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The goal of this paper is to provide updated diagnostic criteria for the epilepsy syndromes that have a variable age of onset, based on expert consensus of the International League Against Epilepsy Nosology and Definitions Taskforce (2017–2021). We use language consistent with current accepted epilepsy and seizure classifications and incorporate knowledge from advances in genetics, electroencephalography, and imaging. Our aim in delineating the epilepsy syndromes that present at a variable age is to aid diagnosis and to guide investigations for etiology and treatments for these patients. [ABSTRACT FROM AUTHOR]

Summary: Objective: To investigate the effects of sodium channel–blocking antiepileptic drugs (AEDs) on functional magnetic resonance imaging (fMRI) language network activations in patients with focal epilepsy. Methods: In a retrospective study, we identified patients who were treated at the time of language fMRI scanning with either carbamazepine (CBZ; n = 42) or lamotrigine (LTG; n = 42), but not another sodium channel–blocking AED. We propensity‐matched 42 patients taking levetiracetam (LEV) as “patient‐controls” and included further 42 age‐ and gender‐matched healthy controls. After controlling for age, age at onset of epilepsy, gender, and antiepileptic comedications, we compared verbal fluency fMRI activations between groups and out‐of‐scanner psychometric measures of verbal fluency. Results: Patients on CBZ performed less well on a verbal fluency tests than those taking LTG or LEV. Compared to either LEV‐treated patients or controls, patients taking CBZ showed decreased activations in left inferior frontal gyrus and patients on LTG showed abnormal deactivations in frontal and parietal default mode areas. All patient groups showed fewer activations in the putamen bilaterally compared to controls. In a post hoc analysis, out‐of‐scanner fluency scores correlated positively with left putamen activation. Significance: Our study provides evidence of AED effects on the functional neuroanatomy of language, which might explain subtle language deficits in patients taking otherwise well‐tolerated sodium channel–blocking agents. Patients on CBZ showed dysfunctional frontal activation and more pronounced impairment of performance than patients taking LTG, which was associated only with failure to deactivate task‐negative networks. As previously shown for working memory, LEV treatment did not affect functional language networks. [ABSTRACT FROM AUTHOR]

The article presents abstracts on medical topics including prediction on functional outcome of epileptics, factors associated with response in patients randomized to placebo in adjunct therapy and risk of mortality in epilepsy after neonatal seizure.

Purpose Clinical, experimental, and neuroimaging data all indicate that the thalamus is involved in the network of changes associated with temporal lobe epilepsy ( TLE), particularly in association with hippocampal sclerosis ( HS), with potential roles in seizure initiation and propagation. Pathologic changes in the thalamus may be a result of an initial insult, ongoing seizures, or retrograde degeneration through reciprocal connections between thalamic and limbic regions. Our aim was to carry out a neuropathologic analysis of the thalamus in a postmortem ( PM) epilepsy series, to assess the distribution, severity, and nature of pathologic changes and its association with HS. Methods Twenty-four epilepsy PM cases (age range 25-87 years) and eight controls (age range 38-85 years) were studied. HS was classified as unilateral ( UHS, 11 cases), bilateral ( BHS, 4 cases) or absent (No- HS, 9 cases). Samples from the left and right sides of the thalamus were stained with cresyl violet ( CV), and for glial firbillary acidic protein (GFAP) and synaptophysin. Using image analysis, neuronal densities ( NDs) or field fraction staining values ( GFAP, synaptophysin) were measured in four thalamic nuclei: anteroventral nucleus ( AV), lateral dorsal nucleus ( LD), mediodorsal nucleus ( MD), and ventrolateral nucleus ( VL). The results were compared within and between cases. Key Findings The severity, nature, and distribution of thalamic pathology varied between cases. A pattern that emerged was a preferential involvement of the MD in UHS cases with a reduction in mean ND ipsilateral to the side of HS (p = 0.05). In UHS cases, greater field fraction values for GFAP and lower values for synaptophysin and ND were seen in the majority of cases in the MD ipsilateral to the side of sclerosis compared to other thalamic nuclei. In addition, differences in the mean ND between classical HS, atypical HS, and No- HS cases were noted in the ipsilateral MD (p < 0.05), with lower values observed in HS. Significance Our study demonstrates that stereotypical pathologic changes, as seen in HS, are not clearly defined in the thalamus. This may be partly explained by the heterogeneity of our PM study group. With quantitation, there is some evidence for preferential involvement of the MD, suggesting a potential role in TLE, which requires further investigation. [ABSTRACT FROM AUTHOR]

