Focal epilepsy with ictal abdominal pain: a case report

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Cerminara, Caterina; El Malhany, Nadia; Roberto, Denis; Curatolo, Paolo

2013-01-01

Focal epilepsy with ictal abdominal pain is an unusual partial epilepsy characterized by paroxysmal episodes of abdominal or visceral pain, disturbance of awareness and electroencephalographic abnormalities. We describe a new case of ictal abdominal pain in which gastrointestinal complaints were the only manifestation of seizures and review the previously described pediatric patients. In our patient clinical findings, ictal EEG abnormalities, and a good response to antiepileptic drugs allowed...

Large-scale cortico-subcortical functional networks in focal epilepsies: The role of the basal ganglia

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Eva Výtvarová

2017-01-01

Significance: Focal epilepsies affect large-scale brain networks beyond the epileptogenic zones. Cortico-subcortical functional connectivity disturbance was displayed in LTLE, FLE, and POLE. Significant changes in the resting-state functional connectivity between cortical and subcortical structures suggest an important role of the BG and thalamus in focal epilepsies.

Voxel based morphometry of FLAIR MRI in children with intractable focal epilepsy: Implications for surgical intervention

International Nuclear Information System (INIS)

Riney, Catherine J.; Chong, William K.; Clark, Chris A.; Cross, J. Helen

2012-01-01

Purpose: Magnetic resonance imaging (MRI), in particular fluid-attenuated inversion-recovery (FLAIR), has transformed the delineation of structural brain pathology associated with focal epilepsy. However, to date there is no literature on voxel based morphometry (VBM) of FLAIR in children with epilepsy. The aim of this study was to explore the role of visual and VBM assessment of FLAIR in pre-operative investigation of children with intractable focal epilepsy. Methods: Children with intractable epilepsy due to focal cortical dysplasia (FCD) and children with intractable cryptogenic focal epilepsy (CFE) were investigated. FLAIR and T1-weighted MRI were acquired on a 1.5T MRI scanner (Siemens, Erlangen, Germany). VBM was performed using SPM5 (Wellcome Institute of Cognitive Neuroscience, London). Results: Eight children with FCD (M = 5, age 7.9–17.3 years) and 14 children with CFE (M = 8, 7.8–16.8 years) were enrolled. VBM of FLAIR detected 7/8 (88%) of FCD whilst VBM of T1-weighted MRI detected only 3/8 (38%) FCD. VBM of FLAIR detected abnormality in 4/14 children with CFE, in 2/14 (14%) the abnormality was concordant with other data on the epileptogenic zone and with visible abnormality on repeat visual inspection of MR data. VBM of T1-weighed MRI detected abnormality in 2/14 children with CFE, none of which correlated with visible abnormality. Discussion: This study highlights the important role that FLAIR imaging has in the pre-operative assessment of children with intractable epilepsy. VBM of FLAIR may provide important information allowing selection of children with intractable CFE who are likely to benefit from further neuroradiological or neurophysiological evaluation.

Familial risk of epilepsy: a population-based study

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Peljto, Anna L.; Barker-Cummings, Christie; Vasoli, Vincent M.; Leibson, Cynthia L.; Hauser, W. Allen; Buchhalter, Jeffrey R.

2014-01-01

Almost all previous studies of familial risk of epilepsy have had potentially serious methodological limitations. Our goal was to address these limitations and provide more rigorous estimates of familial risk in a population-based study. We used the unique resources of the Rochester Epidemiology Project to identify all 660 Rochester, Minnesota residents born in 1920 or later with incidence of epilepsy from 1935–94 (probands) and their 2439 first-degree relatives who resided in Olmsted County. We assessed incidence of epilepsy in relatives by comprehensive review of the relatives’ medical records, and estimated age-specific cumulative incidence and standardized incidence ratios for epilepsy in relatives compared with the general population, according to proband and relative characteristics. Among relatives of all probands, cumulative incidence of epilepsy to age 40 was 4.7%, and risk was increased 3.3-fold (95% confidence interval 2.75–5.99) compared with population incidence. Risk was increased to the greatest extent in relatives of probands with idiopathic generalized epilepsies (standardized incidence ratio 6.0) and epilepsies associated with intellectual or motor disability presumed present from birth, which we denoted ‘prenatal/developmental cause’ (standardized incidence ratio 4.3). Among relatives of probands with epilepsy without identified cause (including epilepsies classified as ‘idiopathic’ or ‘unknown cause’), risk was significantly increased for epilepsy of prenatal/developmental cause (standardized incidence ratio 4.1). Similarly, among relatives of probands with prenatal/developmental cause, risk was significantly increased for epilepsies without identified cause (standardized incidence ratio 3.8). In relatives of probands with generalized epilepsy, standardized incidence ratios were 8.3 (95% confidence interval 2.93–15.31) for generalized epilepsy and 2.5 (95% confidence interval 0.92–4.00) for focal epilepsy. In relatives of

Thermography Examination of Abdominal Area Skin Temperatures in Individuals With and Without Focal-Onset Epilepsy.

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King, Hollis H; Cayce, Charles Thomas; Herrin, Jeph

Early osteopathic theory and practice, and the work of the medical intuitive Edgar Cayce suggested that the abdominal areas of individuals with epilepsy would manifest "cold spots." The etiology for this phenomenon was thought to be abdominal adhesions caused by inflammation and viscero-somatic reflexes caused by adhesions or injury to visceral or musculoskeletal system structures. Indeed, until that advent of electroencephalography in the 1930s, medical practice regarding epilepsy focused on abdominal neural and visceral structures. Following two hypotheses were formulated to evaluate any abdominal temperature phenomena: (1) an abdominal quadrant division analysis would find one or more quadrants "colder" in the focal-onset epilepsy group (ICD9-CM 345.4 and 345.5) compared to controls. (2) Total abdominal areas of individuals with focal-onset epilepsy wound be colder than a control group. Overall, 50 patients with the diagnosis of focal-onset epilepsy were recruited from the office of the Epilepsy Foundation of Florida and 50 control subjects with no history of epilepsy were recruited through advertising to the public. Under controlled room conditions all subjects had infrared thermographic images made and recorded by Med-Hot Model MH-731 FLIR equipment. There were no significant demographic difference between experimental patients and control subjects, though the control group tended to be younger and more often male; however, these were controlled for in all analyses. In the quadrant analysis, there were significant differences in that more epileptic patients had colder left upper abdominal quadrant temperatures than the control group (66.8% versus 44.9%; P = .030). In the total abdominal analysis, however, there were no significant differences. The results support the hypothesis that individuals with focal-onset epilepsy have colder abdominal areas. If substantiated in further research, present study results will require further examination of the mechanisms of

Aspectos clínicos y electroencefalográficos de la epilepsia focal en el niño Clinical and electroencephalographic features of focal epilepsy present in children

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Albia Pozo Alonso

2009-06-01

Full Text Available INTRODUCCIÓN. El objetivo del presente trabajo fue caracterizar un grupo de pacientes con epilepsias focales, según aspectos clínicos y electroencefalográficos. MÉTODOS. Se realizó un estudio descriptivo y prospectivo de 185 niños con diagnóstico de epilepsia focal (2 o más crisis epilépticas no provocadas, con edades entre un mes y 14 años, que fueron hospitalizados en el Departamento de Neuropediatría del Hospital «William Soler» entre diciembre de 2001 y diciembre de 2003. RESULTADOS. La edad media de inicio de la primera crisis epiléptica fue de 5 años. El tipo de crisis epiléptica focal más frecuente fue la simple (49,2 %. El 48,6 % de los niños presentó etiología idiopática y el 33,0 % sintomática. Los factores de la etiología sintomática más frecuentes fueron los prenatales (56,2 %. El 91,4 % de los pacientes presentó electroencefalogramas iniciales interictales anormales. El electroencefalograma focal se observó en el 37,3 % de los niños y el multifocal en el 24,9 %. El síndrome epiléptico más frecuente fue la epilepsia benigna con puntas centrotemporales (5,9 %. CONCLUSIONES. Los niños con epilepsia focal tienen variadas manifestaciones clínicas y electroencefalográficas, y en la mayoría de los pacientes no es posible identificar un síndrome epiléptico.INTRODUCTION: The aim of present paper was to characterize a group of patients presenting with focal epilepsies by clinical and electroencephalographic features. METHODS: Authors made a descriptive and cross-sectional study in 185 children diagnosed with focal epilepsy (two or more non-provoked epilepsy crises, aged from one month to 14, admitted in Neurology Department of "William Soler" Children Hospital between December 2001 to December 2003. RESULTS: Mean age of the first epilepsy crisis was at 5 years. The more frequent type of focal epilepsy crisis was the simple one (49, 2%. The 48, 6% of children presented with a idiopathic origin, and the 33

3 Tesla MRI-negative focal epilepsies: Presurgical evaluation, postoperative outcome and predictive factors.

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Kogias, Evangelos; Klingler, Jan-Helge; Urbach, Horst; Scheiwe, Christian; Schmeiser, Barbara; Doostkam, Soroush; Zentner, Josef; Altenmüller, Dirk-Matthias

2017-12-01

To investigate presurgical diagnostic modalities, clinical and seizure outcome as well as predictive factors after resective epilepsy surgery in 3 Tesla MRI-negative focal epilepsies. This retrospective study comprises 26 patients (11 males/15 females, mean age 34±12years, range 13-50 years) with 3 Tesla MRI-negative focal epilepsies who underwent resective epilepsy surgery. Non-invasive and invasive presurgical diagnostic modalities, type and localization of resection, clinical and epileptological outcome with a minimum follow-up of 1year (range 1-11 years, mean 2.5±2.3years) after surgery as well as outcome predictors were evaluated. All patients underwent invasive video-EEG monitoring after implantation of intracerebral depth and/or subdural electrodes. Ten patients received temporal and 16 extratemporal or multilobar (n=4) resections. There was no perioperative death or permanent morbidity. Overall, 12 of 26 patients (46%) were completely seizure-free (Engel IA) and 65% had a favorable outcome (Engel I-II). In particular, seizure-free ratio was 40% in the temporal and 50% in the extratemporal group. In the temporal group, long duration of epilepsy correlated with poor seizure outcome, whereas congruent unilateral FDG-PET hypometabolism correlated with a favorable outcome. In almost two thirds of temporal and extratemporal epilepsies defined as "non-lesional" by 3 Tesla MRI criteria, a favorable postoperative seizure outcome (Engel I-II) can be achieved with accurate multimodal presurgical evaluation including intracranial EEG recordings. In the temporal group, most favorable results were obtained when FDG-PET displayed congruent unilateral hypometabolism. Copyright © 2017 Elsevier B.V. All rights reserved.

White matter integrity and cerebral network topology in focal epilepsy

NARCIS (Netherlands)

Otte, W.M.

2012-01-01

Worldwide more than fifty million people suffer from recurrent spontaneous seizures. Seizures are considered to be harmful to the brain and may have adverse long-term behavioral and cognitive consequences in particular in people with focal epilepsies that do not respond to pharmacotherapy.

Mutations in KCNT1 cause a spectrum of focal epilepsies

DEFF Research Database (Denmark)

Møller, Rikke Steensbjerre; Heron, Sarah E.; Larsen, Line H. G.

2015-01-01

Autosomal dominant mutations in the sodium-gated potassium channel subunit gene KCNT1 have been associated with two distinct seizure syndromes, nocturnal frontal lobe epilepsy (NFLE) and malignant migrating focal seizures of infancy (MMFSI). To further explore the phenotypic spectrum associated w...

Structural and effective connectivity in focal epilepsy

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Christopher S. Parker

2018-01-01

Full Text Available Patients with medically-refractory focal epilepsy may be candidates for neurosurgery and some may require placement of intracranial EEG electrodes to localise seizure onset. Assessing cerebral responses to single pulse electrical stimulation (SPES may give diagnostically useful data. SPES produces cortico-cortical evoked potentials (CCEPs, which infer effective brain connectivity. Diffusion-weighted images and tractography may be used to estimate structural brain connectivity. This combination provides the opportunity to observe seizure onset and its propagation throughout the brain, spreading contiguously along the cortex explored with electrodes, or non-contiguously. We analysed CCEPs and diffusion tractography in seven focal epilepsy patients and reconstructed the effective and structural brain networks. We aimed to assess the inter-modal similarity of the networks at a large scale across the cortex, the effective and structural connectivity of the ictal-onset zone, and investigate potential mechanisms of non-contiguous seizure spread. We found a significant overlap between structural and effective networks. Effective network CCEP amplitude, baseline variation, and outward connectivity was higher at ictal-onset zones, while structural connection strength within the ictal-onset zone tended to be higher. These findings support the concept of hyperexcitable cortex being associated with seizure generation. The high prevalence of structural and effective connections from the ictal-onset zone to sites of non-contiguous spread suggests that macroscopic structural and effective connections are plausible routes for non-contiguous seizure spread.

Autoantibodies to neuronal antigens in children with focal epilepsy and no prima facie signs of encephalitis.

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Borusiak, Peter; Bettendorf, Ulrich; Wiegand, Gert; Bast, Thomas; Kluger, Gerhard; Philippi, Heike; Münstermann, Dieter; Bien, Christian G

2016-07-01

There is increasing awareness of neuronal autoantibodies and their impact on the pathogenesis of epilepsy. We investigated children with focal epilepsy in order to provide an estimate of autoantibody frequency within a pediatric population without prima facie evidence of encephalitis using a broad panel of autoantibodies. This was done to assess the specificity of antibodies and to see whether antibodies might be of modifying influence on the course of focal epilepsies. We searched for autoantibodies in 124 patients with focal epilepsy (1-18 years; mean 10; 6 years). Sera were tested using a broad panel of surface and intracellular antigens. We found autoantibodies in 5/124 patients (4%): high-positive GAD65 antibodies (n = 1), low-positive GAD65 antibodies (N = 1), VGKC complex antibodies not reactive with LGI1 or CASPR2 (n = 3). We did not find any distinctive features distinguishing antibody positive patients from those without antibodies. The antibodies found in this cohort are probably neither disease-specific nor pathogenic. This has been suggested before for these antigenic targets. Moreover, they do not seem to modify disease severity in the antibody-positive epilepsy patients. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Adult-onset epilepsy in focal cortical dysplasia of Taylor type

NARCIS (Netherlands)

Siegel, A. M.; Cascino, G. D.; Elger, C. E.; Devinsky, O.; Laff, R.; Najjar, S.; Sperling, M. R.; LoRusso, G.; Cossu, M.; Urbach, H.; Aronica, E.; Meyer, F. B.; Scheithauer, B. W.; Dubeau, F.; Andermann, F.

2005-01-01

Focal cortical dysplasia of Taylor type (FCDT) usually presents with seizures at an early age, whereas adult onset of epilepsy is uncommon. We reviewed the medical records of 213 patients with FCDT. In 21 patients (10%), age at seizure onset ranged from 18 to 55 years (mean 25.3). The outcome of

Interictal and Postictal Performances on Dichotic Listening Test in Children with Focal Epilepsy

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Carlsson, G.; Wiegand, G.; Stephani, U.

2011-01-01

Dichotic listening test (DL) is an important tool to disclose speech dominance in healthy subjects and in clinical cases. The aim of this study was to probe if focal epilepsy in children reveals a corresponding suppression of the ear reports contralateral to seizure onset site. Thus, 15 children and adolescents with clinically and…

Incidence of lesions as described by MRI in focal epilepsy of frontal and temporal onset

International Nuclear Information System (INIS)

Menzel, C.; Gruenwald, F.; Biersack, H.J.; Ostertun, B.; Solymosi, L.; Schild, H.; Bockisch, A.; Elger, C.E.

