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Table of Contents Volume 54, Supplement 3, 2013
30th International Epilepsy Congress, Montreal, Canada, 23–27 June, 2013 Late Breaking Abstracts:
Pages
Tuesday, 25 June 2013 Late Breaking Posters – Tuesday
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Wednesday, 26 June 2013 Late Breaking Posters – Wednesday
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Epilepsia, 54(Suppl. 3):341–362, 2013 doi: 10.1111/epi.12248
LATE BREAKING ABSTRACTS
30th International Epilepsy Congress, Montreal, Canada, 23–27 June, 2013 Late Breaking Posters – Tuesday Tuesday, 25 June 2013
side effects, and APBE intolerability for 2915 adult patients. APBEs included were behavioral changes, depression, irritability/moodiness, psychosis, and anxiety. Intolerability was indicated by the occurrence of APBEs that led to a dosage reduction or cessation. The incidence of AED-attributed APBE intolerability was determined in these patients taking any of the sixteen most commonly prescribed AEDs at Columbia and Yale. We compared the rates of APBE intolerability for each AED in patients with vs. those without APBE or APBE intolerability to (1) another specific AED; (2) any other AED.
LBP1084 BE – BEYOND EPILEPSY: AN EDUCATION AND ADVOCACY PROGRAM IN TANZANIA L. Jurasek1, S. Carline1 1 Stollery Children’s Hospital, University of Alberta, Edmonton, AB, Canada Purpose: To develop and implement an epilepsy education and advocacy program for frontline healthcare providers and key community members in rural Tanzania. The districts of Kilombero and Ulanga have a high prevalence of epilepsy and treatment gap of 95–100% largely due to misconceptions of seizures and knowledge gap at the community level. Method: In order to raise awareness and decrease stigma about epilepsy, identify community members with seizures and increase the number of persons with epilepsy receiving treatment; an epilepsy education and awareness program will be implemented in rural Tanzania. Pre and post design will be used to evaluate the benefits of the intervention. Participants will then further disseminate this information to their communities including local leaders, healers and community members. Community assessments of perceptions of epilepsy pre and post intervention will be utilized to assess for a change in perception of those living with epilepsy. Results: To date a needs assessment has been completed in the districts of Ulanga and Kilombero in rural Tanzania reinforcing the position of the International League Against Epilepsy of the need for increased awareness, understanding and treatment of epilepsy in Africa. BEBeyond Epilepsy is an educational and advocacy program developed in collaboration with the Tanzania National Nurses Association (TANNA), Tanzania Training Center for International Health (TTCIH) and the Tanzania Ministry of Health. Conclusion: The community needs assessment has demonstrated a desire for this initiative amongst frontline healthcare providers, healers, persons with epilepsy and community leaders. The pilot project will be implemented in January 2014 with 25 frontline healthcare providers and key community members. Program implementation will take place at the TTCIH with the support of TANNA and the Ministry of Health.
LBP1085 CROSS-SENSITIVITY OF ADVERSE PSYCHIATRIC AND BEHAVIORAL EFFECTS WITH ANTIEPILEPTIC DRUG USE B. Chen1, K. Detyniecki1, L. Hirsch1 1 Yale Comprehensive Epilepsy Center, New Haven, CT, USA Purpose: To determine rates of cross-sensitivity of adverse psychiatric and behavioral effects (APBEs) among commonly used antiepileptic drugs (AEDs) in adult patients (≥16) with epilepsy. Method: As part of the Columbia and Yale AED database, we conducted a retrospective review of patient background, medical history, AED use,
Results: 16.5% (480/2915) of patients had APBE intolerability attributed to at least one AED; 2.6% (75/2915) had intolerability to two or more AEDs. Of patients who had intolerability to levetiracetam (LEV) and also took zonisamide (ZNS), 20/83 (24.1%) had APBE intolerability to ZNS; of patient who had intolerability to ZNS and also took LEV, 20/ 42 (47.6%) had APBE intolerability to LEV. Of patients who had intolerability to any AED other than LEV and also took LEV, 61/147 (41.5%) had APBE intolerability to LEV. Of patients who had intolerability to any AED other than ZNS and also took ZNS, 61/122 (22.1%) had APBE intolerability to ZNS. Conclusion: The probability of APBE intolerability increases if a patient develops APBE to another AED. The cross-sensitivity rate for APBE intolerability between LEV and ZNS are particularly high. Our cross-sensitivity results may be clinically usefully for predicting APBE intolerability in patients taking certain AEDs and for AED selection in individual patients.
LBP1086 OPPORTUNITY OF USING RECOMBINANT HUMAN INTERLEUKIN-2 IN PATIENTS WITH EPILEPSY L. V. Lipatova1, N. B. Serebryanaya2, N. A. Sivakova1 1 Epilepsy Department, St. Petersburg V.M. Bekhterev Psychoneurological Research Institute, St. Petersburg, Russia, 2 Immune Diagnostic Department, North-Western State Medical University named after I.I. Mechnikov, St. Petersburg, Russia Extensive evidence in favor of the inflammatory theory of epileptogenesis are obtained, according to which epilepsy is a consequence of specialty inflammation in the CNS connected with both the induction of convulsions and their progression. The aim of our study is to evaluate the dynamics of immunological parameters in patients with epilepsy (PE) during the treatment with rIL-2-medicament (Roncoleukinumâ), cytokine drug of Interleukin′s series containing recombinant human Interleukin-2 (rIL-2), which is a structural and functional analog of the endogenous IL-2. Levels of cytokines IL-1b, IL-2, soluble IL-2 receptor (sIL-2R), IL-6 and RAIL-1 and Brain-derived neurotrophic factor (BDNF) in 34 patients with epilepsy are studied. The first group comprised 16 PE were treated rIL-2-medicament, the second group – 14 PE did not receive it. Roncoleukinumâ was injected subcutaneously in a solution of 1 ml at 1.0 mg (1,000,000 ME rIL-2), daily, total number 3. Studied parameters were evaluated before and after treatment. Results. Levels of cytokines IL-1b, IL-2, IL-6 and RAIL-1 did not change significantly in both groups, after the short course of the therapy with and without rIL-2-medicament. However, concentration of IL-8 was significantly reduced after treatment (28.7 15.56- before to 6.3 1.4 pg/ml – after treatment with rIL-2-medicament, p < 0.01) and
341
342 Late Breaking Abstracts BDNF production increased by 1.6 times in the first group of PE in comparison with the second group, (P (U) < 0.01), while in the second group significant changes of these parameters were not observed. We obtained the data on decrease of content of proinflammatory cytokine IL-8 and increase content of BDNF in PE as a result of rIL-2-medicament treatment. These results might be used to optimize treatment of PE due to modulation of inflammatory process and increase of neurotrophic factors production required for the processes of brain neuroplasticity and neurogenesis.
LBP1087 WORD-FINDING DIFFICULTIES AND RETIGABINE: ACT WITH CAUTION! I. Unterberger1, M. Prieschl1, G. Walser1, M. Bergmann1, G. Luef1 1 Department of Neurology, Innsbruck Medical University, Innsbruck, Austria Purpose: Retigabine (RTG) is a new antiepileptic drug (AED) licensed in 2011 as adjunctive therapy in adult patients with drug-resistant focal epilepsy. RTG was generally well tolerated in the RESTORE studies, with most adverse events being of mild or moderate severity. This study aimed to investigate CNS-related adverse events with a special focus on word-finding difficulties in epilepsy patients treated with RTG. Method: Data of patients with drug-resistant focal epilepsy who were treated with adjunctive RTG between December 2011 and March 2013 were analyzed retrospectively. Clinical information was extracted from patients’ charts. Results: Eighteen patients (10M/8F) aged median 37.5 years (range 20– 63 years) were identified. Median age at onset was 12 years (range 1–38), median follow up was 7 years (range 1–12 years). Six patients had frontal lobe epilepsy, eight patients suffered from temporal lobe epilepsy, one patient had parietooccipital epilepsy and three patients presented with multifocal epilepsy. Patients were taking one to three concomitant AEDs (median 2). The efficacy of RTG add-on was evaluated at last follow up; none of the patients was seizure free, two patients (11.1%) reported ≥50% seizure reduction, unchanged seizure frequency occurred in sixteen patients (88.8%). RTG was titrated very slowly with 50 mg/d/week. Eleven patients (61.1%) reported AE. Nine patients (81.1%) developed word-finding difficulties, five patients reported tiredness (45.4%), sedation and dizziness occurred in three patients each (27.3%) and one patient (9.1%) had acute psychosis leading to temporary psychiatric hospitalization. Mainly these AEs already occurred at a daily dosage of 400 mg. RTG was discontinued in seven patients due to AEs alone with symptom remission in all patients. Conclusion: Word-finding difficulties are of particular relevance in patients with drug-resistant epilepsy who are treated with adjunctive RTG. A close meshed clinical control is recommended.