Objective Patients with juvenile myoclonic epilepsy ( JME) often present with risk-taking behavior, suggestive of frontal lobe dysfunction. Recent studies confirm functional and microstructural changes within the frontal lobes in JME. This study aimed at characterizing decision-making behavior in JME and its neuronal correlates using functional magnetic resonance imaging (fMRI). Methods We investigated impulsivity in 21 JME patients and 11 controls using the Iowa Gambling Task ( IGT), which measures decision making under ambiguity. Performance on the IGT was correlated with activation patterns during an fMRI working memory task. Results Both patients and controls learned throughout the task. Post hoc analysis revealed a greater proportion of patients with seizures than seizure-free patients having difficulties in advantageous decision making, but no difference in performance between seizure-free patients and controls. Functional imaging of working memory networks showed that overall poor IGT performance was associated with an increased activation in the dorsolateral prefrontal cortex (DLPFC) in JME patients. Impaired learning during the task and ongoing seizures were associated with bilateral medial prefrontal cortex ( PFC) and presupplementary motor area, right superior frontal gyrus, and left DLPFC activation. Significance Our study provides evidence that patients with JME and ongoing seizures learn significantly less from previous experience. Interictal dysfunction within 'normal' working memory networks, specifically, within the DLPFC and medial PFC structures, may affect their ability to learn. [ABSTRACT FROM AUTHOR]

Purpose Temporal lobe epilepsy ( TLE) has been considered to impair long-term memory, whilst not affecting working memory, but recent evidence suggests that working memory is compromised. Functional MRI ( fMRI) studies demonstrate that working memory involves a bilateral frontoparietal network the activation of which is disrupted in hippocampal sclerosis ( HS). A specific role of the hippocampus to deactivate during working memory has been proposed with this mechanism faulty in patients with HS. Structural correlates of disrupted working memory in HS have not been explored. Methods We studied 54 individuals with medically refractory TLE and unilateral HS (29 left) and 28 healthy controls. Subjects underwent 3 T structural MRI, a visuospatial n-back fMRI paradigm and diffusion tensor imaging ( DTI). Working memory capacity assessed by three span tasks (digit span backwards, gesture span, motor sequences) was combined with performance in the visuospatial paradigm to give a global working memory measure. Gray and white matter changes were investigated using voxel-based morphometry and voxel-based analysis of DTI, respectively. Key Findings Individuals with left or right HS performed less well than healthy controls on all measures of working memory. fMRI demonstrated a bilateral frontoparietal network during the working memory task with reduced activation of the right parietal lobe in both patient groups. In left HS, gray matter loss was seen in the ipsilateral hippocampus and parietal lobe, with maintenance of the gray matter volume of the contralateral parietal lobe associated with better performance. White matter integrity within the frontoparietal network, in particular the superior longitudinal fasciculus and cingulum, and the contralateral temporal lobe, was associated with working memory performance. In right HS, gray matter loss was also seen in the ipsilateral hippocampus and parietal lobe. Working memory performance correlated with the gray matter volume of both frontal lobes and white matter integrity within the frontoparietal network and contralateral temporal lobe. Significance Our data provide further evidence that working memory is disrupted in HS and impaired integrity of both gray and white matter is seen in functionally relevant areas. We suggest this forms the structural basis of the impairment of working memory, indicating widespread and functionally significant structural changes in patients with apparently isolated HS. [ABSTRACT FROM AUTHOR]

