those with epilepsy (N = 20) (p = 0.01). In the overall sample, the hazard of seizure recurrence was increased by lifetime generalized anxiety disorder (3.0-fold) and by low collective efficacy (2.7-fold). In a second model, the hazard was increased by lifetime mood disorder (2.1-fold) and low collective efficacy (2.5-fold). Significance Markers of stress (i.e., low collective efficacy, lifetime mood disorder, and lifetime generalized anxiety disorder) were associated with an increased risk for seizure recurrence in adults with a single unprovoked seizure or newly diagnosed epilepsy. Stress-reducing interventions, such as mindfulness, may be a useful, safe, and inexpensive adjunctive treatment for epilepsy. [ABSTRACT FROM AUTHOR]

Objective Predictors of morbidity and mortality after status epilepticus ( SE) have been studied extensively in hospital- and population-based cohorts. However, little attention has been directed toward SE recurrence after an incident episode. We investigated clinical and demographic characteristics of patients presenting SE recurrence and its specific prognostic role. Methods In this observational cohort study, we screened our prospective registry of consecutive adults with SE between April 2006 and February 2014. Demographic and clinical data were compared between incident and recurrent SE episodes; risk of SE recurrence was assessed through survival analysis, and the prognostic role of SE recurrence with multivariable logistic regressions. Results Of the incident cohort (509 patients), 68 (13%) experienced recurrent SE. The cumulative recurrence rate over 4 years was 32%. Recurrence risk was significantly reduced after an acute SE etiology ( hazard ratio [HR] 0.5, 95% confidence interval [CI] 0.31-0.82; p = 0.005), and was borderline increased in women ( HR 1.59, 95% CI 0.97-2.65; p = 0.06). Although recurrent SE episodes showed lower morbidity and mortality, prognosis was independently related to Status Epilepticus Severity Score (STESS) and potentially fatal etiology, but not to SE recurrence. Significance This study provides class III evidence that SE recurrence involves a significant proportion of patients, and that recurrence risk is independently associated with chronic etiology and to a lesser extent with female gender. However, contrary to underlying cause and SE severity, SE recurrence per se does not independently correlate with outcome. Early identification of patients at higher risk of SE recurrence may influence their management during follow-up. [ABSTRACT FROM AUTHOR]

Objective To describe the etiologies, clinical presentations, outcomes, and predictors of postencephalitic epilepsy ( PE) in a large series of adult patients with acute encephalitis. Methods We conducted a retrospective review of patients diagnosed with acute encephalitis at Mayo Clinic, Rochester, between January 2000 and December 2012. The patients were categorized into two groups based on the presence or absence of PE at last follow-up. Multivariate logistic regression analysis was used to analyze factors associated with PE. Results We identified 198 consecutive patients (100 [50.5%] male and 98 [49.5%] female) with a median age of 58 years (range 41.8-69). Etiologies included viral infection (n = 95, 48%), autoimmune (n = 44, 22%), and unknown/others (n = 59, 30%). During hospitalization, seizures were seen in patients with autoimmune encephalitis (n = 24, 54.5%), unknown/others (n = 20, 33.9%), and viral encephalitis (n = 23, 24.2%). Interictal epileptiform discharges on electroencephalography (EEG) were present in 34 (54%), whereas periodic lateralized epileptiform discharges (PLEDs) and generalized periodic discharges (GPDs) were seen in 14 (41.2%) and 2 (5.9%) patients. Forty-six patients with seizures (70.8%) had fluid-attenuated inversion recovery ( FLAIR)/T2 abnormalities, 20 (31.3%) diffusion abnormalities, and 43 (66.2%) cortical involvement. Good outcome at discharge among patients with seizures was seen in 8/23 patients with viral etiology (34.8%), 10/24 patients with autoimmune encephalitis (45.5%), and 12/20 patients with unknown cause (60%). PE was present in 43 patients (29.9%). On multivariate regression analysis, the factors associated with PE were generalized seizures during hospitalization (p = 0.03), focal seizures (p ≤ 0.001), and the presence of FLAIR/T2 abnormalities on brain magnetic resonance imaging ( MRI) (p = 0.003). Significance The presence of seizures during hospitalization and an abnormal brain MRI are the strongest predictors of the development of PE. The etiology of encephalitis, presence of focal neurologic deficits, and interictal EEG abnormalities did not influence the development of PE. [ABSTRACT FROM AUTHOR]