LBP1088 PHARMACOKINETICS AND TOLERABILITY OF INTRANASAL DIAZEPAM IN ADULT PATIENTS WITH EPILEPSY M. Sperling1, H. Seif Eddeine2, K. F. Haas3, G. Krauss4, H. R. Henney III5, A. L. Rabinowicz5, G. Bream6, A. Grinspan5, E. J. Carrazana5 1 Comprehensive Epilepsy Center, Thomas Jefferson University, Philadelphia, PA, USA, 2St. Joseph’s Hospital and Medical Center, Barrow Neurology Clinics, Phoenix, AZ, USA, 3 Department of Neurology, Vanderbilt University Medical Center, Nashville, TN, USA, 4Department of Neurology, Johns Hopkins University, Baltimore, MD, USA, 5Acorda Therapeutics Inc, Ardsley, PA, USA, 6Neuronex, Inc., Morrisville, NC, USA Epilepsia, 54(Suppl. 3):341–362, 2013 doi: 10.1111/epi.12248
Purpose: A diazepam nasal spray (D-NS) formulation is under development for treating cluster seizures. This study determined the pharmacokinetics and tolerability of diazepam following a single dose of 12.5, 15, 17.5, or 20 mg D-NS based on body weight and the ability to administer during the ictal or peri-ictal period. Method: A multi-center, open-label, pharmacokinetic study was conducted in 31 adult patients admitted to the epilepsy monitoring unit (EMU) for pre-surgical evaluation or medical management of epilepsy. Prior antiepileptic drugs (AEDs) were discontinued or reduced in most patients during their stay at EMU. Following standard EMU treatment rules, when an acute benzodiazepine was indicated, eligible patients received a single dose of D-NS approximating 0.2 mg/kg bodyweight. Patients were observed and plasma diazepam concentrations were measured serially up to 12 h post-dose. Pharmacokinetic parameters for Cmax, Tmax, and AUC0-12 were estimated using noncompartmental analyses. Safety parameters and tolerability were assessed. Results: Thirty-one patients were dosed and 30 had post-dose pharmacokinetic data; mean age 35 years, range 18–65. Ten patients received DNS during a seizure. Diazepam was absorbed intranasally with median Tmax at 45 min. The dose-adjusted means and CV% for Cmax and AUC0– 12 were 208 ng/ml and 1227 h/ng/ml, and 43% and 40%, respectively. In this unstable patient population, 65% were seizure-free for 12 h. Treatment was well-tolerated with 74% of the treatment-emergent adverse events mild and local. The most common adverse events related to the route of administration included, dysgeusia(26%), nasal discomfort (23%), lacrimation increased(16%), rhinorrhoea(16%), oropharyngeal pain(13%), paranasal sinus hypersecretion(13%), nasal congestion (10%), parosmia(10%), and throat irritation(7%). At study exit, ≥12 h post-dose, 77% were alert, 10% were drowsy, and 13% sleeping but arousable. Conclusion: Results showed feasibility of ictal or peri-ictal administration of D-NS. Single approximately 0.2 mg/kg dose D-NS proved to be well tolerated with favorable pharmacokinetics in patients with epilepsy. Funding: Neuronex, Inc, and Acorda Therapeutics, Inc.
LBP1089 EPILEPSY; WHAT DO HEALTH PROFESSIONALS IN RIYADH KNOW? A. Alaqeel1, F. Alebdi2, A. Sabbagh3,4 1 Division of Neurosurgery, Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia, 2College of Medicine, King Saud University, Riyadh, Saudi Arabia, 3 Department of Neurosurgery, National Neuroscience Institute, King Fahad Medical City, Riyadh, Saudi Arabia, 4King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia Purpose: The objective of this study is to report on the knowledge of epilepsy, as well as attitudes and practices toward individuals with epilepsy among health professionals in Riyadh, capital city of Saudi Arabia. Methods: A survey consisting of 23 questions pertaining to epilepsy awareness was distributed to health education workers (n = 51), nutrition (n = 56), medicine (n = 265), dentist (n = 58), pharmacist (n = 41), nursing (n = 224) and physiotherapy (n = 65), final year medical student (n = 62) in three tertiary hospitals in Riyadh. Results: Of the 822 respondents who completed the questionnaire, (100%) had heard about epilepsy, (92.6%) would allow their children to interact with an individual who had epilepsy. However, (67%) respondents would not want their children to marry an individual with epilepsy, (97.9%) respondents believed that individuals with epilepsy should have the same employment opportunities as the general population, (10.5%) believes that supernatural power is the cause of epilepsy, (67.2%) did not know how to deal with an individual experiencing an epileptic episode and (56%) did not know that surgery was a treatment option for individuals with epilepsy in Saudi Arabia. Of the 822 respondents, (39%) would
343 Abstracts not abide by a physician′s advice not to operate a motor vehicle because of their illness, of whom (91%) cited problems with the public transportation system as a reason for disregarding the doctor′s advice. When asked if the participants had access to any information of how to deal with epilepsy during their professional training, (60.3%) had access to such information during their graduate studies. Ninety-eight percent of those with access to this information were physicians and health care educators (p < 0.001). Physicians and health care educators were significantly more aware of epilepsy than any of the other groups. Conclusion: The level of epilepsy awareness among health professional needs improvement.
LBP1090 VOLTAGE GATED SODIUM CHANNEL GENE POLYMORPHISMS ARE ASSOCIATED WITH EPILEPSY L. Baum1, B. S. Haerian2, H.-K. Ng3, V. Wong4, P. W. Ng5, C. H. T. Lui6, N. C. Sin7, C. Zhang8, B. Tomlinson9, W. K. G. Wong10, K. S. Wong11, H. J. Tan12, A. A. Raymond12, M. Zahurin2, P. Kwan11,13 1 School of Pharmacy, The Chinese University of Hong Kong, Shatin, China, 2Department of Pharmacology, Faculty of Medicine, University of Malaya, Pharmacogenomics Laboratory, Kuala Lumpur, Malaysia, 3Department of Anatomical and Cellular Pathology, The Chinese University of Hong Kong, Shatin, China, 4Hospital Authority Head Office, Hong Kong, China, 5Department of Paediatric and Adolescent Medicine, The University of Hong Kong, Hong Kong, China, 6 United Christian Hospital, Division of Neurology, Department of Medicine and Geriatrics, Hong Kong, China, 7Queen Elizabeth Hospital, Division of Neurology, Department of Medicine, Kowloon, China, 8University of Georgia College of Pharmacy, Department of Pharmaceutical and Biomedical Sciences, Athens, GA, USA, 9Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Shatin, China, 10Department of Paediatrics, The Chinese University of Hong Kong, Shatin, China, 11Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Division of Neurology, Shatin, China, 12Department of Medicine, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia, 13Departments of Medicine and Neurology, Royal Melbourne Hospital, The University of Melbourne, Melbourne, Vic., Australia Purpose: High frequency action potentials are mediated by voltagegated sodium channels, composed of one large a subunit and two small b subunits, encoded mainly by SCN1A, SCN2A, SCN3A, SCN1B, and SCN2B genes in the CNS. Since mutations of these can cause certain genetic epilepsy syndromes, we investigated whether polymorphisms in these genes may affect epilepsy risk in general. Method: Epilepsy patients and control subjects from Hong Kong and Kuala Lumpur were matched in age, sex and ethnicity. Epilepsy was broadly classified based on ILAE criteria. Blood was withdrawn for DNA extraction. Using Haploview, we tagged the five genes with 43 polymorphisms: 27 in Hong Kong, 28 in Malaysia, and 12 in both locations. Polymorphisms were genotyped by Sequenom Mass Array. Results: The study included 1529 epilepsy patients (meanSD age: 35 16 years) and 1935 control subjects (34 16 years) from four ethnic groups or locations: Malay, Indian, and Chinese, all from Malaysia, and Chinese from Hong Kong (the latter comprising 54% of patients and 44% of controls). Of patients, 19% were idiopathic, 42%
symptomatic, and 40% cryptogenic. The strongest association with epilepsy was rs3812718, or SCN1A IVS5N+5G>A, odds ratio (OR) = 0.85 for allele G (p = 0.0009) and 0.73 for genotypes GG vs. AA (p = 0.003). The association was consistent across ethnicities. Allele G is known to affect splicing and to speed recovery from inactivation. Since SCN1A is preferentially expressed in inhibitory neurons, G may decrease epilepsy risk. SCN1A rs10188577 displayed OR = 1.20 for allele C (p = 0.003); SCN2A rs12467383 had OR = 1.16 for allele A (p = 0.01), and displayed LD with rs2082366 (r2 = 0.67), whose genotypes tended toward association with SCN2A brain expression (p = 0.10). SCN1A rs2298771 was associated with epilepsy in Indians (OR = 0.56, p = 0.005). SCN2B rs602594 was associated with idiopathic epilepsy (OR = 0.62, p = 0.002). Conclusion: Common genetic variants in neuronal sodium channel genes are associated with the risk of epilepsy.