1997-01-01

Aim: Today, MRI is an integral part of the presurgical evaluation of patients suffering from partial epilepsy. These patients frequently show focal morphological abnormalities with potential epileptogenic character and surgical resection of these lesions is associated with superior postsurgical outcome as to seizure frequency. Apart from easily detectable defects, such as post-traumatic lesions or cerebral infarction, as wide variety of mainly small abnormalities can be detected using MRI. Methods: In this study, 484 patients suffering from partial epilepsy of temporal or frontal onset were evaluated for the incidence of different lesions in this population. Results: All lesions found were included without evaluating their potential epileptogenicity, which remains to be proven using other procedures (EEG, SPECT, PET, etc.). Involvement of the hippocampal formation was a major finding in temporal lobe epilepsy, which could be detected as sclerosis (T2w-images), atrophy (T2w-TSE or T1w-IR-images) or both (15%). In addition and in declining frequency various tumors (14%), post-traumatic lesion (-5%), and focal cortical dysplasia or other disturbances of cortical integrity (-4%) were found. These lesions are detectable with best contrast on different sequences. As a consequence it is suggested to acquire sequences in 3 dimensions including a T1w-SE, two (coronal and axial) double-echo-SE sequences and similarily two T1w-IR-sequences. The application of contrast media can be restricted to special questions, derived either from the first imaging results or from the patients history. Conclusion: Using qualitative data for interpretation, the sensitivity as to the detection of any focal pathology of a recent-generation MRI in this population was 75%, with 79% for temporal lobe epilepsies and 67% for frontal lobe epilepsies. Quantitative measurements of hippocampal volume or signal seem to be able to increase the sensitivity of the method. (orig.) [de

Germline and somatic mutations in the MTOR gene in focal cortical dysplasia and epilepsy

DEFF Research Database (Denmark)

Møller, Rikke S; Weckhuysen, Sarah; Chipaux, Mathilde

2016-01-01

OBJECTIVE: To assess the prevalence of somatic MTOR mutations in focal cortical dysplasia (FCD) and of germline MTOR mutations in a broad range of epilepsies. METHODS: We collected 20 blood-brain paired samples from patients with FCD and searched for somatic variants using deep-targeted gene panel...... sequencing. Germline mutations in MTOR were assessed in a French research cohort of 93 probands with focal epilepsies and in a diagnostic Danish cohort of 245 patients with a broad range of epilepsies. Data sharing among collaborators allowed us to ascertain additional germline variants in MTOR. RESULTS: We...... detected recurrent somatic variants (p.Ser2215Phe, p.Ser2215Tyr, and p.Leu1460Pro) in the MTOR gene in 37% of participants with FCD II and showed histologic evidence for activation of the mTORC1 signaling cascade in brain tissue. We further identified 5 novel de novo germline missense MTOR variants in 6...

Perampanel for focal epilepsy: insights from early clinical experience

OpenAIRE

Trinka, E.; Steinhoff, B. J.; Nikanorova, M.; Brodie, M. J.

2015-01-01

Perampanel is approved for adjunctive therapy of focal epilepsy with or without secondarily generalized seizures in patients aged >12 years. This narrative review uses real-world and clinical trial data to elucidate perampanel's role in the clinic. Audit data show good tolerability with perampanel and higher freedom-from-seizure rates in elderly vs younger patients. When using perampanel in elderly patients, special attention should be given to comorbidities and co-medication to avoid potenti...

Focal cortical malformations in children with early infantile epilepsy and PCDH19 mutations: case report.

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Kurian, Mary; Korff, Christian M; Ranza, Emmanuelle; Bernasconi, Andrea; Lübbig, Anja; Nangia, Srishti; Ramelli, Gian Paolo; Wohlrab, Gabriele; Nordli, Douglas R; Bast, Thomas

2018-01-01

In this case report we assess the occurrence of cortical malformations in children with early infantile epilepsy associated with variants of the gene protocadherin 19 (PCDH19). We describe the clinical course, and electrographic, imaging, genetic, and neuropathological features in a cohort of female children with pharmacoresistant epilepsy. All five children (mean age 10y) had an early onset of epilepsy during infancy and a predominance of fever sensitive seizures occurring in clusters. Cognitive impairment was noted in four out of five patients. Radiological evidence of cortical malformations was present in all cases and, in two patients, validated by histology. Sanger sequencing and Multiplex Ligation-dependent Probe Amplification analysis of PCDH19 revealed pathogenic variants in four patients. In one patient, array comparative genomic hybridization showed a microdeletion encompassing PCDH19. We propose molecular testing and analysis of PCDH19 in patients with pharmacoresistant epilepsy, with onset in early infancy, seizures in clusters, and fever sensitivity. Structural lesions are to be searched in patients with PCDH19 pathogenic variants. Further, PCDH19 analysis should be considered in epilepsy surgery evaluation even in the presence of cerebral structural lesions. Focal cortical malformations and monogenic epilepsy syndromes may coexist. Structural lesions are to be searched for in patients with protocadherin 19 (PCDH19) pathogenic variants with refractory focal seizures. © 2017 Mac Keith Press.

Epilepsy of infancy with migrating focal seizures: three patients treated with the ketogenic diet.

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Caraballo, Roberto; Noli, Daniel; Cachia, Pedro

2015-06-01

We present three patients with epilepsy of infancy with migrating focal seizures treated with the ketogenic diet. Between February 1, 2012 and January 31, 2014, three patients who met the diagnostic criteria for migrating focal seizures in infancy at our department were placed on the ketogenic diet and followed for a minimum of seven months. Two of the three children responded well to the ketogenic diet. One of these patients became seizure-free and his neuropsychological performance also significantly improved. The other child had a seizure reduction of 75% to 99% with only weekly seizures and moderate psychomotor improvement. For these two patients who responded well to the ketogenic diet, hospital admission was not required. The remaining patient had a seizure reduction of less than 50%. Tolerability of the diet was good in all three patients. Early treatment with the ketogenic diet should be considered for epilepsy of infancy with migrating focal seizures to control seizures and status epilepticus, and avoid progressive cognitive impairment.

[Adult patients treated for focal epilepsy with antiepileptic drugs (AEDs) in combination in France: description according to the 2009 ILAE definition of AED resistance (ESPERA study)].

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Vespignani, H; de Zélicourt, M; Laurendeau, C; Fagnani, F; Levy-Bachelot, L; Murat, C; Kahane, P; de Toffol, B

2014-02-01

To describe the adult population treated with antiepileptic drugs (AEDs) in combination for focal epilepsy according to the definition of AED resistance proposed by the International League Against Epilepsy (ILAE) in 2009 and to evaluate its implementation in current practice. ESPERA was a multicenter, observational, cross-sectional study with a clinical data collection covering the past 12 months conducted by neurologists. Classifications according to AED responsiveness established by investigators for each enrolled patient were revised by two experts. Seventy-one neurologists enrolled 405 patients. Their mean age was 42.7 years (sex-ratioM/F 0.98). According to the investigators, 60% of epilepsies were drug-resistant, 37% drug-responsive and 3% had an undefined drug-responsiveness. After revision of experts, 71% of epilepsies were classified as drug resistant, 22% as responsive and 7% as undefined. Among the participating neurologists, 76% have made at least one error in classifying their patients according to the 2009 ILAE definition of AED resistance. Because of epilepsy, 24% of patients (age≤65) were inactive and 42% could not drive (respectively 29 and 49% of patients with AED resistant epilepsy). Half of patients had at least one other chronic condition. Number of prescribed drugs in combination and health care resource utilisation were significantly higher in patients with drug-resistant epilepsies than in patients with drug responsive epilepsies. ESPERA study shows that the use of new definition of drug-resistance in everyday practice seems difficult without any additional training and that the social and professional disability is frequent in adults with focal epilepsies treated with polytherapy. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Value of repeat brain MRI in children with focal epilepsy and negative findings on initial MRI

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Jeon, Tae Yeon; Kim, Ji Hye; Lee, Jee Hun; Yoo, So Young; Hwang, Sook Min; Lee, Mun Hyang [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

2017-08-01

To evaluate the value of repeat brain magnetic resonance imaging (MRI) in identifying potential epileptogenic lesions in children with initial MRI-negative focal epilepsy. Our Institutional Review Board approved this retrospective study and waived the requirement for informed consent. During a 15-year period, 257 children (148 boys and 109 girls) with initial MRI-negative focal epilepsy were included. After re-evaluating both initial and repeat MRIs, positive results at repeat MRI were classified into potential epileptogenic lesions (malformation of cortical development and hippocampal sclerosis) and other abnormalities. Contributing factors for improved lesion conspicuity of the initially overlooked potential epileptogenic lesions were analyzed and classified into lesion factors and imaging factors. Repeat MRI was positive in 21% (55/257) and negative in 79% cases (202/257). Of the positive results, potential epileptogenic lesions comprised 49% (27/55) and other abnormalities comprised 11% of the cases (28/257). Potential epileptogenic lesions included focal cortical dysplasia (n = 11), hippocampal sclerosis (n = 10), polymicrogyria (n = 2), heterotopic gray matter (n = 2), microlissencephaly (n = 1), and cortical tumor (n = 1). Of these, seven patients underwent surgical resection. Contributing factors for new diagnoses were classified as imaging factors alone (n = 6), lesion factors alone (n = 2), both (n = 18), and neither (n = 1). Repeat MRI revealed positive results in 21% of the children with initial MRI-negative focal epilepsy, with 50% of the positive results considered as potential epileptogenic lesions. Enhanced MRI techniques or considering the chronological changes of lesions on MRI may improve the diagnostic yield for identification of potential epileptogenic lesions on repeat MRI.

Value of repeat brain MRI in children with focal epilepsy and negative findings on initial MRI

International Nuclear Information System (INIS)

Jeon, Tae Yeon; Kim, Ji Hye; Lee, Jee Hun; Yoo, So Young; Hwang, Sook Min; Lee, Mun Hyang

2017-01-01

To evaluate the value of repeat brain magnetic resonance imaging (MRI) in identifying potential epileptogenic lesions in children with initial MRI-negative focal epilepsy. Our Institutional Review Board approved this retrospective study and waived the requirement for informed consent. During a 15-year period, 257 children (148 boys and 109 girls) with initial MRI-negative focal epilepsy were included. After re-evaluating both initial and repeat MRIs, positive results at repeat MRI were classified into potential epileptogenic lesions (malformation of cortical development and hippocampal sclerosis) and other abnormalities. Contributing factors for improved lesion conspicuity of the initially overlooked potential epileptogenic lesions were analyzed and classified into lesion factors and imaging factors. Repeat MRI was positive in 21% (55/257) and negative in 79% cases (202/257). Of the positive results, potential epileptogenic lesions comprised 49% (27/55) and other abnormalities comprised 11% of the cases (28/257). Potential epileptogenic lesions included focal cortical dysplasia (n = 11), hippocampal sclerosis (n = 10), polymicrogyria (n = 2), heterotopic gray matter (n = 2), microlissencephaly (n = 1), and cortical tumor (n = 1). Of these, seven patients underwent surgical resection. Contributing factors for new diagnoses were classified as imaging factors alone (n = 6), lesion factors alone (n = 2), both (n = 18), and neither (n = 1). Repeat MRI revealed positive results in 21% of the children with initial MRI-negative focal epilepsy, with 50% of the positive results considered as potential epileptogenic lesions. Enhanced MRI techniques or considering the chronological changes of lesions on MRI may improve the diagnostic yield for identification of potential epileptogenic lesions on repeat MRI

Mutations in SLC12A5 in epilepsy of infancy with migrating focal seizures

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Stödberg, Tommy; McTague, Amy; Ruiz, Arnaud J.; Hirata, Hiromi; Zhen, Juan; Long, Philip; Farabella, Irene; Meyer, Esther; Kawahara, Atsuo; Vassallo, Grace; Stivaros, Stavros M.; Bjursell, Magnus K.; Stranneheim, Henrik; Tigerschiöld, Stephanie; Persson, Bengt; Bangash, Iftikhar; Das, Krishna; Hughes, Deborah; Lesko, Nicole; Lundeberg, Joakim; Scott, Rod C.; Poduri, Annapurna; Scheffer, Ingrid E.; Smith, Holly; Gissen, Paul; Schorge, Stephanie; Reith, Maarten E. A.; Topf, Maya; Kullmann, Dimitri M.; Harvey, Robert J.; Wedell, Anna; Kurian, Manju A.

2015-01-01

The potassium-chloride co-transporter KCC2, encoded by SLC12A5, plays a fundamental role in fast synaptic inhibition by maintaining a hyperpolarizing gradient for chloride ions. KCC2 dysfunction has been implicated in human epilepsy, but to date, no monogenic KCC2-related epilepsy disorders have been described. Here we show recessive loss-of-function SLC12A5 mutations in patients with a severe infantile-onset pharmacoresistant epilepsy syndrome, epilepsy of infancy with migrating focal seizures (EIMFS). Decreased KCC2 surface expression, reduced protein glycosylation and impaired chloride extrusion contribute to loss of KCC2 activity, thereby impairing normal synaptic inhibition and promoting neuronal excitability in this early-onset epileptic encephalopathy. PMID:26333769

The phenotypic spectrum of ARHGEF9 includes intellectual disability, focal epilepsy and febrile seizures.

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Klein, Karl Martin; Pendziwiat, Manuela; Eilam, Anda; Gilad, Ronit; Blatt, Ilan; Rosenow, Felix; Kanaan, Moien; Helbig, Ingo; Afawi, Zaid

2017-07-01

Mutations or structural genomic alterations of the X-chromosomal gene ARHGEF9 have been described in male and female patients with intellectual disability. Hyperekplexia and epilepsy were observed to a variable degree, but incompletely described. Here, we expand the phenotypic spectrum of ARHGEF9 by describing a large Ethiopian-Jewish family with epilepsy and intellectual disability. The four affected male siblings, their unaffected parents and two unaffected female siblings were recruited and phenotyped. Parametric linkage analysis was performed using SNP microarrays. Variants from exome sequencing in two affected individuals were confirmed by Sanger sequencing. All affected male siblings had febrile seizures from age 2-3 years and intellectual disability. Three developed afebrile seizures between age 7-17 years. Three showed focal seizure semiology. None had hyperekplexia. A novel ARHGEF9 variant (c.967G>A, p.G323R, NM_015185.2) was hemizygous in all affected male siblings and heterozygous in the mother. This family reveals that the phenotypic spectrum of ARHGEF9 is broader than commonly assumed and includes febrile seizures and focal epilepsy with intellectual disability in the absence of hyperekplexia or other clinically distinguishing features. Our findings suggest that pathogenic variants in ARHGEF9 may be more common than previously assumed in patients with intellectual disability and mild epilepsy.

Polymicrogyria-associated epilepsy: a multi-center phenotypic study from the Epilepsy Phenome/Genome Project

Science.gov (United States)

Shain, Catherine; Ramgopal, Sriram; Fallil, Zianka; Parulkar, Isha; Alongi, Richard; Knowlton, Robert; Poduri, Annapurna

2013-01-01

Purpose Polymicrogyria (PMG) is an epileptogenic malformation of cortical development. We describe the clinical epilepsy and imaging features of a large cohort with PMG-related epilepsy. Methods Participants were recruited through the Epilepsy Phenome/Genome Project, a multi-center collaborative effort to collect detailed phenotypic data on individuals with epilepsy. We reviewed phenotypic data from participants with epilepsy and PMG. Key Findings We identified 87 participants, 43 female and 44 male, with PMG and epilepsy. Median age of seizure onset was 3 years (range <1 month-37 years). Most presented with focal epilepsy (87.4%), some in combination with seizures generalized from onset (23.0%). Focal seizures with dyscognitive features were most common (54.3%). Of those presenting with generalized seizure types, infantile spasms were most prevalent (45.2%). The most common topographic pattern was perisylvian PMG (77.0%), of which the majority was bilateral (56.7%). Generalized PMG presented with an earlier age of seizure onset (median age of 8 months) and an increased prevalence of developmental delay prior to seizure onset (57.1%). Of the focal, unilateral and asymmetric bilateral groups where PMG was more involved in one hemisphere, the majority (71.4%) of participants had seizures that lateralized to the same hemisphere as the PMG or the hemisphere with greater involvement. Significance Participants with PMG had both focal and generalized onset of seizures. Our data confirm the involvement of known topographic patterns of PMG and suggest that more extensive distributions of PMG present with an earlier age of seizure onset and increased prevalence of developmental delay prior to seizure onset. PMID:23750890

Transient attenuation of visual evoked potentials during focal status epilepticus in a patient with occipital lobe epilepsy.