Patients with epilepsy may have diffuse, serious brain disorders including genetically determined, multilobar malformations, traumatic brain injury, encephalitis and meningitis, and the many causes of Lennox-Gastaut syndrome. Transition to adult care needs to consider concomitant intellectual disability, refractory epilepsy, underlying cause, and other nonneurologic but significant problems, especially for genetic etiologies. Adult epilepsy care coupled with dedicated primary/family care is essential. A multidisciplinary setting may be optimal to address the many issues of clinical care, decision making, custody, and ongoing supervision. [ABSTRACT FROM AUTHOR]

Purpose Epilepsy is common in sub- Saharan Africa ( SSA), but the clinical features and consequences are poorly characterized. Most studies are hospital-based, and few studies have compared different ecological sites in SSA. We described active convulsive epilepsy ( ACE) identified in cross-sectional community-based surveys in SSA, to understand the proximate causes, features, and consequences. Methods We performed a detailed clinical and neurophysiologic description of ACE cases identified from a community survey of 584,586 people using medical history, neurologic examination, and electroencephalography ( EEG) data from five sites in Africa: South Africa; Tanzania; Uganda; Kenya; and Ghana. The cases were examined by clinicians to discover risk factors, clinical features, and consequences of epilepsy. We used logistic regression to determine the epilepsy factors associated with medical comorbidities. Key Findings Half (51%) of the 2,170 people with ACE were children and 69% of seizures began in childhood. Focal features ( EEG, seizure types, and neurologic deficits) were present in 58% of ACE cases, and these varied significantly with site. Status epilepticus occurred in 25% of people with ACE. Only 36% received antiepileptic drugs (phenobarbital was the most common drug [95%]), and the proportion varied significantly with the site. Proximate causes of ACE were adverse perinatal events (11%) for onset of seizures before 18 years; and acute encephalopathy (10%) and head injury prior to seizure onset (3%). Important comorbidities were malnutrition (15%), cognitive impairment (23%), and neurologic deficits (15%). The consequences of ACE were burns (16%), head injuries (postseizure) (1%), lack of education (43%), and being unmarried (67%) or unemployed (57%) in adults, all significantly more common than in those without epilepsy. Significance There were significant differences in the comorbidities across sites. Focal features are common in ACE, suggesting identifiable and preventable causes. Malnutrition and cognitive and neurologic deficits are common in people with ACE and should be integrated into the management of epilepsy in this region. Consequences of epilepsy such as burns, lack of education, poor marriage prospects, and unemployment need to be addressed. [ABSTRACT FROM AUTHOR]

Purpose Lennox- Gastaut syndrome ( LGS) is a severe epileptic disorder with characteristic electroclinical features but diverse etiologies. The shared electroclinical characteristics suggest that common cerebral networks are involved in generating seizures. We sought to reveal these networks by comparing ictal and interictal single-photon emission computed tomography ( SPECT). Methods We identified 10 ictal-interictal SPECT pairs from seven patients with LGS (median age 11 years; range 1-38) who were studied during video electroencephalography ( EEG)-confirmed tonic seizures. We performed a voxel-wise comparison of ictal and interictal SPECT studies across the group. The evolution of blood flow changes was explored by examining early and late injection groups. Key Findings Median duration of tonic seizures was 10 s (range 6-29 s), and injection latency from seizure offset was −8 to 48 s. In the early injection group (<10 s; three studies), there was hyperperfusion over pons and cerebellar hemispheres (p < 0.05 cluster corrected family wise error), and hypoperfusion bilaterally over the pericentral region, with a trend toward hyperperfusion over bilateral superior and middle frontal gyri, and lateral parietal cortex. In the late injection group, there was hyperperfusion over midline and lateral cerebellar regions, with hypoperfusion widely over bilateral frontal regions. Significance This study suggests that the tonic seizures of LGS result from activity in a network, containing bilateral frontal and parietal association areas and the pons. We postulate that tonic seizures recruit the corticoreticular system, which connects frontal attentional areas to the pontine reticular formation, and is normally responsible for postural tone and orienting behavior. [ABSTRACT FROM AUTHOR]