LBP1091 MUTATIONS IN GRIN2A CAUSE IDIOPATHIC FOCAL EPILEPSY WITH ROLANDIC SPIKES S. von Spiczak1, D. Lal2,3, E. M. Reinthaler4, I. Steiner5, M. Nothnagel2, B. Laube6, M. Schwake7, P. Hoffmann8, S. Herms8, J. Altm€ uller2, M. R. Toliat2, H. Thiele2, P. N€ urnberg2, 5 1 1 9 C. Wilhelm , U. Stephani , I. Helbig , H. Lerche , F. Zimprich4, B. A. Neubauer3, S. Biskup5, J. R. Lemke10, GRIN2A study group 1 University Medical Center Schleswig-Holstein, Campus Kiel, Kiel, Germany, 2Cologne Center for Genomics, Cologne, Germany, 3University Medical Center Giessen and Marburg, Giessen, Germany, 4Medical University of Vienna, Vienna, Austria, 5CeGaT GmbH, Tuebingen, Germany, 6Technical University Darmstadt, Darmstadt, Germany, 7Bielefeld University, Bielefeld, Germany, 8University of Bonn, Bonn, Germany, 9University of Tuebingen, Tuebingen, Germany, 10 University Children’s Hospital Inselspital, Bern, Switzerland Purpose: To test the hypothesis that mutations and genomic rearrangements in GRIN2A encoding the NMDA-receptor NR2A subunit may contribute significantly to the pathogenesis of idiopathic focal epilepsies (IFE). Method: We analyzed a cohort of 119 patients with IFE by candidate gene sequencing and a replication cohort of 240 patients with IFE by analysis of whole exome sequencing data for mutations in GRIN2A. In addition, 286 IFE patients were screened for copy number variations (CNVs) in GRIN2A. Results: Novel heterozygous mutations in GRIN2A that were not listed in dbSNP, 1000Genomes or the Exome Variant Server (EVS) databases were detected in 8/119 patients (6.7%) and 19/240 patients (7.9%) of the replication cohort giving an overall mutation rate of 27/359 (7.5%). Therefore, mutations occurred significantly more frequently than in the EVS (37/6503 = 0.6%; p = 4.83′10 18, Fisher’s exact test). Exon-disrupting microdeletions were found in 3/286 patients (1.0%), i.e. significantly more often than in 1520 in-house controls (0/1520; p = 0.004, Fisher’s exact test). Conclusion: These results establish alterations of GRIN2A as a causal factor for IFE.
LBP1092 HIPPOCAMPAL MALROTATION IS ASSOCIATED WITH CHROMOSOME 22Q11.2 MICRODELETION D. M. Andrade1, T. Krings2, E. W. C. Chow3, T.-R. Kiehl4, A. S. Bassett5 Epilepsia, 54(Suppl. 3):341–362, 2013 doi: 10.1111/epi.12248
344 Late Breaking Abstracts 1 Neurology, Krembil Neuroscience Program, Toronto Western Hospital/University of Toronto, Toronto, ON, Canada, 2 Radiology/Krembil Neuroscience Program, Toronto Western Hospital/University of Toronto, Toronto, ON, Canada, 3 Psychiatry, Clinical Genetics Research Program, Centre for Addiction & Mental Health/University of Toronto, Toronto, ON, Canada, 4Laboratory Medicine and Pathobiology, University Health Network/University of Toronto, Toronto, ON, Canada, 5 Psychiatry, Clinical Genetics Research Program & Division of Cardiology, Centre for Addiction & Mental Health/University of Toronto, Toronto, ON, Canada
Purpose: Patients with chromosome 22q11.2 deletion syndrome (22q11DS) are at a sevenfold increased risk of developing seizures. However, only a fraction of these patients exhibit structural abnormalities such as polymicrogyria (PMG) and periventricular nodular heterotopia (PNH) that are known to cause seizures and to be associated with 22q11DS. In this study we used a dedicated seizure imaging protocol look for additional structural abnormalities in these individuals that may explain the elevated risk of seizure disorder in this patient group. Methods: Nineteen consecutive adult subjects with 22q11DS underwent a 3 Tesla MRI with a dedicated high-resolution seizure protocol. Neurological exam was performed in all patients. Genome-wide analysis excluded the presence of other pathogenic microdeletions or duplications. Results: Structural abnormalities were found in 11 of 14 subjects with sufficient image quality. These included three patients with PNH, one of whom had associated PMG. In addition, there was a surprisingly high prevalence of unilateral hippocampal malrotation (HIMAL), observed in 9 of 14 cases (64%). EEG findings showed interictal epileptiform discharges with focal distribution in four patients and generalized discharges in one patient. Conclusion: The results suggest that, in addition to other known structural abnormalities, 22q11DS is associated with HIMAL. It has been suggested that this developmental abnormality of the hippocampus may predispose or otherwise contribute to epileptogenesis. However in this study we observed HIMAL in a large proportion of patients, with and without epilepsy. Therefore, other as yet unknown factors may contribute to the high prevalence of epilepsy in this population.
LBP1093 AMYGDALA ENLARGEMENT IN PATIENTS WITH MESIAL TEMPORAL LOBE EPILEPSY WITHOUT HIPPOCAMPAL SCLEROSIS M. E. Morita1, A. C. Coan1, B. M. Campos1, C. L. Yasuda1, F. Cendes1 1 University of Campinas – UNICAMP, Neurology, Campinas, Brazil Purpose: Patients with mesial temporal lobe epilepsy (MTLE) without MRI abnormalities (MTLE-NL) represent a challenge. The objective of this study was to look for undetected areas of increased grey matter volume in MTLE-NL using post processing MRI techniques. Method: We evaluated 66 patients with MTLE-NL on visual analysis and 82 controls. Voxel-based morphometry (VBM) group analysis was performed with VBM8/SPM8 looking for areas of increased grey matter volume. According to VBM results we analyzed the amygdala as a second step. We performed automatic amygdala volumetry using Freesurfer software to confirm VBM findings. To cross validate our results we performed a third analysis using VBM to compare subgroups defined by amygdala volumes. Epilepsia, 54(Suppl. 3):341–362, 2013 doi: 10.1111/epi.12248
Results: VBM group-analysis demonstrated increased amygdala volume in the MTLE-NL group compared to controls. Individual volumetric analysis confirmed amygdala enlargement (AE) in eight (12%). Overall, from all patients with AE and defined epileptic focus, four (57%) had the predominant increased volume ipsilateral to the epileptic focus. Secondary VBM analysis confirmed that a subgroup of patients with MTLE-NL had MRI signs of AE that was detected by two different methods. There were no clinical differences between patients with and without AE. Conclusion: This study demonstrates the occurrence of AE in 12% of patients with MTLE-NL. This finding supports the hypothesis that there might be a subgroup of patients with MTLE-NL in which the enlarged amygdala could be related to the epileptogenic process. Further studies are necessary but this finding could be of great importance in the understanding of MTLE-NL.