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Tsai, Meng-Han; Hsu, Shih-Pin; Huang, Chi-Ren; Chang, Chen-Sheng; Chuang, Yao-Chung

2010-06-01

Seizures originating in the occipital areas are relatively uncommon. They are usually characterized by visual hallucinations and illusions or other symptoms related to the eyes and vision. In a 54-year-old woman with occipital lobe epilepsy, complex visual hallucinations, illusions, and migraine-like headache constitute the major clinical manifestations. During focal status epilepticus, ictal electroencephalography revealed rhythmic focal spikes in the right occipital region, rapidly propagating to the right parietal and contralateral occipital areas. Ictal brain single-photon emission computed topography revealed hyperperfusion of the right occipital region. Using a full-field pattern-shift visual evoked potential (VEP) study, we found that the P100 responses on both sides were markedly attenuated in amplitude during occipital focal status epilepticus, whereas the latencies of the VEPs were normal. The amplitude and morphology of P100 responses on both sides, however, returned to the normal range 7 days after cessation of the seizures. In addition to clinical seizure semiology, scalp EEG, SPECT and neuroimaging studies, VEP studies may be used as a supplementary examination tool to provide further information in the patients with occipital lobe seizures or epilepsies.

Comparative Long-Term Effectiveness of a Monotherapy with Five Antiepileptic Drugs for Focal Epilepsy in Adult Patients: A Prospective Cohort Study

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Zhu, Pan; He, Ru-Qian; Bao, Yi-Xin; Zheng, Rong-Yuan; Xu, Hui-Qin

2015-01-01

Objective To evaluate and compare long-term effectiveness of five antiepileptic drugs (AEDs) for monotherapy of adult patients with focal epilepsy in routine clinical practice. Methods Adult patients with focal epilepsy, who were prescribed with carbamazepine (CBZ), valproate (VPA), lamotrigine (LTG), topiramate (TPM), or oxcarbazepine (OXC) as monotherapy, during the period from January 2004 to June 2012 registered in Wenzhou Epilepsy Follow Up Registry Database (WEFURD), were included in the study. Prospective long-term follow-up was conducted until June 2013. The endpoints were time to treatment failure, time to seizure remission, and time to first seizure. Results This study included 654 patients: CBZ (n=125), VPA (n=151), LTG (n=135), TPM (n=76), and OXC (n=167). The retention rates of CBZ, VPA, LTG, TPM, and OXC at the third year were 36.1%, 32.4%, 57.6%, 37.9%, and 41.8%, respectively. For time to treatment failure, LTG was significantly better than CBZ and VPA (LTG vs. CBZ, hazard ratio, [HR] 0.80 [95% confidence interval: 0.67-0.96], LTG vs. VPA, 0.53 [0.37-0.74]); TPM was worse than LTG (TPM vs. LTG, 1.77 [1.15-2.74]), and OXC was better than VPA (0.86 [0.78-0.96]). After initial target doses, the seizure remission rates of CBZ, VPA, LTG, TPM, and OXC were 63.0%, 77.0%, 83.6%, 67.9%, and 75.3%, respectively. LTG was significantly better than CBZ (1.44 [1.15-1.82]) and OXC (LTG vs. OXC, 0.76 [0.63-0.93]); OXC was less effective than LTG in preventing the first seizure (1.20 [1.02-1.40]). Conclusion LTG was the best, OXC was better than VPA only, while VPA was the worst. The others were equivalent for comparisons between five AEDs regarding the long-term treatment outcomes of monotherapy for adult patients with focal epilepsy in a clinical practice. For selecting AEDs for these patients among the first-line drugs, LTG is an appropriate first choice; others are reservation in the first-line but VPA is not. PMID:26147937

Comparative Long-Term Effectiveness of a Monotherapy with Five Antiepileptic Drugs for Focal Epilepsy in Adult Patients: A Prospective Cohort Study.

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Qing-Yi Zeng

Full Text Available To evaluate and compare long-term effectiveness of five antiepileptic drugs (AEDs for monotherapy of adult patients with focal epilepsy in routine clinical practice.Adult patients with focal epilepsy, who were prescribed with carbamazepine (CBZ, valproate (VPA, lamotrigine (LTG, topiramate (TPM, or oxcarbazepine (OXC as monotherapy, during the period from January 2004 to June 2012 registered in Wenzhou Epilepsy Follow Up Registry Database (WEFURD, were included in the study. Prospective long-term follow-up was conducted until June 2013. The endpoints were time to treatment failure, time to seizure remission, and time to first seizure.This study included 654 patients: CBZ (n=125, VPA (n=151, LTG (n=135, TPM (n=76, and OXC (n=167. The retention rates of CBZ, VPA, LTG, TPM, and OXC at the third year were 36.1%, 32.4%, 57.6%, 37.9%, and 41.8%, respectively. For time to treatment failure, LTG was significantly better than CBZ and VPA (LTG vs. CBZ, hazard ratio, [HR] 0.80 [95% confidence interval: 0.67-0.96], LTG vs. VPA, 0.53 [0.37-0.74]; TPM was worse than LTG (TPM vs. LTG, 1.77 [1.15-2.74], and OXC was better than VPA (0.86 [0.78-0.96]. After initial target doses, the seizure remission rates of CBZ, VPA, LTG, TPM, and OXC were 63.0%, 77.0%, 83.6%, 67.9%, and 75.3%, respectively. LTG was significantly better than CBZ (1.44 [1.15-1.82] and OXC (LTG vs. OXC, 0.76 [0.63-0.93]; OXC was less effective than LTG in preventing the first seizure (1.20 [1.02-1.40].LTG was the best, OXC was better than VPA only, while VPA was the worst. The others were equivalent for comparisons between five AEDs regarding the long-term treatment outcomes of monotherapy for adult patients with focal epilepsy in a clinical practice. For selecting AEDs for these patients among the first-line drugs, LTG is an appropriate first choice; others are reservation in the first-line but VPA is not.

Slow Activity in Focal Epilepsy During Sleep and Wakefulness

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Pellegrino, Giovanni; Tombini, Mario; Curcio, Giuseppe

2017-01-01

Introduction We aimed to test differences between healthy subjects and patients with respect to slow wave activity during wakefulness and sleep. Methods Fifteen patients affected by nonlesional focal epilepsy originating within temporal areas and fourteen matched controls underwent a 24-hour EEG....... The effect was widespread for alpha band and above, while localized over the affected hemisphere for delta (sleep cycle 1, P = .006; sleep cycle 2, P = .008; sleep cycle 3, P = .017). The analysis of interhemispheric differences showed that the only frequency band stronger over the affected regions...

Ear-EEG detects ictal and interictal abnormalities in focal and generalized epilepsy

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Zibrandtsen, I. C.; Kidmose, P.; Christensen, C. B.

2017-01-01

-EEG and scalp-EEG from 15 patients with suspected temporal lobe epilepsy. EEGs were compared visually by independent neurophysiologists. Correlation and time-frequency analysis was used to quantify the similarity between ear and scalp electrodes. Spike-averages were used to assess similarity of interictal...... and frequency dynamics can be observed from visual inspection and time-frequency analysis. Spike averages derived from ear-EEG electrodes yield a recognizable spike appearance. Conclusions Our results suggest that ear-EEG can reliably detect electroencephalographic patterns associated with focal temporal lobe...... seizures. Interictal spike morphology from sufficiently large temporal spike sources can be sampled using ear-EEG. Significance Ear-EEG is likely to become an important tool in clinical epilepsy monitoring and diagnosis....

Interval analysis of interictal EEG: pathology of the alpha rhythm in focal epilepsy

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Pyrzowski, Jan; Siemiński, Mariusz; Sarnowska, Anna; Jedrzejczak, Joanna; Nyka, Walenty M.

2015-11-01

The contemporary use of interictal scalp electroencephalography (EEG) in the context of focal epilepsy workup relies on the visual identification of interictal epileptiform discharges. The high-specificity performance of this marker comes, however, at a cost of only moderate sensitivity. Zero-crossing interval analysis is an alternative to Fourier analysis for the assessment of the rhythmic component of EEG signals. We applied this method to standard EEG recordings of 78 patients divided into 4 subgroups: temporal lobe epilepsy (TLE), frontal lobe epilepsy (FLE), psychogenic nonepileptic seizures (PNES) and nonepileptic patients with headache. Interval-analysis based markers were capable of effectively discriminating patients with epilepsy from those in control subgroups (AUC~0.8) with diagnostic sensitivity potentially exceeding that of visual analysis. The identified putative epilepsy-specific markers were sensitive to the properties of the alpha rhythm and displayed weak or non-significant dependences on the number of antiepileptic drugs (AEDs) taken by the patients. Significant AED-related effects were concentrated in the theta interval range and an associated marker allowed for identification of patients on AED polytherapy (AUC~0.9). Interval analysis may thus, in perspective, increase the diagnostic yield of interictal scalp EEG. Our findings point to the possible existence of alpha rhythm abnormalities in patients with epilepsy.

EFFICACY OF RUFINAMIDE IN THE TREATMENT OF DRUG-RESISTANT FOCAL EPILEPSIES IN PAEDIATRIC PRACTICE

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I. O. Shchederkina

2016-01-01

Full Text Available Among drug-resistant epilepsies, epileptic syndromes, characterized by combination of several types of seizures, are considered to be the most difficult in terms of treatment. Lennox–Gastaut syndrome is one of them. It manifests with polymorphic seizures (tonic axial, myatonic, atypical absence seizures, status epilepticus of minor motor seizures, myoclonic, generalized convulsive, and focal seizures. This is a heterogeneous disease, represented by a complex of clinical and electroencephalographic manifestations with various etiology. Current review is devoted to a novel antiepileptic drug rufinamide, which has a new mechanism of action. The drug has been registered in Russia in 2015. The authors also describe their own experience of rufinamide usage in the treatment of drug-resistant focal epilepsy as a part of multicomponent therapy for polymorphic seizures. One patient achieved clinical remission for 16 months; the second one had more than 50 % decrease in seizures frequency with a remission of drop-attacks.

Genetic determinants of common epilepsies

DEFF Research Database (Denmark)

2014-01-01

and insufficient power. We aimed to identify risk loci through meta-analyses of genome-wide association studies for all epilepsy and the two largest clinical subtypes (genetic generalised epilepsy and focal epilepsy). METHODS: We combined genome-wide association data from 12 cohorts of individuals with epilepsy...... not previously implicated in epilepsy and provides further evidence about the genetic architecture of these disorders, with the ultimate aim of assisting in disease classification and prognosis. The data suggest that specific loci can act pleiotropically raising risk for epilepsy broadly, or can have effects...... and controls from population-based datasets. Controls were ethnically matched with cases. We phenotyped individuals with epilepsy into categories of genetic generalised epilepsy, focal epilepsy, or unclassified epilepsy. After standardised filtering for quality control and imputation to account for different...

Differences in Memory Functioning between Children with Attention-Deficit/Hyperactivity Disorder and/or Focal Epilepsy

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Lee, Sylvia E.; Kibby, Michelle Y.; Cohen, Morris J.; Stanford, Lisa; Park, Yong; Strickland, Suzanne

2016-01-01

Prior research has shown that attention-deficit/hyperactivity disorder (ADHD) and epilepsy are frequently comorbid and that both disorders are associated with various attention and memory problems. Nonetheless, limited research has been conducted comparing the two disorders in one sample to determine unique versus shared deficits. Hence, we investigated differences in working memory and short-term and delayed recall between children with ADHD, focal epilepsy of mixed foci, comorbid ADHD/epilepsy and controls. Participants were compared on the Core subtests and the Picture Locations subtest of the Children’s Memory Scale (CMS). Results indicated that children with ADHD displayed intact verbal working memory and long-term memory (LTM), as well as intact performance on most aspects of short-term memory (STM). They performed worse than controls on Numbers Forward and Picture Locations, suggesting problems with focused attention and simple span for visual-spatial material. Conversely, children with epilepsy displayed poor focused attention and STM regardless of modality assessed, which affected encoding into LTM. The only loss over time was found for passages (Stories). Working memory was intact. Children with comorbid ADHD/epilepsy displayed focused attention and STM/LTM problems consistent with both disorders, having the lowest scores across the four groups. Hence, focused attention and visual-spatial span appear to be affected in both disorders, whereas additional STM/encoding problems are specific to epilepsy. Children with comorbid ADHD/epilepsy have deficits consistent with both disorders, with slight additive effects. This study suggests that attention and memory testing should be a regular part of the evaluation of children with epilepsy and ADHD. PMID:26156331

Heterogeneous contribution of microdeletions in the development of common generalised and focal epilepsies

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Pérez-Palma, Eduardo; Helbig, Ingo; Klein, Karl Martin; Anttila, Verneri; Horn, Heiko; Reinthaler, Eva Maria; Gormley, Padhraig; Ganna, Andrea; Byrnes, Andrea; Pernhorst, Katharina; Toliat, Mohammad R; Saarentaus, Elmo; Howrigan, Daniel P; Hoffman, Per; Miquel, Juan Francisco; De Ferrari, Giancarlo V; Nürnberg, Peter; Lerche, Holger; Zimprich, Fritz; Neubauer, Bern A; Becker, Albert J; Rosenow, Felix; Perucca, Emilio; Zara, Federico; Weber, Yvonne G; Lal, Dennis

2017-01-01

Background Microdeletions are known to confer risk to epilepsy, particularly at genomic rearrangement ‘hotspot’ loci. However, microdeletion burden not overlapping these regions or within different epilepsy subtypes has not been ascertained. Objective To decipher the role of microdeletions outside hotspots loci and risk assessment by epilepsy subtype. Methods We assessed the burden, frequency and genomic content of rare, large microdeletions found in a previously published cohort of 1366 patients with genetic generalised epilepsy (GGE) in addition to two sets of additional unpublished genome-wide microdeletions found in 281 patients with rolandic epilepsy (RE) and 807 patients with adult focal epilepsy (AFE), totalling 2454 cases. Microdeletions were assessed in a combined and subtype-specific approaches against 6746 controls. Results When hotspots are considered, we detected an enrichment of microdeletions in the combined epilepsy analysis (adjusted p=1.06×10−6,OR 1.89, 95% CI 1.51 to 2.35). Epilepsy subtype-specific analyses showed that hotspot microdeletions in the GGE subgroup contribute most of the overall signal (adjusted p=9.79×10−12, OR 7.45, 95% CI 4.20–13.5). Outside hotspots , microdeletions were enriched in the GGE cohort for neurodevelopmental genes (adjusted p=9.13×10−3,OR 2.85, 95% CI 1.62–4.94). No additional signal was observed for RE and AFE. Still, gene-content analysis identified known (NRXN1, RBFOX1 and PCDH7) and novel (LOC102723362) candidate genes across epilepsy subtypes that were not deleted in controls. Conclusions Our results show a heterogeneous effect of recurrent and non-recurrent microdeletions as part of the genetic architecture of GGE and a minor contribution in the aetiology of RE and AFE. PMID:28756411

Heterogeneous contribution of microdeletions in the development of common generalised and focal epilepsies.