Purpose Lennox- Gastaut syndrome ( LGS) is a devastating childhood-onset epilepsy syndrome. The cause is unknown in 25% of cases. Little has been described about the specific clinical or electroencephalography ( EEG) features of LGS of unknown or genetic cause ( LGSu). The Epilepsy Phenome/ Genome Project ( EPGP) aims to characterize LGSu by phenotypic analysis of patients with LGSu and their parents. Methods One hundred thirty-five patients with LGS with no known etiology and their parents were enrolled from 19 EPGP centers in the United States and Australia. Clinical data from medical records, standardized questionnaires, imaging, and EEG were collected with use of online informatics systems developed for EPGP. Key Findings LGSu in the EPGP cohort had a broad range of onset of epilepsy from 1 to 13 years, was male predominant (p < 0.0002), and was associated with normal development prior to seizure onset in 59.2% of patients. Despite the diagnosis, almost half of the adult patients with LGSu completed secondary school. Parents were cognitively normal. All subjects had EEG recordings with generalized epileptiform abnormalities with a spike wave frequency range of 1-5 Hz (median 2 Hz), whereas 8.1% of subjects had EEG studies with a normal posterior dominant rhythm. Almost 12% of patients evolved from West syndrome. Significance LGSu has distinctive characteristics including a broad age range of onset, male predominance, and often normal development prior to the onset of seizures. Cognitive achievements such as completion of secondary school were possible in half of adult patients. Our phenotypic description of LGSu coupled with future genetic studies will advance our understanding of this epilepsy syndrome. [ABSTRACT FROM AUTHOR]

Summary Determining a prognosis for functional recovery after prolonged status epilepticus can be difficult. Prior case studies have shown that despite seizure control, functional outcomes are typically poor unless a reversible cause is identified. Herein we present a case of idiopathic status epilepticus with a surprisingly good outcome after a 125-day drug-induced coma. [ABSTRACT FROM AUTHOR]

The article presents abstracts on medical topics including "Does Etiology of Epilepsy Predispose to Status Epilepticus?," by G. Leuf and colleagues, "Treatment of Status Epilepticus in a Large Community Hospital," by C. Kellinghaus and S. Berning, and "Mood Change and Mutism in the Elderly and Status Epilepticus," by C. N. Lo and Y. L. Liang.

Frontal lobe epilepsy (FLE) is considered the second most common type of the localization-related epilepsies of childhood. Still, the etiology of FLE in children, its impact on cognitive functioning and behavior, as well as the response to antiepileptic drug treatment in children has not been sufficiently studied. This review focuses on these aspects of FLE in childhood, and reveals that FLE in childhood is most often cryptogenic, and impacts on a broad range of cognitive functions. The nature and severity of cognitive deficits are highly variable, although impaired attention and executive functions are most frequent. Young age at seizure onset is the only potential risk factor for poor cognitive outcome that has been consistently reported. The behavioral disturbances associated with FLE are also highly variable, although attention deficit/hyperactivity disorder seems most frequent. In 40% of children with FLE satisfactory seizure control could not be achieved. This is a higher percentage than reported for the general population of children with epilepsy. Therefore, pediatric FLE, even if cryptogenic in nature, is frequently complicated by impairment of cognitive function, behavioral disturbances, and therapy-resistance. Given the impact of these complications, there is a need for studies of the etiology of frontal lobe epilepsy-associated cognitive and behavioral disturbances, as well as pharmacotherapy-resistance. [ABSTRACT FROM AUTHOR]

The aim of the study was to evaluate the outcome of status epilepticus (SE) in children and to define predictors for morbidity, mortality, and SE recurrence. The study included 302 children (age 2 months to less than 18 years; mean age ± SD 4.7 ± 4.2 years) with 489 episodes of SE. Etiology, treatment, and clinical and electroencephalography (EEG) features of SE and their impact on the outcome were analyzed. The outcome was classified into three categories: unchanged neurologic status, neurologic consequences, and lethal outcome. Univariate and multivariate Cox hazard regression analyses were used to define predictors of mortality, morbidity, and SE recurrence. Neurologic status was unchanged in 235 children (77.8%) and neurologic consequences occurred in 39 patients (12.9%); case-fatality ratio was 9.3% and recurrence rate was 21%. Mortality was related to progressive encephalopathy, preexisting neurologic abnormalities, specific EEG findings, and generalized convulsive type of SE. Neurologic consequences were associated with younger age, progressive encephalopathy, duration of SE >24 h, prior epilepsy, and specific EEG findings. Multivariate analyses showed that etiology of SE and prior neurologic abnormalities were independent predictors of mortality, whereas younger age, etiology, and very long duration of SE were predictors of morbidity. Outcome of SE in children is favorable in most of the cases, but mortality and morbidity rates are still high. Etiology and prior neurologic abnormalities were the main predictors of mortality, whereas the main predictor of morbidity was underlying etiology. [ABSTRACT FROM AUTHOR]