LBP1094 INTERNATIONAL CONSENSUS CLASSIFICATION OF HIPPOCAMPAL SCLEROSIS IN TEMPORAL LOBE EPILEPSY: A TASK FORCE REPORT FROM THE ILAE COMMISSION ON DIAGNOSTIC METHODS R. Coras1, I. Bl€ umcke2, M. Thom3, E. Aronica4, D. Armstrong5, 6 F. Bartolomei , A. Bernasconi7, N. Bernasconi7, C. G. Bien8, F. Cendes9, H. J. Cross10, T. S. Jacques11, P. Kahane12, M. W. 16 € Gary13, H. Miyata14, S. L. Moshe15, B. Oz16, C. Ozkara , E. Perucca17, S. Sisodiya3, S. Wiebe18, R. Spreafico19 1 Department of Neuropathology, Erlangen, Germany, 2 Department of Neuropathology, University Hospital Erlangen, Erlangen, Germany, 3Institute of Neurology/University College London, London, UK, 4Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands, 5Baylor College of Medicine, Houston, TX, USA, 6Brain Dynamic Institute, INSERM U1006, Marseille, France, 7Montreal Neurological Hospital and Institute, McGill University, Montreal, QC, Canada, 8Hospital Mara, Epilepsy Center Bethel, Bielefeld, Germany, 9University of Campinas UNICAMP, Campinas, Brazil, 10The Prince of Wales’s Cahir of Childhood Epilepsy UCL-Institute of Child Health, London, UK, 11UCL Institute of Child Health, London, UK, 12Neurology Department and INSERM U836, Grenoble, France, 13Departments of Neurosurgery, and Psychiatry & BioBehavioral Medicine, Los Angeles, CA, USA, 14Department of Neuropathology, Research Institute for Brain and Blood Vessels, Akita, Japan, 15Saul R. Korey Department of Neurology, Albert Einstein College of Medicine, New York, NY, USA, 16Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey, 17 Department of Pharmacology, University of Pavia, Pavia, Italy, 18Departments of Clinical Neurosciences, Community Health Sciences and Paediatrics, University of Calgary, Calgary, AB, Canada, 19Department of Epilepsy Clinic and Experimental Neurophysiology, Neurological Institute “C. Besta”, Milan, Italy Purpose: Hippocampal sclerosis (HS) is the most frequent histopathology encountered in patients with drug-resistant temporal lobe epilepsy (TLE). In past decades, various attempts have been made to classify specific patterns of hippocampal neuronal cell loss and correlate subtypes with postsurgical outcome. However, no international consensus about definitions and terminology use has been achieved. Method: A Task Force reviewed previous classification schemes and proposes a system based on semi-quantitative hippocampal cell loss patterns that can be applied in any histopathology laboratory.
345 Abstracts Results: Inter- and intraobserver agreement studies reached consensus to classify three types in anatomically well preserved hippocampal specimens: HS ILAE Type 1 refers always to severe neuronal cell loss and gliosis predominantly in CA1 and CA4 regions, compared to CA1 predominant neuronal cell loss and gliosis (HS ILAE Type 2), or CA4 predominant neuronal cell loss and gliosis (HS ILAE Type 3). Surgical hippocampus specimens obtained from TLE patients may also show normal content of neurons with reactive gliosis only (no-HS). HS ILAE Type 1 is more often associated with a history of initial precipitating injuries before age five years, with early seizure onset and favourable postsurgical seizure control. CA1 predominant HS ILAE Type 2 and CA4 predominant HS ILAE Type 3 have been studied less systematically so far, but some reports point to less favourable outcome, and to differences regarding epilepsy history, including age of seizure onset. Conclusion: The proposed international consensus classification will aid in the characterization of specific clinico-pathological syndromes, explore variability in imaging and electrophysiology findings, and postsurgical seizure control.
LBP1095 ANGIOCENTRIC GLIOMA: SEVEN CASES REPORT INCLUDING TWO CASES WITH UNUSUAL HISTOLOGICAL FEATURES Y.-S. Piao1, H.-C. Ni1, L. Chen1, D.-H. Lu1 1 Department of Neuropathology, Xuanwu Hospital, Capital Medical University, Beijing, China
Department of Neurology, “Evaggelismos” Hospital, Athens, Greece, 2Department of Neurosurgery, Epilepsy Surgery Unit, “Evaggelismos” Hospital, Athens, Greece, 3Department of Neurology, “Eginition” Hospital, Athens, Greece
1
Purpose: The term alexithymia indicates a form of affect dysregulation, which mainly results in difficulty in recognizing and describing feelings with words. High rates of alexithymia have been reported in several medical conditions. This study evaluates the levels of alexithymia in epileptic patients. Method: One hundred and forty-six patients with epilepsy aged 15–60years-old (57% female) and equal number of non-epileptic individuals of the same age completed the Schalling-Sifneos Personality Scale – Revised (SSPS-R) for alexithymia assessment. Individuals scoring ≥10 were classified as alexithymic. Results: Epileptic patients scored higher than the controls (p = 0.000) with 33.5% (N = 49) identified as being alexithymic in comparison to 10.2% (N = 15) of the control group. The epileptic patients had a mean score of 8.36 (SD = 2.443) whereas controls had a mean score of 6.53 (SD = 2.294). The statistical analysis revealed also differences in alexithymia levels between men and women with epilepsy (p = 0.014). Men score higher (mean = 8.92, SD = 2.523) than women (mean = 7.93, SD = 2.305). No significant correlation was found between the type of seizures and alexithymia scores (p = 0.48). Conclusion: A significant correlation exists between alexithymia and epilepsy, with men having higher risk for developing alexithymic characteristics. We could not detect a direct relationship between type of seizures and alexithymia.
Purpose: Angiocentric glioma (AG) is a rare, slow-growing tumor of the CNS, which is usually associated with refractory epilepsy and primarily affecting children and young adults. Method: Here, we report additional seven cases with clinical, radiological, histologic and immunohistochemical characteristics. Results: These seven cases of AG were three males and four females with a median age at surgery of 17.6 years. The mean duration of pre-surgical epilepsy is 4.7 years. All seven cases of AG were located in superficial cerebrocortical locations with 3 in temporal cortex, 2 in frontal cortex and 2 in parietal cortex. In all of the seven cases, the mass appeared hyperintense on T2-weighted images (T2WI) and fluid-attenuated inversion recovery (FLAIR) images, and was non-enhanced after gadolinium contrast injection. Histologically, seven cases of AG revealed mainly of monomorphic, bipolar spindle-shaped cells, showing an angiocentric growth pattern and features of ependymal differentiation. However, besides these characteristics of AG, two cases had unusual histological features. In one case, one region had histological features like astroblastoma, showing unipolar columnar tumor cells aligned radially along blood vessels, and the perivascular pseudorosette with stout processes and pseudo-papillary appearance. In another case, a large cystic pattern was found. In the cystic region, there were many bipolar spindled cells aligned radially or longitu-dinally along parenchymal blood vessels. Immunohistochemically, all cases were positive for glial fibrillary acidic protein (GFAP) and vimentin, and negative for IDH1R132H and the neuronal marker NeuN. Epithelial membrane antigen (EMA) was found positive in five cases with a case showing dot-like pattern, while the other were negative. D2-40 was diffuse positive in six cases, with one case having a dot-like pattern which similar to EMA. Conclusion: These findings further suggest that besides the angiocentric growth pattern, there may be unusual histological features in AG.
LBP1096 ALEXITHYMIA IN EPILEPSY PATIENTS V. Zisimopoulou1, A. Siatouni2, N. Triantafyllou3, A. Tavernarakis1, D. Sakas2, S. Gatzonis2
LBP1097 RESEARCH OF COMMUNICATION SKILLS IN PATIENTS WITH EPILEPSY R. T. B. Abbas1, C. S. Messas2 1 Faculdade de Ci^encias Medicas da Santa Casa de S~ ao Paulo, Fonoaudiologia, S~ ao Paulo, Brazil, 2Faculdade de Ci^encias Medicas da Santa Casa de S~ ao Paulo, S~ ao Paulo, Brazil Purpose: Investigate the self-perception of communication difficulties in adult patients with Temporal Lobe Epilepsy; characterize the possible communication difficulties in these patients. Method: We interviewed 30 patients with temporal lobe epilepsy, 30 control group subjects via questionnaire communicative aspects of language, specifically developed for this research. Results: The patient group scores positive points for difficulty in understanding functional language (20%), difficulty in semantic recall (57%), pragmatic communication difficulties (67%, 50% and 50%). The control group scored statistically below the group of patients evaluated for these aspects. Conclusion: The comparison between groups showed the presence of perceived difficulties in communication skills in patients with epilepsy, especially in relation to specific aspects of language, as an evocation lexical-semantic and pragmatic functions of communication. The clarification of this aspect may contribute to specific health programs aimed at improving the quality of life of these patients and contribute to the specificity of the performance of speech therapy for this population.