Purpose: Cognitive impairment is frequent in children with frontal lobe epilepsy (FLE), but its etiology is unknown. With functional magnetic resonance imaging (fMRI), we have explored the relationship between brain activation, functional connectivity, and cognitive functioning in a cohort of pediatric patients with FLE and healthy controls. Methods: Thirty-two children aged 8-13 years with FLE of unknown cause and 41 healthy age-matched controls underwent neuropsychological assessment and structural and functional brain MRI. We investigated to which extent brain regions activated in response to a working memory task and assessed functional connectivity between distant brain regions. Data of patients were compared to controls, and patients were grouped as cognitively impaired or unimpaired. Key Findings: Children with FLE showed a global decrease in functional brain connectivity compared to healthy controls, whereas brain activation patterns in children with FLE remained relatively intact. Children with FLE complicated by cognitive impairment typically showed a decrease in frontal lobe connectivity. This decreased frontal lobe connectivity comprised both connections within the frontal lobe as well as connections from the frontal lobe to the parietal lobe, temporal lobe, cerebellum, and basal ganglia. Significance: Decreased functional frontal lobe connectivity is associated with cognitive impairment in pediatric FLE. The importance of impairment of functional integrity within the frontal lobe network, as well as its connections to distant areas, provides new insights in the etiology of the broad-range cognitive impairments in children with FLE. [ABSTRACT FROM AUTHOR]

Several abstract related to neurology which are presented in the seminar "European Congress on Epileptology" in London, England including sytaxin binding protein mutation, syngap1 mutation screening and mutation in CDKL5 gene.

The dynamic aspects of epilepsy, in which seizures occur sporadically and are interspersed with periods of relatively normal brain function, present special challenges for neuroanatomical studies. Although numerous morphologic changes can be identified during the chronic period, the relationship of many of these changes to seizure generation and propagation remains unclear. Mossy fiber sprouting is an example of a frequently observed morphologic change for which a functional role in epilepsy continues to be debated. This review focuses on neuroanatomically identified changes that would support high levels of activity in reorganized mossy fibers and potentially associated granule cell activation. Early ultrastructural studies of reorganized mossy fiber terminals in human temporal lobe epilepsy tissue have identified morphologic substrates for highly efficacious excitatory connections among granule cells. If similar connections in animal models contribute to seizure activity, activation of granule cells would be expected. Increased labeling with two activity-related markers, Fos and phosphorylated extracellular signal-regulated kinase, has suggested increased activity of dentate granule cells at the time of spontaneous seizures in a mouse model of epilepsy. However, neuroanatomical support for a direct link between activation of reorganized mossy fiber terminals and increased granule cell activity remains elusive. As novel activity-related markers are developed, it may yet be possible to demonstrate such functional links and allow mapping of seizure activity throughout the brain. Relating patterns of neuronal activity during seizures to the underlying morphologic changes could provide important new insights into the basic mechanisms of epilepsy and seizure generation. [ABSTRACT FROM AUTHOR]

Summary Purpose: Neuropeptide Y (NPY) is an inhibitory neurotransmitter that suppresses focal and generalized seizures in animal models. In this study, we investigated the sites within the thalamocortical circuit that NPY acts to suppress seizures in genetic absence epilepsy rats from Strasbourg (GAERS). Methods: In conscious freely moving GAERS, NPY was administered via intracerebral microcannulae implanted bilaterally into one of the following regions: primary somatosensory cortex (S1), secondary somatosensory cortex (S2), the primary motor cortex (M1), caudal nucleus reticular thalamus (nRT), or ventrobasal thalamus (VB). Animals received vehicle and up to three doses of NPY, in a randomized order. Electroencephalography (EEG) recordings were carried out for 30 min prior to injection and 90 min after the injection of NPY or vehicle. Key Findings: Focal microinjections of NPY into the S2 cortex suppressed seizures in a dose-dependent manner, with the response being significantly different at the highest dose (1.5 m m) compared to vehicle for total time in seizures postinjection (7.2 ± 3.0% of saline, p < 0.01) and average number of seizures (9.4 ± 4.9% of saline, p < 0.05). In contrast NPY microinjections into the VB resulted in an aggravation of seizures. Significance: NPY produces contrasting effects on absence-like seizures in GAERS depending on the site of injection within the thalamocortical circuit. The S2 is the site at which NPY most potently acts to suppress absence-like seizures in GAERS, whereas seizure-aggravating effects are seen in the VB. These results provide further evidence to support the proposition that these electroclinically 'generalized' seizures are being driven by a topographically restricted region within the somatosensory cortex. [ABSTRACT FROM AUTHOR]