Science.gov (United States)

Pérez-Palma, Eduardo; Helbig, Ingo; Klein, Karl Martin; Anttila, Verneri; Horn, Heiko; Reinthaler, Eva Maria; Gormley, Padhraig; Ganna, Andrea; Byrnes, Andrea; Pernhorst, Katharina; Toliat, Mohammad R; Saarentaus, Elmo; Howrigan, Daniel P; Hoffman, Per; Miquel, Juan Francisco; De Ferrari, Giancarlo V; Nürnberg, Peter; Lerche, Holger; Zimprich, Fritz; Neubauer, Bern A; Becker, Albert J; Rosenow, Felix; Perucca, Emilio; Zara, Federico; Weber, Yvonne G; Lal, Dennis

2017-09-01

Microdeletions are known to confer risk to epilepsy, particularly at genomic rearrangement 'hotspot' loci. However, microdeletion burden not overlapping these regions or within different epilepsy subtypes has not been ascertained. To decipher the role of microdeletions outside hotspots loci and risk assessment by epilepsy subtype. We assessed the burden, frequency and genomic content of rare, large microdeletions found in a previously published cohort of 1366 patients with genetic generalised epilepsy (GGE) in addition to two sets of additional unpublished genome-wide microdeletions found in 281 patients with rolandic epilepsy (RE) and 807 patients with adult focal epilepsy (AFE), totalling 2454 cases. Microdeletions were assessed in a combined and subtype-specific approaches against 6746 controls. When hotspots are considered, we detected an enrichment of microdeletions in the combined epilepsy analysis (adjusted p=1.06×10 -6 ,OR 1.89, 95% CI 1.51 to 2.35). Epilepsy subtype-specific analyses showed that hotspot microdeletions in the GGE subgroup contribute most of the overall signal (adjusted p=9.79×10 -12 , OR 7.45, 95% CI 4.20-13.5). Outside hotspots , microdeletions were enriched in the GGE cohort for neurodevelopmental genes (adjusted p=9.13×10 -3 ,OR 2.85, 95% CI 1.62-4.94). No additional signal was observed for RE and AFE. Still, gene-content analysis identified known ( NRXN1 , RBFOX1 and PCDH7 ) and novel ( LOC102723362 ) candidate genes across epilepsy subtypes that were not deleted in controls. Our results show a heterogeneous effect of recurrent and non-recurrent microdeletions as part of the genetic architecture of GGE and a minor contribution in the aetiology of RE and AFE. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Real-life memory and spatial navigation in patients with focal epilepsy: ecological validity of a virtual reality supermarket task.

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Grewe, P; Lahr, D; Kohsik, A; Dyck, E; Markowitsch, H J; Bien, C G; Botsch, M; Piefke, M

2014-02-01

Ecological assessment and training of real-life cognitive functions such as visual-spatial abilities in patients with epilepsy remain challenging. Some studies have applied virtual reality (VR) paradigms, but external validity of VR programs has not sufficiently been proven. Patients with focal epilepsy (EG, n=14) accomplished an 8-day program in a VR supermarket, which consisted of learning and buying items on a shopping list. Performance of the EG was compared with that of healthy controls (HCG, n=19). A comprehensive neuropsychological examination was administered. Real-life performance was investigated in a real supermarket. Learning in the VR supermarket was significantly impaired in the EG on different VR measures. Delayed free recall of products did not differ between the EG and the HCG. Virtual reality scores were correlated with neuropsychological measures of visual-spatial cognition, subjective estimates of memory, and performance in the real supermarket. The data indicate that our VR approach allows for the assessment of real-life visual-spatial memory and cognition in patients with focal epilepsy. The multimodal, active, and complex VR paradigm may particularly enhance visual-spatial cognitive resources. Copyright © 2013 Elsevier Inc. All rights reserved.

KANSL1 variation is not a major contributing factor in self-limited focal epilepsy syndromes of childhood.

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Kenneth A Myers

Full Text Available KANSL1 haploinsufficiency causes Koolen-de Vries syndrome (KdVS, characterized by dysmorphic features and intellectual disability; amiable personality, congenital malformations and seizures also commonly occur. The epilepsy phenotypic spectrum in KdVS is broad, but most individuals have focal seizures with some having a phenotype resembling the self-limited focal epilepsies of childhood (SFEC. We hypothesized that variants in KANSL1 contribute to pathogenesis of SFEC.We screened KANSL1 for single nucleotide variants in 90 patients with SFEC. We then screened a cohort of 208 patients with two specific SFEC syndromes, childhood epilepsy with centrotemporal spikes (CECTS and atypical childhood epilepsy with centrotemporal spikes (ACECTS for KANSL1 variants. The second cohort was also used to evaluate minor allelic variants that appeared overrepresented in the initial cohort.One variant, p.Lys104Thr, was predicted damaging and appeared overrepresented in our 90-patient cohort compared to Genome Aggregation Database (gnomAD allele frequency (0.217 to 0.116, with no homozygotes in gnomAD. However, there was no difference in p.Lys104Thr allele frequency in the follow-up CECTS/ACECTS cohort and controls. Four rare KANSL1 variants of uncertain significance were identified in the CECTS/ACECTS cohort.Our data do not support a major role for KANSL1 variants in pathogenesis of SFEC.

In vivo inhibition of incorporation of (U-/sup 14/C)glucose into proteins in experimental focal epilepsy

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Coutinho-Netto, J.; Boyar, M.M.; Abdul-Ghani, A.S.; Bradford, H.F.

1982-08-01

The in vivo incorporation of (/sup 14/C) from (U-/sup 14/C)-glucose into rat brain proteins from different cortical areas was examined in three different experimental focal epilepsies: cobalt, freeze-lesions, and tityustoxin. When (U-/sup 14/C)-glucose was injected intraperitoneally into awake and unrestrained animals with marked signs of epileptic hyperactivity, the inhibition of incorporation of (/sup 14/C)-amino acids into trichloracetic acid (TCA)-insoluble proteins was highest in the focal (sensorimotor) area when compared with distant regions (approx. 60%), but less when compared with the contralateral (sensorimotor) region (approx. 23%). Greatly decreased incorporation caused by both cobalt and freeze-lesion-induced epilepsies was also observed in the contralateral area when a comparison was made with distant regions (approx. 50%), but there were no significant differences in protein-specific radioactivity between the different distant areas.

FOCAL CORTICAL DYSPLASIAS: CLINICAL AND ELECTRO-NEUROIMAGING CHARACTERISTICS

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K. Yu. Mukhin

2016-01-01

Full Text Available In spite of a notable advance made in epileptology, resistant epilepsies account for approximately 30 % of all forms of epilepsy particularly in patients with focal seizures. One of the main causes of therapy-resistant focal epilepsies is focal cortical dysplasias (FCD. This term was first introduced by D. Taylor et al. in 1971. FCD belongs to abnormal cortical development. Among all abnormalities of cortical development, FCD in surgically treated children amounts to 75 %. FCD is the most common cause of resistant epilepsy in children and the most frequent reason for diagnosing cryptogenic focal epilepsy with intractable seizures. The author gives a detailed literature review dedicated to FCD as a cause of resistant epilepsy, including the classification and histologic characteristics of FCD, its clinical manifestations and prognosis, and approaches to medical and surgical treatments. 

Partial monosomy Xq(Xq23 --> qter) and trisomy 4p(4p15.33 --> pter) in a woman with intractable focal epilepsy, borderline intellectual functioning, and dysmorphic features.

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Bartocci, Arnaldo; Striano, Pasquale; Mancardi, Maria Margherita; Fichera, Marco; Castiglia, Lucia; Galesi, Ornella; Michelucci, Roberto; Elia, Maurizio

2008-06-01

Studies of epilepsy associated with chromosomal abnormalities may provide information about clinical and EEG phenotypes and possibly to identify new epilepsy genes. We describe a female patient with intractable focal epilepsy, borderline intellectual functioning, and facial dysmorphisms, in whom genetic study (i.e., karyotype and array-CGH analysis) revealed a distal trisomy 4p and distal monosomy Xq. Although any genetic hypothesis remains speculative, several genes are located in the 4p chromosome segment involved in the rearrangement, some of which may be related to epilepsy.

Expression and cellular distribution of multidrug transporter proteins in two major causes of medically intractable epilepsy: Focal cortical dysplasia and glioneuronal tumors

NARCIS (Netherlands)

Aronica, E.; Gorter, J. A.; Jansen, G. H.; van Veelen, C. W. M.; van Rijen, P. C.; Leenstra, S.; Ramkema, M.; Scheffer, G. L.; Scheper, R. J.; Troost, D.

2003-01-01

The cell-specific distribution of multidrug resistance extrusion pumps was studied in developmental glioneuronal lesions, including focal cortical dysplasia (15 cases) and ganglioglioma (15 cases) from patients with medically intractable epilepsy. Lesional, perilesional, as well as normal brain

Characterization of Functional and Structural Integrity in Experimental Focal Epilepsy: Reduced Network Efficiency Coincides with White Matter Changes

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Otte, W.M.; Dijkhuizen, R.M.; van Meer, M.P.A.; Van der Hel, W.S.; Verlinde, S.A.M.W.; van Nieuwenhuizen, O.; Viergever, M.A.; Stam, C.J.; Braun, K.P.J.

2012-01-01

Background: Although focal epilepsies are increasingly recognized to affect multiple and remote neural systems, the underlying spatiotemporal pattern and the relationships between recurrent spontaneous seizures, global functional connectivity, and structural integrity remain largely unknown.

Experimental focal neocortical epilepsy is associated with reduced white matter volume growth : results from multiparametric MRI analysis

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Otte, Wim; van Meer, Maurits P A; van der Marel, Kajo; Zwartbol, René; Viergever, Max A.; Braun, Kees P J; Dijkhuizen, Rick M.

2015-01-01

Focal epilepsy has recently been associated with remote white matter damage, including reduced white matter volume. Longitudinal assessment of these white matter changes, in relation to functional mechanisms and consequences, may be ideally done by in vivo neuroimaging in well-controlled

COMPARATIVE EFFICАCY AND TOLERABILITY OF MONOTHERAPY WITH DEPAKINE CHRONOSPHERE, DRUGS OF CARBAMAZEPINE GROUP WITH EXTENDED RELEASE AND OXCARBAZEPINE IN SYMPTOMATIC AND CRYPTOGENIC FOCAL EPILEPSY (SVT. LUKA’S INSTITUTE OF CHILD NEUROLOGY AND EPILEPSY

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K. Yu. Mukhin

2015-01-01

Full Text Available Research on comparative efficаcy and tolerability of monotherapy with Depakine chronosphere, drugs of Carbamazepine group with extended release and oxcarbazepine in symptomatic and cryptogenic focal epilepsy has been conducted at Svt. Luka’s Institute of Child Neurology and Epilepsy (ICNE (Moscow. This retrospective study covers a random sample of patients treated in ICNE in the period from December 1, 2013 to September 1, 2014.  The study included 131 patients aged 1 to 18 years with symptomatic and cryptogenic focal epilepsy receiving treatment with one of the study drugs in monotherapy: group 1 – monotherapy with Depakine chronosphere (n = 56; group 2 – monotherapy with drugs of carbamazepine group with extended release (n = 55; group 3 – monotherapy with oxcarbazepine (trileptal (n = 20. The obtained results allow us to conclude that the effectiveness of Depakin chronosphere, carbamazepine with extended release and oxcarbazepine in monotherapy of symptomatic and cryptogenic focal epilepsy was comparable (statistically significant differences in efficacy were not found. However, carbamazepine was awarded the highest frequency of seizures aggravation. Drugs showed approximately same tolerability (statistically significant differences in tolerability were not found. However, withdrawal of the drug due to side effects was the rarest in Depakine (3.5 %, and withdrawal due to intolerance was higher in carbamazepine and oxcarbazepine (5 and 10 % respectively. Depakinum and oxcarbazepine had the best results in the blocking of pathological activity on the electroencephalogram, whereas carbamazepine was clearly inferior to them. In this regard, complete clinical-electroencephalographic remission (lasting 12 months or more was achieved under treatment of Depakine chromosphere in 21.5 % of cases, oxcarbazepi on therapy for 12 months was similar in all study drugs. Considering that the objective of epilepsy treatment is to achieve complete

Temporal lobe epilepsy and focal cortical dysplasia in children: A tip to find the abnormality.

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Bartolini, Luca; Whitehead, Matthew T; Ho, Cheng-Ying; Sepeta, Leigh N; Oluigbo, Chima O; Havens, Kathryn; Freilich, Emily R; Schreiber, John M; Gaillard, William D

2017-01-01

To demonstrate an association between magnetic resonance imaging (MRI) findings and pathologic characteristics in children who had surgery for medically refractory epilepsy due to focal cortical dysplasia (FCD). We retrospectively studied 110 children who had epilepsy surgery. Twenty-seven patients with FCD were included. Thirteen had temporal lobe epilepsy (TLE) and 14 had extra-temporal lobe epilepsy (ETLE). Three patients had associated mesial temporal sclerosis. Preoperative 3T MRIs interleaved with nine controls were blindly re-reviewed and categorized according to signal alteration. Pathologic specimens were classified according to the 2011 International League Against Epilepsy (ILAE) classification and compared to MRI studies. Rates of pathology subtypes differed between TLE and ETLE (χ 2 (3) = 8.57, p = 0.04). FCD type I was more frequent in TLE, whereas FCD type II was more frequent in ETLE. In the TLE group, nine patients had temporal tip abnormalities. They all exhibited gray-white matter blurring with decreased myelination and white matter hyperintense signal. Blurring involved the whole temporal tip, not just the area of dysplasia. These patients were less likely to demonstrate cortical thickening compared to those without temporal tip findings (χ 2 (1) = 9.55, p = 0.002). Three of them had FCD Ib, three had FCD IIa, two had FCD IIIa, and one had FCD IIb; MRI features could not entirely distinguish between FCD subtypes. TLE patients showed more pronounced findings than ETLE on MRI (χ 2 (1) = 11.95, p = 0.003, odds ratio [OR] 18.00). In all cases of FCD, isolated blurring was more likely to be associated with FCD II, whereas blurring with decreased myelination was seen with FCD I (χ 2 (6) = 13.07, p = 0.042). Our study described associations between MRI characteristics and pathology in children with FCD and offered a detailed analysis of temporal lobe tip abnormalities and FCD subtypes in children with TLE. These findings may contribute to the

Characterization of functional and structural integrity in experimental focal epilepsy: reduced network efficiency coincides with white matter changes.

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Willem M Otte

Full Text Available BACKGROUND: Although focal epilepsies are increasingly recognized to affect multiple and remote neural systems, the underlying spatiotemporal pattern and the relationships between recurrent spontaneous seizures, global functional connectivity, and structural integrity remain largely unknown. METHODOLOGY/PRINCIPAL FINDINGS: Here we utilized serial resting-state functional MRI, graph-theoretical analysis of complex brain networks and diffusion tensor imaging to characterize the evolution of global network topology, functional connectivity and structural changes in the interictal brain in relation to focal epilepsy in a rat model. Epileptic networks exhibited a more regular functional topology than controls, indicated by a significant increase in shortest path length and clustering coefficient. Interhemispheric functional connectivity in epileptic brains decreased, while intrahemispheric functional connectivity increased. Widespread reductions of fractional anisotropy were found in white matter regions not restricted to the vicinity of the epileptic focus, including the corpus callosum. CONCLUSIONS/SIGNIFICANCE: Our longitudinal study on the pathogenesis of network dynamics in epileptic brains reveals that, despite the locality of the epileptogenic area, epileptic brains differ in their global network topology, connectivity and structural integrity from healthy brains.