LBP1098 ARE AMERICAN ACADEMY OF NEUROLOGY EPILEPSY QUALITY MEASURES BIENG FOLLOWED IN CHILDREN? Epilepsia, 54(Suppl. 3):341–362, 2013 doi: 10.1111/epi.12248
346 Late Breaking Abstracts D. Clarke1,2, S. Jean3, K. Tindall1, S. LeSure1, F. Perkins1,2 1 Dell Childrens Medical Center, Austin, TX, USA, 2University of Texas Southwestern, Austin, TX, USA, 3University of Texas, Austin, TX, USA Purpose: Eight quality measures were recommended by a subcommittee of the AAN in 2011. We reviewed investigative studies (VEEG/EMU, MRI, Neuropsychological evaluation) by our 14 Pediatric Neurology Practice providers (4 epileptologists). 2011 Charts were reviewed and after intervention we are actively reviewing ongoing 2012 and 2013 practice after educational intervention. Method: Two months of charts were reviewed prior to discussing the AAN quality measures with the group to determine if measures were routinely followed. Epilepsy ICD-9 codes were reviewed for May and June of 2011. Other related codes for seizures, convulsions etc. were excluded to limit the possibility of diagnostic ambiguity. Results: Two months of review yielded 423 children (56 in the Epilepsy Unit and Epilepsy Surgery clinic). 165/367 (45%) patients in the outpatient setting with recurrent seizures were analyzed. 38/165 (23%) had tried 1 AED, 17.6% two AED’s, and 57.6% had tried at least 3 AED’s. 21/95 (22%) had no documentation of having a prolonged VEEG in the Epilepsy monitoring unit. A further 20/95 (21%) had not been re-evaluated in over 3 years. Ten percent had no documentation of receiving MRI’s and only 11.6% received neuropsychology evaluation. 2012 evaluations are near completion for comparison. Conclusion: This data from a very active EMU, 350–400 admissions/ year, suggests the need for a more protocol driven approach. Education and dedication at all levels of care will be required to acomplish the defined measures.
LBP1099 ATYPIC SSPE CASE WITH MYOCLONIC JERKS WITHOUT EEG FINDINGS M. Uzun1, B. C ß inar1 1 Pediatric Health and Diseases and Pediatric Neurology, Konya Education and Research Hospital, Konya, Turkey Subacute sclerosing panencephalitis (SSPE) is a rare complication of measles infection characterized by progressive, fatal disease of the central nervous system. It clinically presents after six or more years after acute measles infection with the onset of intellectual deterioration and behavioral changes followed by myoclonias and eventually complete neurologic deterioration. Case: We report a 12-year-old boy, with myoclonic jerks on the head and right side of the body as presenting symptom and progressed with the evidence of poor school performance, He had been healthy up until about 2 months prior to presentation. Before the onset of his symptoms, he had no history of acute, chronic illness, fever or head injury. Results: Laboratory investigations including metabolic screening revealed no abnormality. The initial EEG was normal and repeated after diazepam injection there was no evidence for the typical EEG pattern of SSPE. Brain magnetic resonance imagining (MRI) was also ormal. Increased anti-measles antibody was detected in serum and CSF. Conclusion: SSPE is one of the most important complications of measles in children and adolescents The disease is fatal in 90–95% of cases. In recent years, a lack of SSPE-specific EEG pattern and atypical SSPE presentation could be an increasing differential problem leading to erroneous diagnosis of other neurogical condition and subsequently to an inappropriate treatment We could not observe typical EEG complexes for SSPE with or without diazepam injection. Positive antibody titer against measles in CSF verified the diagnosis of SSPE. This is a rare case of atypical SSPE with no evidence of EEG findings. Measles and SSPE still remains an important problem especially in developing countries. Epilepsia, 54(Suppl. 3):341–362, 2013 doi: 10.1111/epi.12248
Due to uncommon clinical presentation at onset, early diagnosis of SSPE could be difficult and delayed. A high index of suspicion is needed to detect SSPE in its atypical forms, especially in measles endemic countries.
LBP1100 DOES THE SLEEP DISTURBANCE SCALE DISTINGUISH AMONG CHILDREN WITH DIFFERENT NEUROLOGICAL DISORDERS? A. Shuper1,2, R. Cohen1,2, A. Halevi1,2 1 Schneider Children’s Medical Center of Israel/Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel, 2Pediatric Neurology, Petach Tikva, Israel Purpose: The Sleep Disturbance Scale for Children (SDSC) is used routinely in our neurology clinic. This study sought to examine its ability to distinguish among children with different neurological disorders. Method: The SDSC was completed for all children aged 2–18 years who attended the Neurology Clinic of Schneider Children’s Medical Center of Israel in March-June 2010. The children were divided into three groups by major diagnostic category: attention-deficit hyperactivity disorder (ADHD), epilepsy, and others. Data were analyzed using BMDP software. The total sleep score as well as each of the six subscale scores were converted into binary variables based on a T-score of >70 (>95th percentile) [Bruni O, et al. J Sleep Res, 1996;5:251–261] and recoded as 0 or 1. Crude odds ratios (OR) for the total and subscale scores were calculated by multivariate logistic regression models. Results: The sample included 186 children, 109 (58.6%) male and 77 (41.4%) female. Sixty-two (33.3%) had ADHD, 58 (31.2%) had epilepsy, and 66 (35.4%) had other neurological disorders. Fifty children (27%) had a pathological total score on the SDSC. No significant association was found between the SDSC subscale scores and category of neurological disorder. The epilepsy group had a higher frequency of sleep-wake transition disorder than the other groups (OR = 2.48. 95% CI 0.99–6.23, p = .06). Conclusion: Epileptic children have a high incidence of ADHD according to the MOXO test. The manifestations are no worse in children with BECCTS than children with other epilepsy types. The main disturbances associated with BECCTS are timing and attention rather than hyperactivity or impulsivity.
LBP1101 SUCCESSFUL TREATMENT TWO CHILDREN WITH DRUG RESISTANT MYOCLONIC-ASTATIC EPILEPSY (DOOSE SYNDROME) WITH KETOGENIC DIET M. Bacha nski1, A. Wierzbicka2, T. Kmiec1, D. Rokicki1, M. Olszaniecka1 1 Child Neurology Department, The Children’s Memorial Health Institute, Warszawa, Poland, 2Department of Biochemistry, Radioimmunology and Experimental Medicine, The Children’s Memorial Health Institute, Warszawa, Poland Purpose: We describe two children with drug resistant myoclonic-astatic epilepsy (Doose syndrome) in order to provide confirmation, that such treatment is safe, successful and could be an option in such cases. Method: In two children (two boys) with drug resistant myoclonic-astatic epilepsy ketogenic diet was initiated. In the first child it was 3.5:1 ketogenic diet and in second child 2:1. Lab values (e.g. cholesterol and beta-hydroxybutyrate levels), eeg data were provided. Over 3 years observation period.
347 Abstracts Results: Complete cessation of seizures in two observed children. Good tolerability of the diet. Marked EEG improvement.
AERRPS were enrolled in the study. Patient information was obtained from clinical research forms, which were filled out by clinicians.
Conclusion: Our observation may confirm that treatment with ketogenic diet is useful option in the treatment of children with drug resistant myoclonic-astatic epilepsy (Doose syndrome).
Results: The age at onset ranged from 3 to 15 years (median; 5 years). Neurological manifestations were preceded by febrile prodromes in 11 of 13 patients. Seizures were of focal origin and were stereotypical: eye deviation (n = 9), facial twitch (n = 7), apnea (n = 7), and hemiclonic seizures (n = 5). Other neurological manifestations included psychosis (n = 3) and oral dyskinesia (n = 3). Cerebrospinal fluid pleocytosis was observed in 9 of 11 patients, although to a modest degree. Electroencephalography showed either high voltage slowing (n = 5) or epileptiform discharges (n = 9). Brain magnetic resonance imaging was abnormal in 9 of 13 patients; the basal ganglia (n = 4), cerebral cortex (n = 3), thalami (n = 2), and hippocampi (n = 2) were involved. Autoantibody screening tests revealed anti-neuronal antibodies of unknown specificity in two patients. Treatments included methylprednisolone pulse therapy (n = 12), intravenous immunoglobulin (n = 9), and burst-suppression coma (n = 10). The outcome was variable: a full recovery occurred in 4 of 10 patients; mild to moderate neurological deficits were observed in 3 of 10 patients; and severe to profound deficits occurred in 3 of 10 patients.