Reading epilepsy (RE) is an idiopathic reflex epilepsy syndrome characterized by perioral myoclonic jerks (PMJs) during reading associated with left-dominant frontotemporal spike-wave discharges (SWDs). To better understand the pathophysiology of this syndrome, we studied a 45-year-old patient using magnetic source imaging (MSI). The patient underwent two whole-head magnetoencephalography (MEG) recordings (Elekta Neuromag Oy) within 2 months while reading aloud. Forty-two SWDs associated with PMJs were recorded and averaged with respect to SWDs peak power. Epileptic discharges were then reconstructed using conventional equivalent current dipoles (ECDs) modeling, distributed sources sLORETA modeling, and beamformer approach. These methods identified two brain sources located in the left supplementary motor cortex (SMC) and the left primary sensorimotor face area (PSMFA). The spatiotemporal pattern of the sources was characterized by a cross-talk between these two brain regions, with an initial source in the left SMC. This MSI investigation suggests that RE-PMJs are associated with reading-induced activation of hyperexcitable neurons in the left SMC, followed by secondary propagation to the left PSMFA producing the myoclonus. [ABSTRACT FROM AUTHOR]

One possible cause for surgical failure following temporal lobectomy for the treatment of epilepsy due to classical hippocampal sclerosis (CHS) is the presence of more widespread cortical changes. Neocortical changes in CHS shown by quantitative neuroimaging studies may involve hippocampal projection pathways. Our aim was to quantitate neocortical pathology using a postmortem series of brains from patients with epilepsy and CHS. Sections from 13 cortical regions from both left and right hemispheres, including hippocampal projection pathways, were examined from nine epilepsy patients with unilateral CHS (4), bilateral CHS (2), non-CHS (3), and non-epilepsy controls (4). Using GFAP, CD68, and NPY immunohistochemistry as markers of acquired neocortical pathology, quantitative analysis of the staining fractions in the cortex and white matter was carried out. Higher staining fractions were observed for all markers in both cortex and white matter in CHS patients, which was significantly different for CD68 and NPY compared to controls (p < 0.05) but not to non-CHS epilepsy cases. There was no significant difference between staining fractions in left and right hemispheres for unilateral CHS cases. Regional analysis showed preferential gliosis and microgliosis of temporal poles, frontal poles, and orbitofrontal cortex in epilepsy cases. This study supports acquired neocortical pathology in epilepsy patients both with and without CHS. Cortical pathology does not show lateralization to the side of CHS. Preferential involvement of the temporal and frontal poles may relate to other factors, such as cortical injury associated with seizures, rather than involvement through hippocampal pathways. [ABSTRACT FROM AUTHOR]

Reading epilepsy is a distinct form of epilepsy in which all or almost all seizures are precipitated by reading. Seizures typically show orofacial or jaw myoclonus. Nevertheless, reading epilepsy is not homogenous and its classification is unclear. We report a patient with reading-induced prolonged left temporal seizures, presenting clinically as dyslexia. [ABSTRACT FROM AUTHOR]

Purpose: Around one-third of patients undergoing temporal lobe surgery for the treatment of intractable temporal lobe epilepsy with hippocampal sclerosis (HS) fail to become seizure-free. Identifying reliable predictors of poor surgical outcome would be helpful in management. Atypical patterns of HS may be associated with poorer outcomes. Our aim was to identify atypical HS cases from a large surgical series and to correlate pathology with clinical and outcome data. Methods: Quantitative neuropathologic evaluation on 165 hippocampal surgical specimens and 21 control hippocampi was carried out on NeuN-stained sections. Neuronal densities (NDs) were measured in CA4, CA3, CA2, and CA1 subfields. The severity of granule cell dispersion (GCD) was assessed. Results: Comparison with control ND values identified the following patterns based on the severity and distribution of neuronal loss: classical HS (CHS; n = 60) and total HS (THS; n = 39). Atypical patterns were present in 30% of cases, including end-folium sclerosis (EFS; n = 5), CA1 predominant pattern (CA1p; n = 9), and indeterminate HS (IHS, n = 35). No HS was noted in 17 cases. Poorest outcomes were noted for no-HS, and CA1p groups with 33–44% International League Against Epilepsy (ILAE) class I at up to 2 years follow-up compared to 69% for CHS (p < 0.05). GCD associated with HS type (p < 0.01), but not with outcome. Conclusions: These findings support the identification and delineation of atypical patterns of HS using quantitative methods. Atypical patterns may represent distinct clinicopathologic subtypes and may have predictive value following epilepsy surgery. [ABSTRACT FROM AUTHOR]