SPECT in Focal Epilepsies

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Roderick Duncan

2000-01-01

Full Text Available Brain perfusion changes during seizures were first observed in the 1930s. Single Photon Emission Computed Tomography (SPECT was developed in the 1970s, and tracers suitable for the imaging of regional cerebral perfusion (rCP became available in the 1980s. The method was first used to study rCP in the interictal phase, and this showed areas of low perfusion in a proportion of cases, mainly in patients with temporal lobe epilepsies. However, the trapping paradigm of tracers such as hexamethyl propyleneamine oxime (HMPAO provided a practicable method of studying changes in rCP during seizures, and a literature was established in the late 1980s and early 1990s showing a typical sequence of changes during and after seizures of mesial temporal lobe origin; the ictal phase was associated with large increases in perfusion throughout the temporal lobe, with first the lateral, then the mesial temporal lobe becoming hypoperfused in the postictal phase. Activation and inhibition of other structures, such as the basal ganglia and frontal cortex, were also seen. Studies of seizures originating elsewhere in the brain have shown a variety of patterns of change, according to the structures involved. These changes have been used practically to aid the process of localisation of the epileptogenic zone so that epilepsy surgery can be planned.

[Formula: see text]Differences in memory functioning between children with attention-deficit/hyperactivity disorder and/or focal epilepsy.

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Lee, Sylvia E; Kibby, Michelle Y; Cohen, Morris J; Stanford, Lisa; Park, Yong; Strickland, Suzanne

2016-01-01

Prior research has shown that attention-deficit/hyperactivity disorder (ADHD) and epilepsy are frequently comorbid and that both disorders are associated with various attention and memory problems. Nonetheless, limited research has been conducted comparing the two disorders in one sample to determine unique versus shared deficits. Hence, we investigated differences in working memory (WM) and short-term and delayed recall between children with ADHD, focal epilepsy of mixed foci, comorbid ADHD/epilepsy and controls. Participants were compared on the Core subtests and the Picture Locations subtest of the Children's Memory Scale (CMS). Results indicated that children with ADHD displayed intact verbal WM and long-term memory (LTM), as well as intact performance on most aspects of short-term memory (STM). They performed worse than controls on Numbers Forward and Picture Locations, suggesting problems with focused attention and simple span for visual-spatial material. Conversely, children with epilepsy displayed poor focused attention and STM regardless of the modality assessed, which affected encoding into LTM. The only loss over time was found for passages (Stories). WM was intact. Children with comorbid ADHD/epilepsy displayed focused attention and STM/LTM problems consistent with both disorders, having the lowest scores across the four groups. Hence, focused attention and visual-spatial span appear to be affected in both disorders, whereas additional STM/encoding problems are specific to epilepsy. Children with comorbid ADHD/epilepsy have deficits consistent with both disorders, with slight additive effects. This study suggests that attention and memory testing should be a regular part of the evaluation of children with epilepsy and ADHD.

Young woman with a four-year history of epilepsy and progressive focal cortical atrophy — What is the diagnosis?

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S. Pati

2014-01-01

Full Text Available The pathogenesis of disease progression in drug-refractory epilepsy is poorly understood. We report the case of a young woman with a four-year history of epilepsy that progressed rapidly as evidenced by the development of progressive focal cortical atrophy. She underwent biopsy that showed perinatal ischemia and a prominent inflammatory response, including T-cell infiltration and microglial activation. There was no consensus reached on the final diagnosis although the hypothesis of dual pathology (adult variant of Rasmussen's encephalitis and perinatal stroke was considered. The possible role of inflammation in the progression of epilepsy caused by a “static” lesion (perinatal stroke is discussed.

Determining the relationship between sleep architecture, seizure variables and memory in patients with focal epilepsy.

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Miller, Laurie A; Ricci, Monica; van Schalkwijk, Frank J; Mohamed, Armin; van der Werf, Ysbrand D

2016-06-01

Sleep has been shown to be important to memory. Both sleep and memory have been found to be abnormal in patients with epilepsy. In this study, we explored the effects that nocturnal epileptiform discharges and the presence of a hippocampal lesion have on sleep patterns and memory. Twenty-five patients with focal epilepsy who underwent a 24-hr ambulatory EEG also completed the Everyday Memory Questionnaire (EMQ). The EEG record was scored for length of time spent in the various sleep stages, time spent awake after sleep onset, and rapid eye movement (REM) latency. Of these sleep variables, only REM latency differed when the epilepsy patients were divided on the bases of either presence/absence of nocturnal discharges or presence/absence of a hippocampal lesion. In both cases, presence of the abnormality was associated with longer latency. Furthermore, longer REM latency was found to be a better predictor of EMQ score than either number of discharges or presence of a hippocampal lesion. Longer REM latency was associated with a smaller percentage of time spent in slow-wave sleep in the early part of the night and may serve as a particularly sensitive marker to disturbances in sleep architecture. (PsycINFO Database Record (c) 2016 APA, all rights reserved).

Surgical and postmortem pathology studies: contribution for the investigation of temporal lobe epilepsy.

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Caboclo, Luís Otávio Sales Ferreira; Neves, Rafael Scarpa; Jardim, Anaclara Prada; Hamad, Ana Paula Andrade; Centeno, Ricardo Silva; Lancellotti, Carmen Lucia Penteado; Scorza, Carla Alessandra; Cavalheiro, Esper Abrão; Yacubian, Elza Márcia Targas; Sakamoto, Américo Ceiki

2012-12-01

Pathology studies in epilepsy patients bring useful information for comprehending the physiopathology of various forms of epilepsy, as well as aspects related to response to treatment and long-term prognosis. These studies are usually restricted to surgical specimens obtained from patients with refractory focal epilepsies. Therefore, most of them pertain to temporal lobe epilepsy (TLE) with mesial temporal sclerosis (MTS) and malformations of cortical development (MCD), thus providing information of a selected group of patients and restricted regions of the brain. Postmortem whole brain studies are rarely performed in epilepsy patients, however they may provide extensive information on brain pathology, allowing the analysis of areas beyond the putative epileptogenic zone. In this article, we reviewed pathology studies performed in epilepsy patients with emphasis on neuropathological findings in TLE with MTS and MCD. Furthermore, we reviewed data from postmortem studies and discussed the importance of performing these studies in epilepsy populations.

Early classification of childhood focal idiopathic epilepsies: is it possible at the first seizure?

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Gaggero, Roberto; Pistorio, Angela; Pignatelli, Sara; Rossi, Alessandra; Mancardi, Maria Margherita; Baglietto, Maria Giuseppina; Striano, Pasquale; Verrotti, Alberto

2014-05-01

To evaluate the possibility of early syndrome classification of idiopathic partial epilepsies in children at the first seizure. In this observational study we prospectively evaluated 298 patients, aged between 1 month and 17 years and consecutively referred for the first unprovoked focal seizure. The whole cohort included 133 patients; the final analysis was carried out on 107 (59 males) individuals. Age at the first seizure ranged between 2.3 and 13.0 years. Clinical and EEG data of all patients were independently reviewed by two medical doctors. Patients were followed-up for at least 5 years, with a mean period of follow-up of 6.9 years. After the first seizure, a specific syndrome could be diagnosed in eighty (74.7%) children. In particular, Childhood Epilepsy with Centro-Temporal Spikes (CECTS) 42.9% of cases, Panayiotopoulos Syndrome (PS) 28.9%, idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) 2.8%. Unclassified cases were 25.4%. At the end of the follow-up, the diagnosis was confirmed in 72 of 80 children (90%): BCECTS 89% of patients, PS 90% and ICOE-G 100%: among the unclassified cases, in 11 patients (40.7%) the diagnosis did not change, whereas 16 patients (59.3%) evolved into other syndromes or into atypical forms. At the onset an initial diagnosis is possible in the majority of cases; epilepsy syndromes can be identified at the time of the initial diagnosis and at follow up this diagnosis has not to be revised in 90% of the cases. Copyright © 2014 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Surgical and postmortem pathology studies: contribution for the investigation of temporal lobe epilepsy

Directory of Open Access Journals (Sweden)

Luís Otávio Sales Ferreira Caboclo

2012-12-01

Full Text Available Pathology studies in epilepsy patients bring useful information for comprehending the physiopathology of various forms of epilepsy, as well as aspects related to response to treatment and long-term prognosis. These studies are usually restricted to surgical specimens obtained from patients with refractory focal epilepsies. Therefore, most of them pertain to temporal lobe epilepsy (TLE with mesial temporal sclerosis (MTS and malformations of cortical development (MCD, thus providing information of a selected group of patients and restricted regions of the brain. Postmortem whole brain studies are rarely performed in epilepsy patients, however they may provide extensive information on brain pathology, allowing the analysis of areas beyond the putative epileptogenic zone. In this article, we reviewed pathology studies performed in epilepsy patients with emphasis on neuropathological findings in TLE with MTS and MCD. Furthermore, we reviewed data from postmortem studies and discussed the importance of performing these studies in epilepsy populations.

Accelerated long-term forgetting in focal epilepsies with special consideration given to patients with diagnosed and suspected limbic encephalitis.

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Helmstaedter, Christoph; Winter, Babette; Melzer, Nico; Lohmann, Hubertus; Witt, Juri-Alexander

2018-01-31

Accelerated long-term forgetting (ALF) is a phenomenon found in late onset epilepsy and in transient epileptic amnesia (TEA). Here we evaluated ALF in patients with focal epilepsies and limbic encephalitis (LE) in particular. ALF was assessed in 36 patients with focal epilepsy and 154 healthy subjects using an extended version of the Verbal Learning and Memory Test (VLMT), with free recall after 30 min and again after one week. From these patients, 89% had temporal lobe epilepsy; 42% left-lateralized; 39% right; 19% bilateral; 17% were diagnosed with hippocampal sclerosis; 64% displayed features indicating LE; 52% with amygdala pathology, and 61% were antibody positive. ALF was defined as either having unimpaired free recall after 30 min and impaired recall after a week (A) or as a loss in recall exceeding the absolute (B) and percentage loss (C) in the interval of the 30 min and one week recall seen in controls by more than one standard deviation. Repeated measures analysis revealed an association between LE and ALF. Depending on its definition (A, B, or C), ALF was evident in 31%, 42%, or 67% of the patients. Poor verbal memory and ALF (C) were prominent in left-lateralized epilepsies. ALF (A) appeared more frequently in auto-antibody negative patients with LE, ALF (B) less frequently with hippocampal sclerosis. Seizures during the interval did not explain ALF. Depending on its definition, ALF is seen in patients with normal or impaired memory at ½ h. ALF seems related to LE but might as well be the first sign of memory impairment in patients with milder epilepsies and not yet definite structural temporal lobe pathology. Longitudinal assessment would be essential for discerning when ALF becomes evident, whether conditions exist in which ALF precedes short-term forgetting, and whether ALF responds to treatment. Copyright © 2018 Elsevier Ltd. All rights reserved.

Surgical pathology of epilepsy-associated non-neoplastic cerebral lesions: a brief introduction with special reference to hippocampal sclerosis and focal cortical dysplasia

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Miyata, Hajime; Hori, Tomokatsu; Vinters, Harry V.

2014-01-01

Among epilepsy-associated non-neoplastic lesions, mesial temporal lobe epilepsy with hippocampal sclerosis (mTLE-HS) and malformation of cortical development (MCD) including focal cortical dysplasia (FCD), are the two most frequent causes of drug-resistant focal epilepsies constituting about 50% of all surgical pathology of epilepsy. Several distinct histological patterns have been historically recognized in both HS and FCD, and several studies have tried to perform clinicopathological correlation; results, however, have been controversial, particularly in terms of postsurgical seizure outcome. Recently, the International League Against Epilepsy constituted a Task Forces of Neuropathology and FCD within the Commission on Diagnostic Methods, to establish an international consensus of histological classification of HS and FCD, respectively, based on agreement with the recognition of the importance of defining a histopathological classification system that reliably has some clinicopathological correlation. Such consensus classifications are likely to facilitate future clinicopathological study. Meanwhile, we reviewed neuropathology of 41 surgical cases of mTLE, and confirmed three type/patterns of HS along with no HS, based on the qualitative evaluation of the distribution and severity of neuronal loss and gliosis within hippocampal formation; i.e., HS type 1 (61%) equivalent to ‘classical’ Ammon’s horn sclerosis, HS type 2 (2%) representing CA1 sclerosis, HS type 3 (17%) equivalent to end folium sclerosis, and no HS (19%). Furthermore we performed a neuropathological comparative study on mTLE-HS and dementia associated HS (d-HS) in elderly, and confirmed that neuropathological features differ between mTLE-HS and d-HS in the distribution of hippocampal neuronal loss and gliosis, morphology of reactive astrocytes and their protein expression, and presence of concomitant neurodegenerative changes particularly Alzheimer type and TDP-43 pathologies. These

Classification of Focal and Non Focal Epileptic Seizures Using Multi-Features and SVM Classifier.

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Sriraam, N; Raghu, S

2017-09-02

Identifying epileptogenic zones prior to surgery is an essential and crucial step in treating patients having pharmacoresistant focal epilepsy. Electroencephalogram (EEG) is a significant measurement benchmark to assess patients suffering from epilepsy. This paper investigates the application of multi-features derived from different domains to recognize the focal and non focal epileptic seizures obtained from pharmacoresistant focal epilepsy patients from Bern Barcelona database. From the dataset, five different classification tasks were formed. Total 26 features were extracted from focal and non focal EEG. Significant features were selected using Wilcoxon rank sum test by setting p-value (p z > 1.96) at 95% significance interval. Hypothesis was made that the effect of removing outliers improves the classification accuracy. Turkey's range test was adopted for pruning outliers from feature set. Finally, 21 features were classified using optimized support vector machine (SVM) classifier with 10-fold cross validation. Bayesian optimization technique was adopted to minimize the cross-validation loss. From the simulation results, it was inferred that the highest sensitivity, specificity, and classification accuracy of 94.56%, 89.74%, and 92.15% achieved respectively and found to be better than the state-of-the-art approaches. Further, it was observed that the classification accuracy improved from 80.2% with outliers to 92.15% without outliers. The classifier performance metrics ensures the suitability of the proposed multi-features with optimized SVM classifier. It can be concluded that the proposed approach can be applied for recognition of focal EEG signals to localize epileptogenic zones.

Imaging of the epilepsies

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Urbach, H. [University of Bonn Medical Center, Department of Radiology/Neuroradiology, Bonn (Germany)

2005-03-01

Imaging of epilepsy patients is challenging, since epileptogenic lesions (defined as structural lesions causally related to the epilepsy syndrome) may be small and often do not change during life. Prior clinical information about the epilepsy syndrome and the semiology of the seizures is needed in order to plan the examination properly. The effort to detect an epileptogenic lesion is directed to partial (focal) epilepsy syndromes whereas - by definition - no lesion is identified in idiopathic epilepsies. Most patients with partial epilepsies suffer from mesial temporal lobe epilepsies. In these patients, 2- to 3-mm-thick T2-weighted and fluid-attenuated inversion-recovery (FLAIR) fast spin echo slices along or perpendicular to the temporal lobe length axis have the highest diagnostic efficacy. In contrast, in patients with extratemporal lobe epilepsies perpendicular FLAIR slices through the anatomic region, from which, due to clinical and EEG criteria, the seizures are likely to originate, are preferred. The imaging features of common epileptogenic lesions (hippocampal sclerosis, long-term epilepsy-associated tumours, focal cortical dysplasias, vascular malformations, encephalitis including limbic and Rasmussen's encephalitis, gyral scarring including ulegyria) are detailed in the second section of this paper. (orig.)

Imaging of the epilepsies

International Nuclear Information System (INIS)

Urbach, H.