LBP1102 INSIGHT IN THE ANTERIOR PREFRONTAL CORTEX: THE PATIENT WHO BECAME GOOFY AND DANCED LIKE MICHAEL JACKSON J. Neilson1, T. Snyder2, J. Pugh3, M. Wheatley3, R. Tang-Wai1 1 Pediatric Neurology, University of Alberta, Edmonton, AB, Canada, 2Department of Psychiatry, University of Alberta, Edmonton, AB, Canada, 3Department of Surgery, University of Alberta, Edmonton, AB, Canada Purpose: The anterior prefrontal cortex (aPFC), Brodmann Area 10 (BA10), is thought to play a role in strategic processing, memory recall, and executive functioning. It is one of the least understood areas of the brain, and its connectivity to other cortical regions has yet to be fully elucidated. This case report gives insight in the function of the aPFC and its interconnectivity.
Conclusion: Major characteristics of AERRPS included male predominance, stereotypical seizure phenotype, frequent cerebrospinal fluid pleocytosis, and various abnormalities on neuroimaging.
Method: We present a 10 year old patient with right frontal lobe epilepsy due to a lesion with electrographic ictal onset precisely localized to BA10.
LBP1104 STUDY OF ETIOLOGICAL PROFILE OF INFANTILE AND CHILDHOOD PARTIAL SEIZURES AT A TERTIARY CARE CENTRE IN SOUTH INDIA K. N. Vykuntaraju1,2, S. Smitha3, P. Kumar3, R. Premalatha4 1 Department of Pediatric Neurology, Indira Gandhi Institute of Child Health, Bangalore, India, 2Department of Pediatric Neurology, Bangalore Child Neurology and Rehabilitation Center, Bangalore, India, 3Department of Pediatrics, Indira Gandhi Institute of Child Health, Bangalore, India, 4Indira Gandhi Institute of Child Health, Bangalore, India
Results: Thirty-four seizures were recorded and consisted of a variety of different behaviors ranging from sudden episodes of spontaneous laughter, performing specific celebratory behaviours that had been popular among his classmates, and one episode of performing a Michael Jackson dance move. Electrographically, the seizures were all stereotyped and arose from the right frontopolar region followed by a distinct left temporal ictal rhythm that corresponded with the onset of the behaviours. All seizures involved a demonstration of euphoria and elation. Conclusion: The electrographic analysis of the seizures and the video analysis of the unique seizure semiology suggest an interconnectivity between the aPFC and the contralateral temporal lobe; moreover, this case suggests that the aPFC plays a role in the retrieval of specific memories and emotional states.
LBP1103 ACUTE ENCEPHALITIS WITH REFRACTORY, REPETITIVE PARTIAL SEIZURES (AERRPS)/FEBRILE INFECTION-RELATED EPILEPSY SYNDROME (FIRES): A MULTICENTER STUDY ON 13 CHILDREN H. Sakuma1, I. Kuki2, N. Tanuma3, M. Hayashi1 1 Department of Brain Development and Neural Regeneration, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan, 2 Department of Pediatrics, Medical Center for Children, Osaka City General Hospital, Osaka, Japan, 3Department of Pediatrics, Tokyo Metropolitan Fuchu Medical Center for the Disabled, Tokyo, Japan Purpose: To report the clinical and laboratory findings of patients with acute encephalitis with refractory, repetitive partial seizures (AERRPS); results are based on the second nationwide surveillance study carried out in Japan. Methods: This was a prospective multicenter study executed between 2010 and 2012. Thirteen patients who fulfilled the diagnostic criteria of
Purpose: To evaluate the etiology of focal seizures at tertiary care center. Method: This is a Cross sectional study. A total of 150 children aged 1 month to 18 years presenting with focal seizures defined as per International League against Epilepsy Classification, participated in the study. A detailed history was taken and clinical examination was done and was investigated for the etiology of focal seizures with routine and specific investigations, CT scan and/or MRI and EEG. All the findings were recorded in a pre designed proforma and results were analyzed. Results: The mean age presentation was 6.5 years. Male preponderance was noticed with a ratio of 1.2:1. Complex partial seizures were the commonest (68%) type of focal seizures. Neurologic deficits were noticed in 36(24%) patients. CT scan abnormalities were noted in 76% of the patients, most common CT finding was inflammatory granuloma. In 4 cases, where CT scan was inconclusive of neurocysticercosis vs. tuberculoma, MRI showed features of tuberculoma. The most common etiology of focal seizures was inflammatory granulomas; neurocysticercosis (69%) and tuberculoma (15%), followed by perinatal insult (8%) and stroke (7.3%). EEG was diagnostic in cases of rolandic epilepsy in 7 (4.6%) cases. Conclusion: The most common etiology of focal seizures was inflammatory granulomas and infections. CT Brain is a valuable tool in identifying the underlying etiology of focal seizures. MRI is helpful in cases of diagnostic dilemma between neurocysticercosis and tuberculoma by CT scan. EEG is diagnostic of Benign Rolandic Epilepsy. Epilepsia, 54(Suppl. 3):341–347, 2013 doi: 10.1111/epi.12248
348 Late Breaking Abstracts LBP1105 AUTOMATED DETECTION AND VIDEO ANALYSIS FOR NOCTURNAL CHILDHOOD EVENTS (ADVANCE) E. S. Ng1, W. S. D. Chan2, H. L. Eng1, W. Darsono1, Y. L. Ng2 1 Institute for Infocomm Research, Singapore, Singapore, 2KK Women’s and Children’s Hospital, Singapore, Singapore Purpose: Nocturnal seizures are cause for concern in epileptic children and their caregivers. These concerns result in caregiver stress and unwitnessed seizures could lead to SUDEP. We present a video-based system to detect hypermotor seizures in children with seizures and epilepsy. Method: Seizures were recorded from children with epilepsy admitted to the Epilepsy Monitoring Unit in our institution. Nine children with seizures during the admission were selected for analysis. Video sequences with hypermotor seizures were captured and compared to manual analysis. Results: Video sequences of 30 seizures in nine patients were selected. Manual analysis identified seizure onset and duration. An automated video analysis system was applied to the same sequences and accuracy compared to manual analysis. The system achieved a sensitivity of 93.86% and specificity of 93.33%. Performance with long seizures and low motion interference rose to a sensitivity of 100% and specificity of 97.78%. Conclusion: Automated video analysis provides a viable non-invasive monitoring solution for pediatric patients with seizures and epilepsy. Accurate detection of hypermotor seizures is feasible with a low false alarm rate. This can form an adjunct to caregiver monitoring and a noninvasive solution suitable for children.