Idiopathic partial reading epilepsy (RE) is a rare syndrome. We report the clinical and electroencephalographic characteristics of two right-handed patients with the following: reading-induced independent bilateral temporal lobe seizures, accompanied by alexia in left (dominant) sided seizures recorded on video-EEG (electroencephalography); subclinical activation over left posterior temporal and occipital electrodes during reading; no spontaneous seizure and no other trigger than reading; onset in adolescence; and history of varying resistance to treatment. Bilateral independent temporal lobe reflex seizures are part of the clinical spectrum of RE. It may result from hyperexcitability of bilateral cortical networks involved in the early steps of the reading process. [ABSTRACT FROM AUTHOR]

The article presents abstracts on medical topics which include the effectiveness of vagus nerve stimulation to patients with medically-refractory epilepsy, importance of magnetic resonance imaging (MRI) to detect patients with tuberous sclerosis complex (TSC) and surgical management of intractable temporal lobe epilepsy.

The article presents abstracts on the studies presented at the 28th International Epilepsy Congress which was held in Budapest, Hungary including the Serbian version of the quality-of-life in epilepsy inventory (QUOLIE-31), the patterns of psychiatric illness in juvenile myoclonic epilepsy, and the alterations of cognitive function and relative factors in epilepsy.

To characterize the spatial relationship between activations related to language-induced seizure activity, language processing, and motor control in patients with reading epilepsy. We recorded and simultaneously monitored several physiological parameters [voice-recording, electromyography (EMG), electrocardiography (ECG), electroencephalography (EEG)] during blood oxygen level-dependent (BOLD) functional magnetic resonance imaging (fMRI) in nine patients with reading epilepsy. Individually tailored language paradigms were used to induce and record habitual seizures inside the MRI scanner. Voxel-based morphometry (VBM) was used for structural brain analysis. Reading-induced seizures occurred in six out of nine patients. One patient experienced abundant orofacial reflex myocloni during silent reading in association with bilateral frontal or generalized epileptiform discharges. In a further five patients, symptoms were only elicited while reading aloud with self-indicated events. Consistent activation patterns in response to reading-induced myoclonic seizures were observed within left motor and premotor areas in five of these six patients, in the left striatum (n = 4), in mesiotemporal/limbic areas (n = 4), in Brodmann area 47 (n = 3), and thalamus (n = 2). These BOLD activations were overlapping or adjacent to areas physiologically activated during language and facial motor tasks. No subtle structural abnormalities common to all patients were identified using VBM, but one patient had a left temporal ischemic lesion. Based on the findings, we hypothesize that reflex seizures occur in reading epilepsy when a critical mass of neurons are activated through a provoking stimulus within corticoreticular and corticocortical circuitry subserving normal functions. [ABSTRACT FROM AUTHOR]