2005-01-01

Imaging of epilepsy patients is challenging, since epileptogenic lesions (defined as structural lesions causally related to the epilepsy syndrome) may be small and often do not change during life. Prior clinical information about the epilepsy syndrome and the semiology of the seizures is needed in order to plan the examination properly. The effort to detect an epileptogenic lesion is directed to partial (focal) epilepsy syndromes whereas - by definition - no lesion is identified in idiopathic epilepsies. Most patients with partial epilepsies suffer from mesial temporal lobe epilepsies. In these patients, 2- to 3-mm-thick T2-weighted and fluid-attenuated inversion-recovery (FLAIR) fast spin echo slices along or perpendicular to the temporal lobe length axis have the highest diagnostic efficacy. In contrast, in patients with extratemporal lobe epilepsies perpendicular FLAIR slices through the anatomic region, from which, due to clinical and EEG criteria, the seizures are likely to originate, are preferred. The imaging features of common epileptogenic lesions (hippocampal sclerosis, long-term epilepsy-associated tumours, focal cortical dysplasias, vascular malformations, encephalitis including limbic and Rasmussen's encephalitis, gyral scarring including ulegyria) are detailed in the second section of this paper. (orig.)

Structural magnetic resonance imaging in epilepsy

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Deblaere, Karel [Ghent University Hospital, Department of Neuroradiology, Ghent (Belgium); Ghent University Hospital, MR Department - 1K12, Ghent (Belgium); Achten, Eric [Ghent University Hospital, Department of Neuroradiology, Ghent (Belgium)

2008-01-15

Because of its sensitivity and high tissue contrast, magnetic resonance imaging (MRI) is the technique of choice for structural imaging in epilepsy. In this review the effect of using optimised scanning protocols and the use of high field MR systems on detection sensitivity is discussed. Also, the clinical relevance of adequate imaging in patients with focal epilepsy is highlighted. The most frequently encountered MRI findings in epilepsy are reported and their imaging characteristics depicted. Imaging focus will be on the diagnosis of hippocampal sclerosis and malformations of cortical development, two major causes of medically intractable focal epilepsy. (orig.)

Structural magnetic resonance imaging in epilepsy

International Nuclear Information System (INIS)

Deblaere, Karel; Achten, Eric

2008-01-01

Because of its sensitivity and high tissue contrast, magnetic resonance imaging (MRI) is the technique of choice for structural imaging in epilepsy. In this review the effect of using optimised scanning protocols and the use of high field MR systems on detection sensitivity is discussed. Also, the clinical relevance of adequate imaging in patients with focal epilepsy is highlighted. The most frequently encountered MRI findings in epilepsy are reported and their imaging characteristics depicted. Imaging focus will be on the diagnosis of hippocampal sclerosis and malformations of cortical development, two major causes of medically intractable focal epilepsy. (orig.)

DEPDC5 as a potential therapeutic target for epilepsy.

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Myers, Kenneth A; Scheffer, Ingrid E

2017-06-01

Dishevelled, Egl-10 and Pleckstrin (DEP) domain-containing protein 5 (DEPDC5) is a protein subunit of the GTPase-activating proteins towards Rags 1 (GATOR1) complex. GATOR1 is a recently identified modulator of mechanistic target of rapamycin (mTOR) activity. mTOR is a key regulator of cell proliferation and metabolism; disruption of the mTOR pathway is implicated in focal epilepsy, both acquired and genetic. Tuberous sclerosis is the prototypic mTOR genetic syndrome with epilepsy, however GATOR1 gene mutations have recently been shown to cause lesional and non-lesional focal epilepsy. Areas covered: This review summarizes the mTOR pathway, including regulators and downstream effectors, emphasizing recent developments in the understanding of the complex role of the GATOR1 complex. We review the epilepsy types associated with mTOR overactivity, including tuberous sclerosis, polyhydramnios megalencephaly symptomatic epilepsy, cortical dysplasia, non-lesional focal epilepsy and post-traumatic epilepsy. Currently available mTOR inhibitors are discussed, primarily rapamycin analogs and ATP competitive mTOR inhibitors. Expert opinion: DEPDC5 is an attractive therapeutic target in focal epilepsy, as effects of DEPDC5 agonists would likely be anti-epileptogenic and more selective than currently available mTOR inhibitors. Therapeutic effects might be synergistic with certain existing dietary therapies, including the ketogenic diet.

Genetics Home Reference: familial focal epilepsy with variable foci

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... with FFEVF have developed psychiatric disorders (such as schizophrenia ), behavioral problems, or intellectual disability. It is unclear ... 5 links) American Epilepsy Society Brain Foundation (Australia) CURE: Citizens United for Research in Epilepsy Epilepsy Foundation ...

Improvement of visual field defects after focal resection for occipital lobe epilepsy: case report.

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Yamamoto, Takahiro; Hamasaki, Tadashi; Nakamura, Hideo; Yamada, Kazumichi

2018-03-01

Improvement of visual field defects after surgical treatment for occipital lobe epilepsy is rare. Here, the authors report on a 24-year-old man with a 15-year history of refractory epilepsy that developed after he had undergone an occipital craniotomy to remove a cerebellar astrocytoma at the age of 4. His seizures started with an elementary visual aura, followed by secondary generalized tonic-clonic convulsion. Perimetry revealed left-sided incomplete hemianopia, and MRI showed an old contusion in the right occipital lobe. After evaluation with ictal video-electroencephalography, electrocorticography, and mapping of the visual cortex with subdural electrodes, the patient underwent resection of the scarred tissue, including the epileptic focus at the occipital lobe. After surgery, he became seizure free and his visual field defect improved gradually. In addition, postoperative 123 I-iomazenil (IMZ) SPECT showed partly normalized IMZ uptake in the visual cortex. This case is a practical example suggesting that neurological deficits attributable to the functional deficit zone can be remedied by successful focal resection.

Focal epilepsies in adult patients attending two epilepsy centers

DEFF Research Database (Denmark)

Gilioli, Isabella; Vignoli, Aglaia; Visani, Elisa

2012-01-01

, and we evaluated the risk factors associated with AED resistance using logistic regression analysis. We further grouped AED-resistant patients in different grades (I, II, and III) according to the number of AEDs already tried as proposed by Perucca. KEY FINDINGS: AED resistance occurred in 57...... consecutively after 1990 and followed regularly at two epilepsy centers. We systematically collected the clinical, diagnostic, and therapeutic data using a custom-written database. We classified the patients as seizure-free or AED resistant according to the International League Against Epilepsy (ILAE) criteria...... control (14.9% needed three or more AEDs). Furthermore, among seizure-free patients who could be previously classified as resistant to two or more AEDs, 52.2% reached seizure freedom while receiving treatment with "new generation" AEDs. SIGNIFICANCE: The ILAE classification of AED resistance, as well...

Positron emission tomography in epilepsy

International Nuclear Information System (INIS)

Hosokawa, Shinichi; Kato, Motohiro; Otsuka, Makoto; Kuwabara, Yasuo; Ichiya, Yuichi; Goto, Ikuo

1989-01-01

Positron emission tomography (PET) was performed with the 18 F-fluoro-deoxy-glucose method on 29 patients with epilepsy (generalized epilepsy, 4; partial epilepsy, 24; undetermined type, 1). The subjects were restricted to patients with epilepsy without focal abnormality on X-CT. All the patients with generalized epilepsy showed a normal pattern on PET. Fourteen out of the 24 patients with partial epilepsy and the 1 with epilepsy of undermined type showed focal hypometabolism on PET. The hypometabolic zone was localized in areas including the temporal cortex in 11 patients, frontal in 2 and thalamus in 1. The location of hypometabolic zone and that of interictal paroxysmal activity on EEG were well correlated in most patients. The patients with poorly-controlled seizure showed a higher incidence of PET abnormality (12 out of 13) than those with well-controlled seizures (2 out of 11). The incidence of abnormality on PET and MRI and the location of both abnormality were not necessarily coincident. These results indicated that the PET examination in epilepsy provides valuable information about the location of epileptic focus, and that the findings on PET in patients with partial epilepsy may be one of the good indicators about the intractability of partial epilepsy, and that PET and MRI provide complementary information in the diagnosis of epilepsy. (author)

HIPPOCAMPAL SCLEROSIS IN EPILEPSY AND CHILDHOOD FEBRILE SEIZURES

NARCIS (Netherlands)

KUKS, JBM; COOK, MJ; FISH, DR; STEVENS, JM; SHORVON, SD

1993-01-01

The connection between hippocampal sclerosis and childhood febrile seizures (CFS) is a contentious issue in the study of epilepsy. We investigated 107 patients with drug-resistant epilepsy by high-resolution volumetric magnetic resonance imaging (MRI). 20 had a history of CFS, 45 had focal (26) or

Clinical presentation of neurocysticercosis-related epilepsy.

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Duque, Kevin R; Burneo, Jorge G

2017-11-01

Neurocysticercosis (NCC) is the most common parasitic infection of the central nervous system and a major risk factor for seizures and epilepsy. Seizure types in NCC vary largely across studies and seizure semiology is poorly understood. We discuss here the studies regarding seizure types and seizure semiology in NCC, and examine the clinical presentation in patients with NCC and drug-resistant epilepsy. We also provide evidence of the role of MRI and EEG in the diagnosis of NCC-related epilepsy. Focal seizures are reported in 60-90% of patients with NCC-related epilepsy, and around 90% of all seizures registered prospectively are focal not evolving to bilateral tonic-clonic seizures. A great number of cases suggest that seizure semiology is topographically related to NCC lesions. Patients with hippocampal sclerosis and NCC have different clinical and neurophysiological characteristics than those with hippocampal sclerosis alone. Different MRI protocols have allowed to better differentiate NCC from other etiologies. Lesions' stages might account on the chances of finding an interictal epileptiform discharge. Studies pursuing the seizure onset in patients with NCC are lacking and they are specially needed to determine both whether the reported events of individual cases are seizures, and whether they are related to the NCC lesion or lesions. This article is part of a Special Issue entitled "Neurocysticercosis and Epilepsy". Copyright © 2017 Elsevier Inc. All rights reserved.

Zoomed MRI Guided by Combined EEG/MEG Source Analysis: A Multimodal Approach for Optimizing Presurgical Epilepsy Work-up and its Application in a Multi-focal Epilepsy Patient Case Study.

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Aydin, Ü; Rampp, S; Wollbrink, A; Kugel, H; Cho, J -H; Knösche, T R; Grova, C; Wellmer, J; Wolters, C H

2017-07-01

In recent years, the use of source analysis based on electroencephalography (EEG) and magnetoencephalography (MEG) has gained considerable attention in presurgical epilepsy diagnosis. However, in many cases the source analysis alone is not used to tailor surgery unless the findings are confirmed by lesions, such as, e.g., cortical malformations in MRI. For many patients, the histology of tissue resected from MRI negative epilepsy shows small lesions, which indicates the need for more sensitive MR sequences. In this paper, we describe a technique to maximize the synergy between combined EEG/MEG (EMEG) source analysis and high resolution MRI. The procedure has three main steps: (1) construction of a detailed and calibrated finite element head model that considers the variation of individual skull conductivities and white matter anisotropy, (2) EMEG source analysis performed on averaged interictal epileptic discharges (IED), (3) high resolution (0.5 mm) zoomed MR imaging, limited to small areas centered at the EMEG source locations. The proposed new diagnosis procedure was then applied in a particularly challenging case of an epilepsy patient: EMEG analysis at the peak of the IED coincided with a right frontal focal cortical dysplasia (FCD), which had been detected at standard 1 mm resolution MRI. Of higher interest, zoomed MR imaging (applying parallel transmission, 'ZOOMit') guided by EMEG at the spike onset revealed a second, fairly subtle, FCD in the left fronto-central region. The evaluation revealed that this second FCD, which had not been detectable with standard 1 mm resolution, was the trigger of the seizures.

Ear-EEG detects ictal and interictal abnormalities in focal and generalized epilepsy - A comparison with scalp EEG monitoring.

Science.gov (United States)

Zibrandtsen, I C; Kidmose, P; Christensen, C B; Kjaer, T W

2017-12-01

Ear-EEG is recording of electroencephalography from a small device in the ear. This is the first study to compare ictal and interictal abnormalities recorded with ear-EEG and simultaneous scalp-EEG in an epilepsy monitoring unit. We recorded and compared simultaneous ear-EEG and scalp-EEG from 15 patients with suspected temporal lobe epilepsy. EEGs were compared visually by independent neurophysiologists. Correlation and time-frequency analysis was used to quantify the similarity between ear and scalp electrodes. Spike-averages were used to assess similarity of interictal spikes. There were no differences in sensitivity or specificity for seizure detection. Mean correlation coefficient between ear-EEG and nearest scalp electrode was above 0.6 with a statistically significant decreasing trend with increasing distance away from the ear. Ictal morphology and frequency dynamics can be observed from visual inspection and time-frequency analysis. Spike averages derived from ear-EEG electrodes yield a recognizable spike appearance. Our results suggest that ear-EEG can reliably detect electroencephalographic patterns associated with focal temporal lobe seizures. Interictal spike morphology from sufficiently large temporal spike sources can be sampled using ear-EEG. Ear-EEG is likely to become an important tool in clinical epilepsy monitoring and diagnosis. Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

Optimising EEG-fMRI for Localisation of Focal Epilepsy in Children.

Directory of Open Access Journals (Sweden)

Maria Centeno

Full Text Available Early surgical intervention in children with drug resistant epilepsy has benefits but requires using tolerable and minimally invasive tests. EEG-fMRI studies have demonstrated good sensitivity for the localization of epileptic focus but a poor yield although the reasons for this have not been systematically addressed. While adults EEG-fMRI studies are performed in the "resting state"; children are commonly sedated however, this has associated risks and potential confounds. In this study, we assessed the impact of the following factors on the tolerability and results of EEG-fMRI in children: viewing a movie inside the scanner; movement; occurrence of interictal epileptiform discharges (IED; scan duration and design efficiency. This work's motivation is to optimize EEG-fMRI parameters to make this test widely available to paediatric population.Forty-six children with focal epilepsy and 20 controls (6-18 underwent EEG-fMRI. For two 10 minutes sessions subjects were told to lie still with eyes closed, as it is classically performed in adult studies ("rest sessions", for another two sessions, subjects watched a child friendly stimulation i.e. movie ("movie sessions". IED were mapped with EEG-fMRI for each session and across sessions. The resulting maps were classified as concordant/discordant with the presumed epileptogenic focus for each subject.Movement increased with scan duration, but the movie reduced movement by ~40% when played within the first 20 minutes. There was no effect of movie on the occurrence of IED, nor in the concordance of the test. Ability of EEG-fMRI to map the epileptogenic region was similar for the 20 and 40 minute scan durations. Design efficiency was predictive of concordance.A child friendly natural stimulus improves the tolerability of EEG-fMRI and reduces in-scanner movement without having an effect on IED occurrence and quality of EEG-fMRI maps. This allowed us to scan children as young as 6 and obtain localising

Epilepsy diagnostic and treatment needs identified with a collaborative database involving tertiary centers in France.