Epidemiologic studies of epilepsy from developing countries are scarce. As part of a population-based epidemiologic project in Vietnam, EPIBAVI, we studied the incidence and etiology of epilepsy in people in a representative rural region of the country. Two identical field surveys were carried out 3 years apart (January to December 2005, and June to December 2008) in the same population of the Bavi District in Vietnam. On both occasions, close to 50,000 members of approximately 13,000 households were screened using a questionnaire for epilepsy. A clinical examination of all screened positive was performed by a neurologist to verify the epilepsy diagnosis, and all incident cases were offered EEG and a CT scan. In the first survey 2.8% screened positive according to the questionnaire. Of these, 19 had epilepsy onset within 1 year preceding the screening, yielding an incidence rate of 40.2 per 100,000 [95% confidence interval (CI) 22.1-58.3]. In the second survey 1.8% were screened positive, and 21 of these had epilepsy onset within 1 year preceding the screening, giving an incidence rate of 42.9 per 100,000 (95% CI 24.5-61.2). The age-adjusted incidence was 44.8 per 100,000 (95% CI 30.6-59.0). The incidence was higher in those younger than 16 years, among people with lower education, and among people with lower income. CT scan was performed in 29 cases and only two cases were found with some abnormalities. The incidence rate of epilepsy in rural Vietnam in our study was lower than in developing countries in Latin America and Africa and similar to rates in Europe and North America. [ABSTRACT FROM AUTHOR]

Outcome for patients with status epilepticus (SE) depends strongly on etiology. Duration of SE is also predictive, at least in the first 2 h, but beyond this it is unclear that duration of SE influences outcome significantly. We sought to determine the influence of duration of SE on outcome in patients with prolonged SE, and to compare this influence with that of other factors. We reviewed the clinical course and outcome of 119 patients with SE, diagnosed by both clinical manifestations and electroencephalography (EEG) evidence. Using univariate and multivariate analyses, we sought predictors of outcome (survival vs. death or vegetative state) among age, etiology (epilepsy, anoxia or severe hypoxia, or other), presence of earlier epilepsy, multiple medical problems, presentation in coma, and type of SE (focal or generalized). Median duration of SE was 48 h. Survival was greater with a shorter duration, especially when <10 h (69% vs. 31% for longer duration; p < 0.05). Epilepsy as the etiology, and an earlier diagnosis of epilepsy offered a favorable prognosis (p < 0.01), but only the former on multivariate analysis. Coma and SE caused by anoxia/hypoxia were unfavorable factors. Once corrected for etiology, presentation in coma, and type of SE (focal or generalized), duration of SE did not have a significant effect on outcome. Overall mortality was high, 65%, but 10 patients survived SE lasting over 3.5 days. A duration of <10 h was associated with better outcome in SE, but this was not significant once etiology, presentation in coma, and type of SE were accounted for. Etiology of SE is still the primary determinant of outcome. Unless it follows anoxia, prolonged SE should not be considered a hopeless condition. [ABSTRACT FROM AUTHOR]

In its first description (1989), Panayiotopoulos syndrome was defined as an idiopathic epilepsy syndrome with an excellent prognosis, characterized by a clinical ictal triad of nocturnal seizures, tonic deviation of the eyes, and vomiting. The electroencephalographic and clinical features of this condition were highly suggestive of occipital lobe involvement. Subsequently, the concept of this benign age-related focal epilepsy has been expanded over the years, including a wider and larger spectrum of seizure manifestations far beyond the occipital manifestations, and for which the eponym of Panayiotopoulos syndrome (PS) has been adopted. However, many theoretical and practical points, including diagnostic, genetic, and pathophysiologic issues remain still unresolved for PS. [ABSTRACT FROM AUTHOR]