Saturday, December 6, 2008 Poster Session 1 1:00 p.m.-6:00 p.m. Clinical Neurophysiology Nicholas S. Abend*,†, Dennis Dlugos*,†, S. Herman†, A. Topjian‡,§, M. Donnelly*, R. Ichord*,†, M. Helfaer‡,§, V. Nadkarni‡,§ and R. Clancy*,† *Neurology, The Children's Hospital of Philadelphia, Philadelphia, PA; †Neurology, The University of Pennsylvania School of Medicine, Philadelphia, PA; ‡Critical Care Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA and §Pediatrics, The University of Pennsylvania School of Medicine, Philadelphia, PA Rationale: The incidence and characteristics of acute seizures in children undergoing therapeutic hypothermia following pulseless cardiac arrest (CA) are unknown. Since a high proportion of seizures may be subclinical, diagnosis requires continuous electroencephalography (EEG). We hypothesized that (i) EEG seizures are common during therapeutic hypothermia following CA and (ii) the background patterns would evolve during hypothermia and re-warming, and (iii) the background abnormalities would be associated with survival and short-term outcome. Methods: As part of a feasibility study of therapeutic hypothermia in children, we performed a prospective, pilot, observational study of 10 consecutive children undergoing continuous EEG monitoring during epochs of hypothermia (24 hrs), re-warming (12–24 hours), and an additional 24 hrs. EEG seizures were identified and the background during each epoch was described in a standardized manner. A standardized neurological exam was performed on day 5–7 to provide a cerebral injury score (CIS), rated from normal to profoundly abnormal. Results: Ten patients were studied from March to December 2007 with a mean age 19.9 ± 17.5 mo (range 2.2–51.2 mo). EEG monitoring began within 4 hours of hypothermia initiation in all. Over 720 hours of EEG were reviewed. Electrographic seizures occurred in 5/10 (50%) patients and began during the second 12 hours of hypothermia in 3 and during re-warming in 2. Most seizures were subclinical and electrographically generalized. Eight patients with initially mild/moderate EEG background abnormalities (discontinuity, slowing, attenuation) improved during warming; 2 patients with initially severe background abnormalities (burst suppression) had EEG worsening (longer duration suppression and status epilepticus). Background abnormalities did not predict seizures. Of the two patients with severe background abnormalities, one survivor had a severely abnormal CIS and one died. Of the eight patients with initial mild/moderate background abnormalities, 2 survivors did not have a CIS, 3 had a mild/moderate CIS, and 2 had a severe CIS (1 died). Thus, 1 of 2 (50%) with severe background abnormalities died while 1 of 8 (13%) with mild/moderate background abnormalities died. Also, death or severe CIS occurred in 2 of 2 (100%) with severely abnormal EEG background but only 2 of 6 (33%) with mild/moderate background abnormalities. Conclusions: Seizures occurred in half of those undergoing therapeutic hypothermia after CA and were mostly subclinical and generalized. The EEG background may improve or worsen during hypothermia and re-warming, suggesting that the earliest EEG results may not be useful for prognostication. However, the trend suggests more severe abnormalities are associated with worse survival and short term outcome. Further study correlating continuous EEG background patterns with short- and long-term outcomes in children treated with therapeutic hypothermia is warranted. [ABSTRACT FROM AUTHOR]

As a result of the converging evidence from multiple large independent studies, Panayiotopoulos syndrome (PS) is now formally recognized as a distinct clinical entity within the spectrum of benign focal epilepsies of childhood. Clinically, PS is manifested by predominantly autonomic seizures and electrographically with multifocal interictal spikes, while the few published ictal recordings have documented onsets of variable lobar topography. These typical electroclinical features do not allow straightforward assignment to a distinctive cortical area, rendering the term “focal”—as we currently understand it—problematic. This is a critical review of the clinical and EEG features of PS, focusing on those characteristics that may shed some light on its so far elusive pathophysiology. We also explore its electroclinical similarities to other idiopathic “focal” epilepsies and its differences to symptomatic focal epilepsies that may also manifest with autonomic ictal symptoms and signs. This methodology allows the formation of a rational hypothesis on the pathophysiology of PS that seems to be emerging as a good model for the so-called “system” (nonsymptomatic) epilepsies, with potentially important taxonomic implications. [ABSTRACT FROM AUTHOR]

Purpose: To clarify the source localization of epileptiform activity by using magnetoencephalography (MEG) in patients with graphogenic epilepsy. Methods: MEG and simultaneous EEG were recorded with a 204-channel whole-head MEG system in two patients with graphogenic epilepsy. During the MEG recordings, the patients performed a set of tasks comprising mental arithmetic calculation, speaking, moving the right arm in a manner resembling writing, writing, and thinking of writing. Equivalent current dipoles (ECD) were calculated for epileptiform discharges on MEG by using a single-dipole model. The ECD were superimposed on the magnetic resonance images of the patients. Results: The task of writing provoked seizures, in which both patients jerked the right arms. Thinking of writing also induced these seizures. In both patients, EEG associated with the seizures showed bursts of spike-and-slow-wave complexes predominantly in the centroparietal region. MEG also showed epileptiform discharges corresponding to the EEG bursts. ECDs obtained from the discharges were clustered in the left centroparietal area. Conclusions: Thinking of writing was a trigger for the seizures, as well as the task of writing. The source of the epileptiform discharge associated with the seizures was localized in the unilateral centroparietal area. The findings suggest that the centroparietal region plays an important role in the pathophysiology underlying these two graphogenic epilepsy cases. [ABSTRACT FROM AUTHOR]