LBP1106 USE OF THE MOXO TEST TO EVALUATE THE CORE COMPONENTS OF ADHD IN EPILEPTIC PATIENTS R. Cohen1, A. Halevi1, A. Shuper1 1 Department of Pediatric Neurology, Schneider Children’s Medical Center of Israel/Sackler Faculty of Medicine, Tel Aviv University, Petach Tikva, Israel Purpose: To apply the MOXO, a continuous performance test for the measurement of the four core components of ADHD [Berger I, Goldzweig G. Isr Med Assoc J 2010;12(9):531–535.], in children with epilepsy. Method: All epileptic children aged 6–18 years attending the Neurology Clinic of Schneider Children’s Medical Center of Israel in 2012–2013 were evaluated with the DSM-IV and completed the MOXO test. Background data were collected from the patient files and revision of the electroencephalograms. Scores for the ADHD components (hyperactivity, inattention, timing, impulsivity) were compared to norms using analysis of variance (ANOVA), controlling for age and sex. Results: The sample included 49 children (26 boys, 23 girls) with epilepsy: 32 benign epilepsy of childhood with central spikes (BECCTS), 9 generalized epilepsy, 6 complex partial epilepsy, and 2 juvenile myoclonic epilepsy. Twenty-six children (53%) had a DSM-IV diagnosis of ADHD, 11 with attention deficit only. Eighteen children with BECCTS (56%) had ADHD, 7 (38%) with attention deficit only. On statistical analysis, the effect of group was significant for all ADHD components by the MOXO test (p < 0.001). The BECCTS group had significantly less hyperactivity than the other epilepsy groups (p = 0.01) and a higher proportion of patients free of ADHD symptoms (48.3% vs. 29.4%). Conclusion: Epileptic children have a high incidence of ADHD according to the MOXO test. The manifestations are no worse in children with BECCTS than children with other epilepsy types. The main disturbances associated with BECCTS are timing and attention rather than hyperactivity or impulsivity. Epilepsia, 54(Suppl. 3):341–347, 2013 doi: 10.1111/epi.12248
LBP1107 TWO CHILDREN WITH REFRACTORY OCCIPITAL LOBE EPILEPSY: THE DISTINGUISHING CLINICAL, ELECTROGRAPHIC, ELECTROCORTIGRAPHIC FEATURES OF RASMUSSEN′S ENCEPHALITIS R. Tang-Wai1, L. Bello-Espinoza2, E. Johnson3, W. Hader4, J. Pugh5, M. Wheatley6, Y. Starreveld4, A. Datta2, M. Scantlebury2 1 Department of Pediatrics, University of Alberta/Stollery Children’s Hospital, Edmonton, AB, Canada, 2Department of Pediatrics, University of Calgary and Alberta Children’s Hospital, Calgary, AB, Canada, 3Laboratory Medicine & Pathology, University of Alberta, Edmonton, AB, Canada, 4 Clinical Neurosciences, University of Calgary, Calgary, AB, Canada, 5Stollery Children’s Hospital, University of Alberta, Surgery, Edmonton, AB, Canada, 6Department of Surgery, University of Alberta, Edmonton, AB, Canada Purpose: Rasmussen’s Encephalitis (RE) is a debilitating epilepsy syndrome typically presenting with refractory peri-Rolandic seizures, epilepsia partialis continua (EPC), hemiparesis and cerebral hemiatrophy. Rarely, RE may present with occipital lobe seizures, and these patients are often presumed to have a FCD, especially in the setting of normal imaging. We report two cases of RE presenting with occipital lobe epilepsy to highlight the clinical and electrodiagnositc features that may distinguish RE from FCD. Method: We report two children with RE who presented with occipital epilepsy and features suggesting FCD. Presented are the seizure semiology, scalp-EEG, imaging and electrocorticography (ECoG) to highlight the distinguishing features of RE from FCD. Results: Both patients presented with occipital lobe seizures; moreover, scalp EEG showed occipital rhythmic epileptiform discharges (RED)s suggestive of FCD. Initial brain MRI was normal. The seizures were intractable to treatment and over 1 year the clinical semiology evolved to implicate other cortical regions. At that time subtle hemispheric atrophy consisting of widened sulci began to appear. ECoG showed multifocal electrographic seizures within the posterior region. Conclusion: Despite the initial presentation of occipital seizures and the appearance of REDs, the intractability and evolution of the seizure semiology are clinical markers which may indicate of RE even before the appearance of hemiatrophy and EPC. The multifocal electrocortigraphic findings seen on ECoG may suggest RE and as they appear to mirror the multifocal pathological findings and the juxtaposition of abnormal and normal tissue typically seen in RE, thus further distinguishing RE from FCD.
LBP1108 COMPLICATIONS OF PROLONGED TREATMENT WITH THE KETOGENIC DIET IN TREATMENT RESISTANT EPILEPSY M. Gainza1,2, A. Michoulas2, M. B. Connolly2, F. Zanotta2, C. Simonson2, L. Huh2, K. Selby2, K. Farrell2 1 Universidad Austral de Chile, Valdivia, Chile, 2Division of Pediatric Neurology, Department of Pediatrics, University of British Columbia and British Columbia’s Children’s Hospital, Vancouver, BC, Canada Purpose: The purpose of this study is to determine complications of prolonged treatment with the ketogenic diet (KD) in children treated for a minimum of 3 years. Methods: This study is a retrospective chart review of all epilepsy patients treated with the KD for a minimum of 3 years at BC Children’s Hospital between 1998 and 2013. The study population comprised 41
349 Abstracts patients, 23 males and 18 females. Mean age at initiation of KD was 4.7 years (1–13) and mean duration of KD was 5.79 years (3–10.6). Results: 38.1% (N = 16) patients developed osteopenia, 5 of whom had a bone mineral density (BMD) exam. Mean –-score was 3.178 ( 1.29 to 4.7). 21.4% (N = 9) had fractures, median number 2 (range 1–13) and all of them had osteopenia. The fractures were associated with mild trauma in 10.7% and in 89.3% there was no trauma history. Cerebral palsy was a risk factor for osteopenia (p = 0.0005) and fractures (p = 0.03). 7.1% (N = 3) had renal stones (range 1–13), 2 of whom had also osteopenia. 26.2% (N = 11) had hypercalcuria. Urinary pH decreased by a mean of 1.3 and 9.5% (N = 4) had severe metabolic acidosis. 21.4% of patients had recurrent pneumonia, 9.5% had neutropenia, 7.1% had fatty liver and 4.8% of easy bruising.
1 Division of Child Neurology, University of California at San Francisco, San Francisco, CA, USA, 2Division of Epilepsy and Clinical Neurophysiology, University of California at San Francisco, San Francisco, CA, USA, 3Kaiser Permanente of Northern California, San Francisco, CA, USA, 4Unit of Child Neuropsychiatry, Bolzano Hospital, Bolzano, Italy, 5Unit of Muscular and Neurodegenerative Disease, Gaslini Institute, Genova, Italy
Purpose: We sought to characterize the electroclinical phenotype associated with the recently described KCNQ2 encephalopathy and the response to treatment in this rare and severe neonatal-onset epileptic encephalopathy.
Conclusions: Prolonged treatment with KD is associated with significant morbidity including osteopenia, fractures, kidney stones, and acidosis. Metabolic acidosis may contribute to hypercalciuria, osteopenia, fractures and kidney stones. Monitoring of bone health and acidosis is important in this population.
Method: We reviewed the medical records of two patients with KCNQ2 encephalopathy diagnosed in the neonatal period. We examined clinical data and video-EEG recordings to describe seizure semiology and EEG features, responses to different AEDs, as well as developmental and seizures outcomes.
LBP1109 THE EVALUATION OF 12 PATIENTS WITH HIPPOCAMPAL MALROTATION: A SINGLE CENTRE EXPERIENCE € Duman1 P. Gencßpınar1, K. Karaali2, Sß. Haspolat1, O. 1 Department of Pediatric Neurology, Akdeniz University, Antalya, Turkey, 2Department of Radiology, Akdeniz University, Antalya, Turkey
Results: The patients presented with seizure onset within the first days of life with multiple seizures per day. Seizure semiology was characterized by stereotyped head and eye deviation with asymmetric, tonic posturing of the arm and leg affecting alternating sides of the body, associated with apnea and desaturation. Myoclonic seizures or tonic spasms were not observed. Ictal EEG demonstrated recruiting spikes arising mainly from the central regions of either hemisphere, followed by focal spikes and waves, and then profound, transient, post-ictal background attenuation or burst-suppression pattern. Interictal EEG demonstrated lack of organization, almost-continuous multifocal epileptiform abnormalities with random attenuations. Both patients failed a number of anti-epileptic agents, including Phenobarbital, Phenytoin, Benzodiazepines, Topiramate, and Levetiracetam; and, had seizure freedom shortly after initiation of Carbamazepine or Oxcarbazepine.
Purpose: Hippocampal malrotations (HIMAL) are defined as incomplete rotation with normal intensity. The relationship between HIMAL and temporal lobe epilepsy (TLE) is not clear. The aim of this study is to evaluate clinical, electroencephalographic (EEG), and radiological features of the patients with pure HIMAL. Method: The patients who have various symptoms such as headache, seizures and syncope with pure hippocampal malrotation, were included. The demographic, clinical, EEG, and radiological findings of the patients were re-evaluated. Results: Twelve patients (5 girls and 7 boys) were enrolled in the study. The mean age of the patients was 6.70 4.45 years. Of the patients, malrotations were left sided in the nine, right sided in the one and bilateral in the two patients. The most affected part was the body of hippocampus. Ten patients (83.3%) had convulsion (7/3, afebrile/febrile). Nine (90%) of these ten patients had clinically generalized tonic-clonic (GTC) or secondarily generalized seizures, and one of them had dialeptic seizure. EEG was normal in seven patients. Five patients had abnormal EEG findings (One had focal, three had multifocal, and one had generalized epileptic discharges). The cases with multifocal epileptic discharges also had background dysfunction in their EEG. Conclusion: The majority of cases (83.3%) with HIMAL had convulsions with no TLE symptoms. These findings support the idea of coincidental existence of HIMAL in epileptic patients. However, some patients had epileptic encephalopathy findings in EEG that may be a sign of neurogenetic abnormality in HIMAL cases. Further studies are warranted.
LBP1110 KCNQ2 ENCEPHALOPATHY: ELECTROCLINICAL PRESENTATION AND RESPONSE TO TREATMENT IN TWO NEW CASES A. L. Numis1, J. E. Sullivan2, A. J. Lewis3, N. S. Singhal2, G. Casara4, P. Striano5, M. R. Cilio2
Conclusion: Mutations in KCNQ2 can result in both benign and severe epilepsy syndromes. Here, we report he first description of a distinct electroclinical profile of KCNQ2 encephalopathy, disparate from other neonatal epileptic encephalopathies such as Ohtahara syndrome and Early Myoclonic Encephalopathy. We provide evidence of efficacy of Carbamazepine and its derivatives in this disorder.