Conflicting evidence about impairment of attention systems and the absence of a working model of attention has contributed to lack of clarity about comorbidity of attention problems in rolandic epilepsy (RE). Impairments in distributed attention systems may inform a disease model for RE, as well as direct interventions. We used a systematic review of the relevant literature published in English from 1990 to 2006 to evaluate impairment in attention in RE. The Mirsky and Posner models of attention were evaluated for applicability, and studies were reviewed for design, instrumentation, and congruence with the Posner model of attention. Fourteen studies were identified: seven using a cross-sectional design (six active EEG abnormalities; one EEG remission) and seven longitudinal studies (abnormal EEGs and follow-up until normalized). According to the Posner model of attention, 12 studies employed measures that tapped the alerting network, 11 studies the orienting network, and eight the executive network. Nearly all controlled studies demonstrated impairments in all tested attention networks. In contrast, uncontrolled studies uniformly did not demonstrate impairments. Follow-up studies demonstrated complete or near complete resolution of attention impairments. The weight of evidence, defined as the majority of studies evaluated, suggests that all three attention systems are impaired in children with active centrotemporal spikes (CTS), implying a more widespread functional cortical disturbance in RE than previously held. These impairments resolve upon EEG remission, suggesting a common pathological basis to the autosomal dominant CTS trait. Sources of methodological variation are discussed with recommendations for future investigations. [ABSTRACT FROM AUTHOR]

The major vault protein (MVP) may play a role in multi-drug resistance (MDR). Researchers investigated the expression and cellular localization of MVP in gangliogliomas (GGs), which are increasingly recognized causes of chronic pharmacoresistant epilepsy. Peritumoral and control brain tissues, were examined for the cellular distribution pattern of MVP with immunocytochemistry. In normal brain, MVP expression was below detection in neuronal cells, and only low immunoreactivity levels were detected in blood vessels. MVP expression was observed in the neuronal component of 30 of 30 GGs and in a population of tumor glial cells. The increased expression of MVP in GGs is an example of an MDR-related protein that is upregulated in patients with refractory epilepsy.

We report resolution of an epileptic encephalopathy by administration of transdermal nicotine patches in an adolescent with severe nonlesional refractory frontal lobe epilepsy. The 18.5-year-old female patient had refractory epilepsy from the age of 11. Recurrent electroencephalography (EEG) recordings showed mostly generalized activity, albeit with right frontal predominance. Almost all antiepileptic medications failed to provide benefit. She developed an encephalopathic state with cognitive decline. The nonlesional frontal lobe epilepsy and a family history of a cousin with nocturnal epilepsy with frontal origin suggested genetic etiology. Transdermal nicotine patches brought complete resolution of the seizures, normalization of the EEG, and a significant improvement in her thinking process and speech organization. Sequencing of the CHRNB2 and CHRNA4 genes did not detect a mutation. Transdermal nicotine patches should be considered in severe pharmacoresistant frontal lobe epilepsy. [ABSTRACT FROM AUTHOR]

The article offers the author's insights on the use of the definition of epilepsy as a syndrome or disease as a basis for a classification system. The author states that the identification of epilepsy syndrome and the associated etiology has an important clinical relevance. He says that etiology alone does not provide a specific picture of epilepsy though its identification is important. He also proposes a two-tiered classification system for the disease.

The article offers the author's insights regarding the classification of epilepsies. It states that classification system allows a sense of knowing and recognition that follows several criteria including communication, education and utility. It highlights the importance of knowing the causes and secondary consequences of seizures to have a classification of the epilepsies mechanism. It also suggests renewal of classification system towards higher progress and advancement.

A letter to the editor is presented in response to the article about the postencephalitic epilepsy in children and adults.

The article provides information on burst-suppression. Burst-suppression (BS) is defined as an electroencephalography (EEG) pattern comprising of alternative periods of slow waves of high and low amplitude. It is generally affiliated to comatose states of various etiologies. It is informed that the pseudo-rhythmicity of the BS pattern is dictated by the intensity of extracellular Ca2+ depletion and the ability of neurons to revive this concentration.

In this article, the authors present their views about the Landau-Kleffner syndrome (LKS). According to the authors, regarding the epilepsy component of LKS, a major constituent of the syndrome is the tremendous exaggeration of paroxysmal brain activity during sleep. They further say that no evidence has been reported so far regarding any etiologic relationship between LKS and electroencephalographically similar conditions.