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Chipaux, Mathilde; Szurhaj, William; Vercueil, Laurent; Milh, Mathieu; Villeneuve, Nathalie; Cances, Claude; Auvin, Stéphane; Chassagnon, Serge; Napuri, Sylvia; Allaire, Catherine; Derambure, Philippe; Marchal, Cécile; Caubel, Isabelle; Ricard-Mousnier, Brigitte; N'Guyen The Tich, Sylvie; Pinard, Jean-Marc; Bahi-Buisson, Nadia; de Baracé, Claire; Kahane, Philippe; Gautier, Agnès; Hamelin, Sophie; Coste-Zeitoun, Delphine; Rosenberg, Sarah-Dominique; Clerson, Pierre; Nabbout, Rima; Kuchenbuch, Mathieu; Picot, Marie-Christine; Kaminska, Anna

2016-05-01

To obtain perspective on epilepsy in patients referred to tertiary centers in France, and describe etiology, epilepsy syndromes, and identify factors of drug resistance and comorbidities. We performed a cross-sectional analysis of the characteristics of 5,794 pediatric and adult patients with epilepsy included in a collaborative database in France between 2007 and 2013. Comparisons between groups used Student's t-test or Fisher's exact test for binary or categorical variables. Factors associated with drug resistance and intellectual disability were evaluated in multi-adjusted logistic regression models. Mean age at inclusion was 17.9 years; children accounted for 67%. Epilepsy was unclassified in 20% of patients, and etiology was unknown in 65%, including those with idiopathic epilepsies. Etiologies differed significantly in adult- when compared to pediatric-onset epilepsy; however, among focal structural epilepsies, mesial temporal lobe epilepsy with hippocampal sclerosis began as often in the pediatric as in adult age range. Drug resistance concerned 53% of 4,210 patients evaluable for seizure control and was highest in progressive myoclonic epilepsy (89%), metabolic diseases (84%), focal cortical dysplasia (70%), other cortical malformations (69%), and mesial temporal lobe epilepsy with hippocampal sclerosis (67%). Fifty-nine percent of patients with focal structural epilepsy and 69% with epileptic encephalopathies were drug resistant; however, 40-50% of patients with West syndrome and epileptic encephalopathy with continuous spike-and-waves during sleep were seizure-free. Ages at onset in infancy and in young adults shared the highest risk of drug resistance. Epilepsy onset in infancy comprised the highest risk of intellectual disability, whereas specific cognitive impairment affected 36% of children with idiopathic focal epilepsy. Our study provides a snapshot on epilepsy in patients referred to tertiary centers and discloses needs for diagnosis and treatment

MR contribution in surgery of epilepsy

International Nuclear Information System (INIS)

Meiners, L.C.; Valk, J.; Jansen, G.H.; Veelen, C.W.M. van

1999-01-01

The contribution of MR imaging in patients with drug-resistant epilepsy considered for surgical therapy is discussed. In this review we focus on: (a) focal abnormalities (mesial temporal sclerosis, focal migration disorders, hamartomatous lesions and low-grade tumours, phakomatosis and vascular malformations) associated with therapy-resistant partial epilepsy, requiring resective surgery; (b) abnormalities leading to generalized seizures that require more drastic surgical procedures, such as callosotomy and functional hemispherectomy; and (c) localisation of implanted depth-electrodes. (orig.)

Parental Infertility, Fertility Treatment, and Childhood Epilepsy

DEFF Research Database (Denmark)

Kettner, Laura O; Ramlau-Hansen, Cecilia Høst; Kesmodel, Ulrik S

2016-01-01

. RESULTS: A total of 60 440 pregnancies were included, and 0.8% of the children developed epilepsy.The primary analyses showed no association between parental infertility or fertility treatment, and the overall risk of childhood epilepsy (hazard rate ratios (HRs); 95% confidence intervals (CIs): 1.08 (0......BACKGROUND: A few studies have indicated an increased risk of epilepsy in children conceived by fertility treatment possibly due to characteristics of the infertile couple rather than the treatment. We therefore aimed to investigate the association between parental infertility, fertility treatment......, and epilepsy in the offspring, including the subtypes of epilepsy; idiopathic generalised epilepsy and focal epilepsy. METHODS: This cohort included all pregnancies resulting in liveborn singletons from the Aarhus Birth Cohort, Denmark (1995-2013). Information on time to pregnancy and fertility treatment...

Genetic Forms of Epilepsies and other Paroxysmal Disorders

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Olson, Heather E.; Poduri, Annapurna; Pearl, Phillip L.

2016-01-01

Genetic mechanisms explain the pathophysiology of many forms of epilepsy and other paroxysmal disorders such as alternating hemiplegia of childhood, familial hemiplegic migraine, and paroxysmal dyskinesias. Epilepsy is a key feature of well-defined genetic syndromes including Tuberous Sclerosis Complex, Rett syndrome, Angelman syndrome, and others. There is an increasing number of singe gene causes or susceptibility factors associated with several epilepsy syndromes, including the early onset epileptic encephalopathies, benign neonatal/infantile seizures, progressive myoclonus epilepsies, genetic generalized and benign focal epilepsies, epileptic aphasias, and familial focal epilepsies. Molecular mechanisms are diverse, and a single gene can be associated with a broad range of phenotypes. Additional features, such as dysmorphisms, head size, movement disorders, and family history may provide clues to a genetic diagnosis. Genetic testing can impact medical care and counseling. We discuss genetic mechanisms of epilepsy and other paroxysmal disorders, tools and indications for genetic testing, known genotype-phenotype associations, the importance of genetic counseling, and a look towards the future of epilepsy genetics. PMID:25192505

A computed tomographic study on epilepsy

International Nuclear Information System (INIS)

Bae, Hoon Sik

1980-01-01

140 patients with epileptic seizure were studied by computed tomography during the period from Feb. 1979 to Aug. 1979 in the Department or Radiology, College of Medicine, Hangyang University. Those findings on CT and clinical records including EEG findings were reviewed. The results were as follows: 1. Age distribution of the total 140 patients was broad ranging from 1 month to 63 years. 73.5% of patients was below the age of 30. The patient population was comprised of 93 males and 47 females, and its male to female ratio was 2 : 1. 2. The type of epileptic seizure were classified according to the International League against Epilepsy. 42.9% of patients had primary generalized seizure, 47.1% with partial seizure, and 10% with non classifiable seizure. 3. As additional symptoms and signs except seizure, headache was most common, and the next was nausea and vomiting. Uncommonly, there were also insomnia, personality change, and memory disturbance. 4. 37.1% of patients had less than 1 month of seizure history, 19.3% between 1 year and 5 years. 5. EEG findings were available in 41 patients, and normal in 15 cases. 26 patients revealed abnormal findings. Among those abnormal findings focal slowing was appeared in 19.5% and generalized slowing in 17.1%. 6. 52% of patients showed abnormal findings on CT. The most common abnormal findings was focal low density (30%), and the next was diffuse hydrocephalus (7.1%). After contrast infusion, contrast enhancement was occurred in cases with focal low density, focal high or isodense mass density. In patients with focal low density, ring or nodular enhancement were common, and diffuse or serpentime enhancement in focal high or isodence mass density. 7. The frequency of structural abnormalities on CT was more common in patients below the age of 10 and over 30 than other age groups. The epilepsy starting below 10 and over 30 years of age showed structural abnormalities in 63.6-100%. 8. The patients who had less than 6 months of

A computed tomographic study on epilepsy

Energy Technology Data Exchange (ETDEWEB)

Bae, Hoon Sik [Hanyang University College of Medicine, Seoul (Korea, Republic of)

1980-06-15

140 patients with epileptic seizure were studied by computed tomography during the period from Feb. 1979 to Aug. 1979 in the Department or Radiology, College of Medicine, Hangyang University. Those findings on CT and clinical records including EEG findings were reviewed. The results were as follows: 1. Age distribution of the total 140 patients was broad ranging from 1 month to 63 years. 73.5% of patients was below the age of 30. The patient population was comprised of 93 males and 47 females, and its male to female ratio was 2 : 1. 2. The type of epileptic seizure were classified according to the International League against Epilepsy. 42.9% of patients had primary generalized seizure, 47.1% with partial seizure, and 10% with non classifiable seizure. 3. As additional symptoms and signs except seizure, headache was most common, and the next was nausea and vomiting. Uncommonly, there were also insomnia, personality change, and memory disturbance. 4. 37.1% of patients had less than 1 month of seizure history, 19.3% between 1 year and 5 years. 5. EEG findings were available in 41 patients, and normal in 15 cases. 26 patients revealed abnormal findings. Among those abnormal findings focal slowing was appeared in 19.5% and generalized slowing in 17.1%. 6. 52% of patients showed abnormal findings on CT. The most common abnormal findings was focal low density (30%), and the next was diffuse hydrocephalus (7.1%). After contrast infusion, contrast enhancement was occurred in cases with focal low density, focal high or isodense mass density. In patients with focal low density, ring or nodular enhancement were common, and diffuse or serpentime enhancement in focal high or isodence mass density. 7. The frequency of structural abnormalities on CT was more common in patients below the age of 10 and over 30 than other age groups. The epilepsy starting below 10 and over 30 years of age showed structural abnormalities in 63.6-100%. 8. The patients who had less than 6 months of

Reduced Language Connectivity in Pediatric Epilepsy

Science.gov (United States)

Leigh N., Sepeta; Louise J., Croft; Lauren A., Zimmaro; Elizabeth S., Duke; Virginia K., Terwilliger; Benjamin E., Yerys; Xiaozhen., You; Chandan J., Vaidya; William D., Gaillard; Madison M., Berl

2014-01-01

Objective Functional connectivity (FC) among language regions is decreased in adults with epilepsy compared to controls, but less is known about FC in children with epilepsy. We sought to determine if language FC is reduced in pediatric epilepsy, and examined clinical factors that associate with language FC in this population. Methods We assessed FC during an age-adjusted language task in children with left-hemisphere focal epilepsy (n=19) compared to controls (n=19). Time series data were extracted for three left ROIs and their right homologues: inferior frontal gyrus (IFG), middle frontal gyrus (MFG), and Wernicke's area (WA) using SPM8. Associations between FC and factors such as cognitive performance, language dominance, and epilepsy duration were assessed. Results Children with epilepsy showed decreased interhemispheric connectivity compared to controls, particularly between core left language regions (IFG, WA) and their right hemisphere homologues, as well as decreased intrahemispheric right frontal FC. Increased intrahemispheric FC between left IFG and left WA was a positive predictor of language skills overall, and naming ability in particular. FC of language areas was not affected by language dominance, as the effects remained when only examining study participants with left language dominance. Overall FC did not differ according to duration of epilepsy or age of onset. Significance FC during a language task is reduced in children, similar to findings in adults. In specific, children with left focal epilepsy demonstrated decreased interhemispheric FC in temporal and frontal language connections and decreased intrahemispheric right frontal FC. These differences were present near the onset of epilepsy. Greater FC between left language centers is related to better language ability. Our results highlight that connectivity of language areas has a developmental pattern and is related to cognitive ability. PMID:25516399

Genetics Home Reference: epilepsy-aphasia spectrum

Science.gov (United States)

... Szepetowski P, Scheffer IE, Mefford HC. GRIN2A mutations cause epilepsy-aphasia spectrum disorders. Nat Genet. 2013 Sep;45( ... Neubauer BA, Biskup S, von Spiczak S. Mutations in GRIN2A cause idiopathic focal epilepsy with rolandic spikes. Nat Genet. 2013 Sep;45( ...

99mTc-HM-PAO SPECT of epileptic patients showing focal paroxysm on electroencephalography

International Nuclear Information System (INIS)

Takaishi, Yasuko; Hashimoto, Kiyoshi; Fujino, Osamu; Kamayachi, Satoshi; Fujita, Takehisa; Enokido, Hisashi; Komatsuzaki, Hideki; Kawakami, Yasuhiko; Hirayama, Tsunenori

1995-01-01

The usefulness of 99m Tc-HM-PAO SPECT in diagnosing epilepsy was studied. The subjects were 33 epileptic patients, ranging in age from 5 years and 5 months to 28 years and 3 months, who showed focal paroxysm on electroencephalograms. Lowered accumulation site was found on SPECT in 19 patients. Four patients with abnormal findings on X-ray CT or MRI showed lowered accumulation and focal paroxysm at the same site. Of 29 patients with normal X-ray CT or MRI findings, 15 (52%) showed lowered accumulation. Five patients showed a focal paroxysm at the site of lowered accumulation. In 8 patients the focal paroxysm site was partly coincided with the accumulation site. In some patients the focal site predicted by the findings of clinical symptoms and the lowered accumulation site coincided. SPECT is therefore a useful method in diagnosing a focal site in epilepsy and considered to reflect the severity of disease. (Y.S.)

Left frontal meningioangiomatosis associated with type IIIc focal cortical dysplasia causing refractory epilepsy and literature review.

Science.gov (United States)

Roux, Alexandre; Mellerio, Charles; Lechapt-Zalcman, Emmanuelle; Still, Megan; Zerah, Michel; Bourgeois, Marie; Pallud, Johan

2018-03-29

We report the surgical management of a lesional drug-resistant epilepsy caused by a meningioangiomatosis associated with a type IIIc focal cortical dysplasia located in the left supplementary motor area in a young male patient. A first anatomical-based partial surgical resection was performed at 11 years old under general anaesthesia without intraoperative mapping, which allowed for postoperative seizure control (Engel IA) for six years. The patient then presented with intractable right sensatory and aphasic focal onset seizures despite two appropriate antiepileptic drugs. A second functional-based surgical resection was performed using intraoperative cortico-subcortical functional mapping with direct electrical stimulation under awake conditions. A complete surgical resection was performed and a left partial supplementary motor area syndrome was observed. At six postoperative months, the patient is seizure free (Engel IA) with an ongoing decrease in antiepileptic drug therapy. Intraoperative functional brain mapping can be applied to preserve the brain function and networks around a meningioangiomatosis to facilitate the resection of potentially epileptogenic perilesional dysplastic cortex and to tailor the extent of resection to functional boundaries. Copyright © 2018. Published by Elsevier Inc.

Clinicopathological study on refractory epilepsy treated by several epilepsy surgeries

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Yan LI

2018-04-01

Full Text Available Objective To observe and investigate the clinicopathological features and types of refractory epilepsy treated by several epilepsy surgeries. Methods There were 19 patients with age less than 20 years who underwent 2 (16/19 or 3 (3/19 epilepsy surgeries. After pathological examination, pathological diagnosis and subtype was made according to focal cortical dysplasia (FCD classification proposed by International League Against Epilepsy (ILAE Diagnostic Methods Commission in 2011 and World Health Organization (WHO Classification of Tumors of Central Nervous System in 2007. Results The operation intervals were 1-10 years (average 4.24 years. The pathological diagnoses after first operation were FCDⅠb in 2 cases (2/19, FCDⅡa in 2 cases (2/19, FCDⅢa in one case (1/19, FCDⅢd in one case (1/19, 5 cases of tumor lesions [2 (2/19 of astrocytoma, one (1/19 of oligoastrocytoma, one (1/ 19 of mixed germ cell tumor, one (1/19 of hysembryoplastic neuroepithelial tumor (DNT], one case (1/19 of hamartoma, one case (1/19 of angioma, one case (1/19 of heterotopic gray matter, and 4 cases (4/19 of ulegyria. The last one (1/19 underwent corpus callosal incision. Pathological diagnoses after reoperation were FCDⅢa in 4 cases (4/19, FCDⅢb in 4 cases (4/19, FCDⅢc in one case (1/19, FCDⅢd in 8 cases (8/19, dual pathology (FCDⅢa with oligoastrocytoma and with glial scar and/or ulegyria in 2 cases (2/19. Patients were followed up for 0.50-5.00 years after second or third operation (average 2.34 years, and the results showed Engel Ⅰ in 10 patients (10/19, Engel Ⅱ in 6 patients (6/19 and Engel Ⅲ in 3 patients (3/19. The rate of good prognosis was 84.21%. Conclusions The pathological diagnoses of brain tissue resected from patients accepting several epilepsy surgeries are mainly FCD Ⅲ and dual pathology. It is suggested that the second or third operation would be effective for refractory epilepsy patients who underwent surgery already. DOI: 10

The Ketogenic Diet Improves Recently Worsened Focal Epilepsy

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Villeneuve, Nathalie; Pinton, Florence; Bahi-Buisson, Nadia; Dulac, Olivier; Chiron, Catherine; Nabbout, Rima

2009-01-01

Aim: We observed a dramatic response to the ketogenic diet in several patients with highly refractory epilepsy whose seizure frequency had recently worsened. This study aimed to identify whether this characteristic was a useful indication for the ketogenic diet. Method: From the 70 patients who received the ketogenic diet during a 3-year period at…

Ehlers-Danlos syndrome: a cause of epilepsy and periventricular heterotopia.