LBP1111 RECOVERY OF CLOSE BRAIN INJURY AND ACQUIRED APHASIA: A CASE STUDY S. M. Golubovic1 1 Speech and Language Pathology, Faculty of Special Education and Rehabilitation, University of Belgrade, Belgrade, Serbia Purpose: Boy who is 8 years old is second child from the third pregnancy. He was born on time with Caesarian section, with 4300 g of weight, 55 cm height, AS = 8/9. Psychomotor development is normal. Started to walk and speak on time, and started to control sphincters. Method: When he was 3 years old, he had an allergy with Quincke edema, and when he was 4.5 years old he fell from stairs, lost his consciousness, and stopped talking, and since then he is on logopedic treatment. It is hard to establish contact with the boy. Results: He is socially not adapted, hyperactive, with distractible attention, epilepsy, understand speech of other people, knows letters, numbers, quantity and size, understands commands, counts mechanically till 20. Expressive speech is disordered after the head injury. Fine motoric is undeveloped, graphomotory is clumsy, and drawing is in the phase of scribble. He crossing fast from one activity on the other, and he is not interested in treatment. Epilepsia, 54(Suppl. 3):341–347, 2013 doi: 10.1111/epi.12248
350 Late Breaking Abstracts Conclusion: The boy spent 3 years on the logopedic and psychological treatment by the special program, but with small improvement.
LBP1112 RANDOMIZED CONTROLLED TRIAL OF EXTERNAL TRIGEMINAL NERVE STIMULATION (ETNSTM) FOR EPILEPSY: IMPACT ON MOOD AS MEASURED BY THE BECK DEPRESSION INVENTORY C. DeGiorgio1,2, J. Soss1, I. Cook1, C. Kealey2, D. Markovic1, C. Heck3 1 UCLA, Los Angeles, CA, USA, 2NeuroSigma, Inc., Los Angeles, CA, USA, 3University of Southern California, Los Angeles, CA, USA Purpose: Depression is a major co-morbidity in epilepsy, occurring in 30–58% of people with drug resistant epilepsy (DRE). New devices and treatments which improve both mood and seizures are needed. Method: 50 subjects with DRE were enrolled in a phase II randomized trial of external Trigeminal Nerve Stimulation (eTNS). Mood was evaluated using the Beck Depression Inventory (BDI) at baseline and at 6, 12, and 18 weeks during the double blind treatment period. A BDI score < 10 is considered the absence of clinically depressive symptoms (normal range). Results: At study entry, the mean BDI for the treatment group was 16.7 vs. 12.0 for the control group. The mean reduction in BDI score for the 18-week treatment period was 48% for the treatment group vs. 33% for the control group. (p = 0.02, ANOVA). Improvement in mood was noted at 6-weeks, and continued for the 18-week double blind treatment period. The Odds Ratio for remission from depression was 5.5 in the treatment group vs. 1.6 in the control group. Improvement in mood was not correlated with improvement in seizure frequency. Conclusion: External Trigeminal Nerve Stimulation (eTNS) was associated with early and significant improvements in mood. Remission from depressive symptoms was associated with eTNS therapy. Disclosures: Lead funding by the Epilepsy Therapy Project. CMD and CK are employees of NeuroSigma, Inc (NS). CMD, IC, and CK have equity interests In NS and hold intellectual property in TNS.
Psychiatric disorder was diagnosed if there were symptoms during the interictal period. Cognitive disorders were diagnosed in 125 cases (28.5%), psychoses – in 3 cases (0.7%), depressive disorders – in 12 cases (2.7%), and personality disorders – in 298 cases (68%). It is thought necessary to do special research on the basis of representative selections from the general population aimed at defining the role of epileptic activity which causes mental dysfunction and is involved in the pathogenesis of psychic disorders of epileptic patients.
LBP1114 QUALITY OF LIFE IN CHILDREN WITH EPILEPSY AND CEREBRAL PALSY IN SLOVENIA M. Jekovec- Vrhovsek1, D. Gosar1, M. J. Krzan1, L. Vrba1, S. Simic1, D. Neubauer1 1 Department of Child, Adolescent & Developmental Neurology, University Medical Centre Ljubljana, University Children’s Hospital, Ljubljana, Slovenia Purpose: A pilot study to assess quality of life in families with children with cerebral palsy only or with cerebral palsy and epilepsy. Method: Quality of life was identified using a questionnaire on various aspects of everyday life. It was sent to 57 families. There were 20 mothers and 15 fathers among responders. Sixty percent of children whose parents answered the questionnaire had epilepsy. Results: Parents of children with cerebral palsy and epilepsy were worrying more about their child (p = .041), their family relationships were poorer (p = .107) and they reported more difficulties on family functioning scale (p = .057). Seizure severity was associated with poorer emotional functioning of parents (b = 1.20, p = .061) and with more communication problems in family (b = 1.63, p = .04). Adverse effects of antiepileptic drugs were more commonly reported by families with better emotional functioning (b = 1.20, p = .061) and families with better family relationship (b = 1.28, p = .003). The quality of life was getting worse with more co-morbid disorders. Parents of children with two or more co-morbid disorders (blindness or visual impairment, deafness or hearing impairment, speech impairment, sleep disorder, psychiatric disorder, mental disability) reported greater difficulties in their social functioning (p = .046) and in their family’s daily activities (p = .002). Satisfaction with treatment was estimated as good. In both groups quality of information from internet and from medical staff was estimated to be very good.
LBP1113 MENTAL DISORDERS OF PATIENTS WITH EPILEPSY IN BELARUS M. Makhrov1, F. Hlebokazov1, I. Matveichuk1, T. Dakukina1 1 Republic Mental Health Research Center, Minsk, Belarus
Conclusion: Parents of a child with cerebral palsy and epilepsy were more worried about their child and they had more difficulties in living arrangements and child’s education. Both groups had more difficulties in the family social functioning being more obvious when a child has two or more co-morbid disorders. There was no difference in general quality of life scale.
According to the data of the WHO, epilepsy is the most frequent nervous disease. More than 50 million in the world suffer from this disease (2007). About 80 thousand of people (2009) suffer from this disease in the Republic of Belarus, it is about 0.81% of the Belarusian population. Mental disorders represent an important component of the clinic of epilepsy that complicates its course. The investigation of the dynamics and structure of the incidence and prevalence of mental disorders in epilepsy has an important scientific and practical value which allows revealing shortcomings in rendering therapeutic and rehabilitative assistance to the contingent of epileptic patients with psychiatric disorders in the institutions of the psychiatric service. It should be emphasized, there are no special researches, concerning general and primary disease incidence psychiatric disorders of patients with epilepsy in Belarus. According to the 2012 data of Republic Mental Health Research Center, there were 506 patients with paroxysmal state in the epilepsy ward. Among them 438 cases were accompanied by psychiatric disorders.
LBP1115 THE CLINICAL FEATURES AND PROGNOSIS OF PATIENTS WITH NON-CONVULSIVE STATUS EPILEPTICUS IN THE NEURO-INTENSIVE CARE UNIT OF A TERTIARY REFERRAL CENTER IN TURKEY N. Dericioglu1, E. M. Arsava1, M. A. Topcuoglu1 1 Department of Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey
Epilepsia, 54(Suppl. 3):341–347, 2013 doi: 10.1111/epi.12248
Purpose: The availability of video-EEG monitoring (VEEGM) in neuro-intensive care units (NICU) has allowed the recognition and treatment of non-convulsive status epilepticus (NCSE). However, little is
351 Abstracts known about patient characteristic, management and outcome results in patients with NCSE in developing countries. Method: We retrospectively reviewed VEEGM reports of 120 patients who were monitored between November 2009-March 2013. Indications for VEEGM were unexplained alterations of consciousness or witnessed convulsive seizures. We identified the clinical characteristics, treatment regimes and outcome of patients with NCSE and determined which parameters were associated with outcome. Results: NCSE was detected in 12/120 (10%) patients (3F,9M; age = 24–86). Admission diagnoses were: stroke (3), epilepsy (3), autoimmune limbic encephalitis (3), herpes encephalitis (1), presumed encephalitis-cardiac arrest (1), malignancy (1). Six non-epileptic patients had witnessed seizures before VEEGM. At the onset of monitoring Glasgow coma scale (GCS) score was