Purpose: Perioral reflex myoclonias (PORM) are obvious, frequent, but often unobserved focal seizures in different epileptic syndromes and the leading seizure type in reading epilepsy. PORMs remain often undiagnosed because the patients are not aware that these are epileptic seizures and fail to report them. Their semiology is not fundamentally different in various epileptic syndromes. Methods: We studied the frequency of PORM in patients with juvenile myoclonic epilepsy (JME) compared with patients with focal epilepsies. Twenty-five patients with JME were investigated with a standardized neuropsychological test program and compared with 25 matched patients with focal epilepsies. Statistical significance was calculated by using Fisher's exact test. Results: We found significant differences between the groups regarding both frequency of PORM and activation of epileptic discharges. These observations seem to indicate that PORM, like praxis-induced seizures, are typical traits in JME. Conclusions: PORM are more frequent in JME compared with focal epilepsies. The distinction between focal and generalized epileptic ictogenesis may be less clear than is traditionally believed. [ABSTRACT FROM AUTHOR]

Purpose: Language-induced epilepsy involves seizure precipitation by speaking, reading, and writing. Seizures are similar to those of reading epilepsy (RE). The nosologic position of language-induced epilepsy is not clear. We performed a clinical and neurophysiological study in a multigenerational family with the association of idiopathic generalized epilepsy (IGE) with ictal stuttering as a manifestation of reflex language-induced epilepsy. Methods: Nine members on three generations were studied. All patients underwent video-polygraphic EEG recordings (awake and during sleep). A standardized protocol was applied to test the effect of language and non–language-related tasks. Results: Six patients presented language-induced jaw jerking that mimicked stuttering and corresponded to focal myoclonus involving facial muscles. This was associated with an IGE phenotype in four of these patients. Focal EEG spikes were found in all six patients by visual analysis and/or back-averaging techniques. The focal spikes were either asymptomatic (when followed by a slow wave) or symptomatic of facial myoclonia (when isolated). Levetiracetam, used as add-on or monotherapy in four patients, suppressed ictal stuttering. One additional case only had a phenotype of IGE without focal features. Conclusions: This family study demonstrates the phenotypic heterogeneity of the association of IGE phenotype with ictal stuttering (language-related reflex seizure). Our data suggest that this particular form of reflex epilepsy related to language has more similarities with generalized epilepsies than with focal ones. Neurophysiological investigations should be performed more systematically in patients with acquired stuttering, especially if there is family history of IGE. [ABSTRACT FROM AUTHOR]

This article concentrates on the role of electroencephalograms (EEGs) in the diagnosis and management of patients with idiopathic generalized epilepsies (IGEs). We review the morphologic and behavioral characteristics of the interictal and ictal EEG markers of IGE that should guide recording strategies to augment its diagnostic yield, and we attempt to delineate those particular features that may be relevant to different IGE syndromes. We also explore the electrographic boundaries between IGEs and cryptogenic/symptomatic generalized and focal epilepsies, and focal/secondary generalized epilepsies, with particular relevance to the phenomena of focal abnormalities and secondary bilateral synchrony, commenting on possible diagnostic pitfalls and areas of uncertainty. [ABSTRACT FROM AUTHOR]

The article presents abstracts of non-AED/non-surgical treatments for adult patients. They include "Chronic Measurement of Increased Epileptiform Activity During Menses Using the Responsive Neurostimulator System (RNS) in a Patient With Catamenial Seizures," "Effect of Calcium Antagonists on 99m TC Sestamibi Liver Clearance in Drug-Resistant Epilepsy" and "Changes in Antiepileptic Medications in Adults After 4 Years of Vagus Nerve Stimulation".

The article presents abstracts of non-AED/non-surgical treatments for general age levels. They include "Gradual vs. Fasting Introduction of the Ketogenic Diet Results in Fewer and Milder Adverse Events," "Patient Perception on Voice Changes With Vagal Nerve Stimulation" and "Side Effects of the Ketogenic Diet for Intractable Epilepsy: A Review of Parental Questionnaire at 3 Months on the Diet".

The article presents an abstract of the workshop "Clinical Trials of Antiepileptic Drugs: Do They Answer Pertinent Clinical Questions?," by Edwin Trevathan, Tracy Glauser, and Jackie French. It would discuss about the pediatric antiepileptic drug trial designs and other novel study designs necessary to answer clinical care needs.

The article presents an abstract of the workshop "Gene Therapy in the Epilepsies - Thoughts on the Future," by L. Matthew Frank, Patrick L. Iversen, Steve Wilton, and Matthew J. During. It would discuss the present investigations on genetic treatment of viral and bacterial diseases in addition to the modifying gene expression in multiple different human organ systems.

The article presents an abstract of the study on blood glucose response to ketogenic diet (KD) children with intractable epilepsy. Discussions on the procedures of the research along with results and observations were demonstrated. Results indicated that low blood glucose during KD therapy was not essential for response to KD with clinically significant seizure reduction.

The article presents an abstract of the study "Chronic Measurement of Increased Epileptiform Activity Over Multiple Menstrual Cycles in Two Patients Using the Responsive Neurostimulator System (RNS)." Results indicate that RNS has the capabilities to detect and diagnose an epileptiform activity over a prolonged period of time.

Overexpression of the multidrug efflux transporter P-glycoprotein (Pgp) at the blood-brain barrier (BBB) is thought to be involved in pharmacoresistance in epilepsy by extruding antiepileptic drugs (AEDs) from their target site. To explore this hypothesis, positron emission tomography (PET) scans were performed with the Pgp substrate-verapamil (VPM) in animal models before and after status epilepticus (SE) and in patients with temporal lobe epilepsy (TLE) and healthy controls. In addition to baseline scans, a second VPM-PET scan was performed after administration of the Pgp inhibitor tariquidar (TQD), showing that VPM uptake at baseline and its increase after Pgp inhibition are reduced in animals following SE compared to baseline, and in refractory TLE relative to healthy controls. In animal models, brain regions with increased Pgp expression (cerebellum, thalamus, and hippocampus) showed reduced influx rate constants from blood to brain, K1, of the radiolabeled Pgp substrate relative to control animals. In human studies, preliminary findings are lower K1 values in refractory compared to seizure-free patients and attenuated increase of K1 for temporal lobe regions in patients with TLE compared to healthy controls. In summary, there is lower brain uptake of the Pgp substrate VPM in Pgp-rich areas of animals 2 days following SE, as well as lower increase in VPM brain uptake after TQD in patients with refractory TLE compared to healthy controls, supporting the hypothesis of increased cerebral Pgp function following prolonged seizures and as a mechanism contributing to drug resistance in refractory epilepsy. The observation of reduced VPM uptake in refractory compared to seizure-free patients with TLE is consistent with multiple mechanisms affecting Pgp function, including uncontrolled seizures. [ABSTRACT FROM AUTHOR]

The article presents abstracts of workshop "Opioids in Pain, Placebo and Epilepsy," by Marylou Solbrig, Fabrizio Benedetti, and Matthias J. Koepp. It aims to contribute to a better understanding of endogenous mechanism of seizure suppression modulated through opioid peptide release, provide knowledge on the role of expectations and effect of placebo and the development of therapeutic approaches.

The article presents an abstract of the workshop "Unorthodox Mechanism of Epileptogenesis," by Damir Janigro, Marco de Curtis, Nicola Marchi, and Alon Friedman. It was designed to show how the decreased blood-brain barrier (BBB) function would lead to cortical dysplasia and the BBB failure to acute seizures.

The article presents abstract of the workshop "Defining Hippocampal Malformations in Epilepsy: From Histopathology to MRI," by Andrea Bemasconi, Neda Bemasconi, Michel Baulac and Maria Thom. It examines the hippocampal malformations visualized on the magnetic resonance images and discusses the incidence of malformations in various forms of epilepsy.

The article presents an abstract of the study "Epilepsy in Angelman Syndrome: A Case for Early Ketogenic Diet?". Results show that cases of Angelman Syndrome were completely controlled in patients with Ketogenic diet. It also suggests that Ketogenic diet treatment should be administered in the early stage.

The article presents an abstract of the study on erythrocyte and omega-3 fatty acids in people with epilepsy. Since fatty acids played a significant role in determining optimal structural and functional properties of neuronal membranes, it could have an important impact on epilepsy. Patients were provided doses of different fatty acids.