Science.gov (United States)

Verrotti, Alberto; Monacelli, Debora; Castagnino, Miriam; Villa, Maria Pia; Parisi, Pasquale

2014-11-01

Ehlers-Danlos syndrome (EDS) comprises a variety of inherited connective tissue disorders that have been described in association with various neurological features. Until now the neurological symptoms have not been studied in detail; therefore, the aim of this review is to analyze the possible association between EDS, epilepsy and periventricular heterotopia (PH). We have carried out a critical review of all cases of epilepsy in EDS patients with and without PH. Epilepsy is a frequent neurological manifestation of EDS; generally, it is characterized by focal seizures with temporo-parieto-occipital auras and the most common EEG findings epileptiform discharges and slow intermittent rhythm with delta-theta waves. Epilepsy in EDS patients is usually responsive to common antiepileptic therapy; very few cases of drug resistant focal epilepsy requested surgical treatment, with favorable results in terms of outcome. Epilepsy is the most common presenting neurological manifestation associated with PH in EDS patients. Abnormal anatomic circuitries (including heterotopic nodules) could generate epilepsy in patients with PH. Among the principal neurological manifestations, epilepsy and PH have a considerable importance and can influence the long-term evolution of these patients. We hypothesize that PH may determine the epileptic manifestations in patients with EDS; much remains to be learnt about the relationships between nodules and the epileptic manifestations in EDS syndrome. Copyright © 2014 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Sleep respiratory parameters in children with idiopathic epilepsy: A cross-sectional study.

Science.gov (United States)

Gogou, Maria; Haidopoulou, Katerina; Eboriadou, Maria; Pavlidou, Efterpi; Hatzistylianou, Maria; Pavlou, Evaggelos

2016-10-01

The aim of this study is to explore and compare through polysomnography respiratory sleep parameters between children with idiopathic epilepsy and healthy children. Our cross-sectional study included 40 children with idiopathic epilepsy and 27 healthy children, who underwent overnight polysomnography. Data about sleep respiratory parameters were obtained and statistically analyzed. The level of statistical significance was set at 0.05. The prevalence of Obstructive Sleep Apnea Syndrome was significantly higher in the epilepsy group (35% vs 7.4%, pepilepsy group was 10.6 (95% Confidence Intervals: 3.08-37.08) in comparison to the control group. The mean value of the obstructive apnea-hypopnea index was significantly higher in children with epilepsy compared to healthy children (2.46±1.22 vs 1.21±0.83, p=0.027). The mean values of central apnea index and desaturation index were comparable between these two groups. Longest apnea duration was significantly higher in the group of poor seizure control. All other sleep respiratory variables did not differ significantly between children with poor and good seizure control and between children with generalized and focal epilepsy. Children with epilepsy seem to present more prominent sleep breathing instability in comparison to healthy children, which mainly includes a predisposition to obstructive respiratory events. More studies are needed to investigate the relationship between sleep apneas and seizure control. Copyright © 2016 Elsevier B.V. All rights reserved.

Lateralizing value of unilateral relative ictal immobility in patients with refractory focal seizures--Looking beyond unilateral automatisms.

Science.gov (United States)

Agarwal, Priya; Kaul, Bhavna; Shukla, Garima; Srivastava, Achal; Singh, Mamta Bhushan; Goyal, Vinay; Behari, Madhuri; Suri, Ashish; Gupta, Aditya; Garg, Ajay; Gaikwad, Shailesh; Bal, C S

2015-12-01

Ictal motor phenomena play a crucial role in the localization of seizure focus in the management of refractory focal epilepsy. While the importance of unilateral automatisms is well established, little attention is paid to the contralateral relatively immobile limb. In cases where automatisms mimic clonic or dystonic movements and in the absence of previously well-established signs, unilateral relative ictal immobility (RII) is potentially useful as a lateralizing sign. This study was carried out to examine the lateralizing value of this sign and to define its characteristics among patients of refractory focal epilepsy. VEEGs of 69 consecutive patients of refractory focal epilepsy who had undergone epilepsy surgery at our center over last four years were reviewed and analyzed for the presence of RII. Unilateral RII was defined as a paucity of movement in one limb lasting for at least 10s while the contralateral limb showed purposive or semi-purposive movements (in the absence of tonic or dystonic posturing or clonic movements in the involved limb). The findings were seen in the light of VEEG, radiological and nuclear imaging data, and with post-surgical outcome. Unilateral RII as a lateralizing sign was found in 24 of 69 patients (34.78%), consisting of both temporal and extra temporal epilepsy, with 100% concordance with VEEG and MRI data. All patients demonstrating this sign had a good post-surgical outcome. RII, when well characterized is a frequent and reliable lateralizing sign in patients of refractory focal epilepsy. Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Electroencephalographic patterns in Ethiopian patients with epilepsy

African Journals Online (AJOL)

2mikitser

Conclusion: The most common EEG abnormalities in Ethiopian patients with epilepsy are focal interictal epileptiform discharges, typically ..... EEG is a valuable investigative tool. In the advent of more sophisticated neuroimaging methods, epilepsy remains one of the few common clinical problems routinely demanding EEG.

LGI2 truncation causes a remitting focal epilepsy in dogs.

Directory of Open Access Journals (Sweden)

Eija H Seppälä

2011-07-01

Full Text Available One quadrillion synapses are laid in the first two years of postnatal construction of the human brain, which are then pruned until age 10 to 500 trillion synapses composing the final network. Genetic epilepsies are the most common neurological diseases with onset during pruning, affecting 0.5% of 2-10-year-old children, and these epilepsies are often characterized by spontaneous remission. We previously described a remitting epilepsy in the Lagotto romagnolo canine breed. Here, we identify the gene defect and affected neurochemical pathway. We reconstructed a large Lagotto pedigree of around 34 affected animals. Using genome-wide association in 11 discordant sib-pairs from this pedigree, we mapped the disease locus to a 1.7 Mb region of homozygosity in chromosome 3 where we identified a protein-truncating mutation in the Lgi2 gene, a homologue of the human epilepsy gene LGI1. We show that LGI2, like LGI1, is neuronally secreted and acts on metalloproteinase-lacking members of the ADAM family of neuronal receptors, which function in synapse remodeling, and that LGI2 truncation, like LGI1 truncations, prevents secretion and ADAM interaction. The resulting epilepsy onsets at around seven weeks (equivalent to human two years, and remits by four months (human eight years, versus onset after age eight in the majority of human patients with LGI1 mutations. Finally, we show that Lgi2 is expressed highly in the immediate post-natal period until halfway through pruning, unlike Lgi1, which is expressed in the latter part of pruning and beyond. LGI2 acts at least in part through the same ADAM receptors as LGI1, but earlier, ensuring electrical stability (absence of epilepsy during pruning years, preceding this same function performed by LGI1 in later years. LGI2 should be considered a candidate gene for common remitting childhood epilepsies, and LGI2-to-LGI1 transition for mechanisms of childhood epilepsy remission.

Neurological autoantibodies in drug-resistant epilepsy of unknown cause.

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Tecellioglu, Mehmet; Kamisli, Ozden; Kamisli, Suat; Yucel, Fatma Ebru; Ozcan, Cemal

2018-03-09

Autoimmune epilepsy is a rarely diagnosed condition. Recognition of the underlying autoimmune condition is important, as these patients can be resistant to antiepileptic drugs. To determine the autoimmune and oncological antibodies in adult drug-resistant epilepsy of unknown cause and identify the clinical, radiological, and EEG findings associated with these antibodies according to data in the literature. Eighty-two patients with drug-resistant epilepsy of unknown cause were prospectively identified. Clinical features were recorded. The levels of anti-voltage-gated potassium channel complex (anti-VGKCc), anti-thyroid peroxidase (anti-TPO), anti-nuclear antibody (ANA), anti-glutamic acid decarboxylase (anti-GAD), anti-phospholipid IgG and IgM, anti-cardiolipin IgG and IgM, and onconeural antibodies were determined. Serum antibody positivity suggesting the potential role of autoimmunity in the aetiology was present in 17 patients with resistant epilepsy (22.0%). Multiple antibodies were found in two patients (2.6%). One of these patients (1.3%) had anti-VGKCc and ANA, whereas another (1.3%) had anti-VGKCc and anti-TPO. A single antibody was present in 15 patients (19.5%). Of the 77 patients finally included in the study, 4 had anti-TPO (5.2%), 1 had anti-GAD (1.3%), 4 had anti-VGKCc (5.2%) 8 had ANA (10.3%), and 2 had onconeural antibodies (2.6%) (1 patient had anti-Yo and 1 had anti-MA2/TA). The other antibodies investigated were not detected. EEG abnormality (focal), focal seizure incidence, and frequent seizures were more common in antibody-positive patients. Autoimmune factors may be aetiologically relevant in patients with drug-resistant epilepsy of unknown cause, especially if focal seizures are present together with focal EEG abnormality and frequent seizures.

Multifocal epilepsy: the role of palliative resection - intractable frontal and occipital lobe epilepsy secondary to radiotherapy for acute lymphoblastic leukaemia.

Science.gov (United States)

Radhakrishnan, Ashalatha; Sithinamsuwan, Pasiri; Harvey, A Simon; Flanagan, Danny; Fitt, Gregory; Berlangieri, Sam; Jackson, Graeme D; Berkovic, Samuel F; Scheffer, Ingrid E

2008-12-01

Patients with multifocal epilepsy are often considered unsuitable for epilepsy surgery. We report an adolescent with intractable frontal and occipital lobe seizures, secondary to complications of treatment for acute lymphoblastic leukaemia as a young child. Chemotherapy and radiotherapy were complicated by bilateral, posterior leukoencephalopathy and later an acquired frontal cerebral cavernous malformation (CCM). Detailed electro-clinical and imaging studies showed multiple, frontal lobe seizures per day with less frequent and non-debilitating, simple, occipital lobe seizures. Focal resection of the frontal CCM abolished the socially-disabling seizures with resultant marked improvement in the patient's quality of life at 12 months. Careful analysis of the type and impact of focal seizures in the setting of multifocal epilepsy may demonstrate that one seizure type is more deleterious to quality of life and may be amenable to surgery. In this situation, the patient may benefit significantly from surgery to resect the more active epileptic focus.

Positron emission CT on post-traumatic epilepsy

International Nuclear Information System (INIS)

Tsukiyama, Takashi; Tsubokawa, Takashi; Doi, Nobuyasu; Sato, Kohten; Iio, Masaaki.

1983-01-01

Six patients suffering from post-traumatic epilepsy were checked by encephalography (EEG), X-ray CT and cerebral positron emission computed tomography (PECT) using 11 C-carbon dioxide ( 11 CO 2 ) and 11 C-glucoses as indicators of the local cerebral circulation and local cerebral glucose utilization, in order to assess the diagnostic value of PECT in post-traumatic epilepsy. In those patients (4 cases) who had focal electrical abnormalities or X-ray CT lesions, PECT clearly revealed localized regions of decreased cerebral circulation and glucose utilization. A focal hypometabolic zone also appeared in the post-traumatic epilepsy (1 case) which had a normal X-ray CT. One case, who had been treated for several years by medication but showed no EEG change and no abnormality on X-ray CT, revealed a normal circulation and metabolism by RECT. This case did not require any further medication for epilepsy. It is concluded that positron emission CT represents a useful diagnostic method for post-traumatic epilepsy which does not demonstrate any abnormality on X-ray CT. (author)

Epilepsy

Energy Technology Data Exchange (ETDEWEB)

Fisher, R.S.; Frost, J.J. (Johns Hopkins Univ., Baltimore, MD (USA))

1991-04-01

As surgical treatments for adult and pediatric forms of epilepsy have become more refined, methods for noninvasive localization of epileptogenic foci have become increasingly important. Detection of focal brain metabolic or flow abnormalities is now well recognized as an essential step in the presurgical evaluation of many patients with epilepsy. Positron emission tomography (PET) scanning is most beneficial when used in the context of the total clinical evaluation of patients, including scalp EEG, invasive EEG, neuropsychologic testing, etc. Metabolic PET studies also give insight into pathophysiologic mechanisms of epilepsy. The dynamic nature of the interictal hypometabolism observed with 18(F)FDG in some patients suggests that excitatory or inhibitory neurotransmitters and their receptors may be involved. An exciting current application of PET scanning is the use of tracers for neurotransmitter receptors in the study of epilepsy patients. Mu and non-mu opiate receptors have been extensively studied and are beginning to give new insights into this disorder. Increased labeling of mu receptors in temporal neocortex using 11C-carfentanil has been demonstrated and, in some patients, supplements the clinical localization information from 18(F)FDG studies. Increased mu opiate receptor number or affinity is thought to play a role in anticonvulsant mechanisms. Specificity of increased mu receptors is supported by the absence of significant changes in non-mu opiate receptors. Other brain receptors are also of interest for future studies, particularly those for excitatory neurotransmitters. Combined studies of flow, metabolism, and neuroreceptors may elucidate the factors responsible for initiation and termination of seizures, thus improving patient treatment.95 references.

Epilepsy

International Nuclear Information System (INIS)

Fisher, R.S.; Frost, J.J.

1991-01-01

As surgical treatments for adult and pediatric forms of epilepsy have become more refined, methods for noninvasive localization of epileptogenic foci have become increasingly important. Detection of focal brain metabolic or flow abnormalities is now well recognized as an essential step in the presurgical evaluation of many patients with epilepsy. Positron emission tomography (PET) scanning is most beneficial when used in the context of the total clinical evaluation of patients, including scalp EEG, invasive EEG, neuropsychologic testing, etc. Metabolic PET studies also give insight into pathophysiologic mechanisms of epilepsy. The dynamic nature of the interictal hypometabolism observed with 18[F]FDG in some patients suggests that excitatory or inhibitory neurotransmitters and their receptors may be involved. An exciting current application of PET scanning is the use of tracers for neurotransmitter receptors in the study of epilepsy patients. Mu and non-mu opiate receptors have been extensively studied and are beginning to give new insights into this disorder. Increased labeling of mu receptors in temporal neocortex using 11C-carfentanil has been demonstrated and, in some patients, supplements the clinical localization information from 18[F]FDG studies. Increased mu opiate receptor number or affinity is thought to play a role in anticonvulsant mechanisms. Specificity of increased mu receptors is supported by the absence of significant changes in non-mu opiate receptors. Other brain receptors are also of interest for future studies, particularly those for excitatory neurotransmitters. Combined studies of flow, metabolism, and neuroreceptors may elucidate the factors responsible for initiation and termination of seizures, thus improving patient treatment.95 references

Sequential motor task (Luria's Fist-Edge-Palm Test in children with benign focal epilepsy of childhood with centrotemporal spikes

Directory of Open Access Journals (Sweden)

Carmen Silvia Molleis Galego Miziara

2013-06-01

Full Text Available This study evaluated the sequential motor manual actions in children with benign focal epilepsy of childhood with centrotemporal spikes (BECTS and compares the results with matched control group, through the application of Luria's fist-edge-palm test. The children with BECTS underwent interictal single photon emission computed tomography (SPECT and School Performance Test (SPT. Significant difference occurred between the study and control groups for manual motor action through three equal and three different movements. Children with lower school performance had higher error rate in the imitation of hand gestures. Another factor significantly associated with the failure was the abnormality in SPECT. Children with BECTS showed abnormalities in the test that evaluated manual motor programming/planning. This study may suggest that the functional changes related to epileptiform activity in rolandic region interfere with the executive function in children with BECTS.

Intraoperative Magnetic-Resonance Tomography and Neuronavigation During Resection of Focal Cortical Dysplasia Type II in Adult Epilepsy Surgery Offers Better Seizure Outcomes.

Science.gov (United States)

Roessler, Karl; Kasper, Burkhard S; Heynold, Elisabeth; Coras, Roland; Sommer, Björn; Rampp, Stefan; Hamer, Hajo M; Blümcke, Ingmar; Buchfelder, Michael

2018-01-01

Focal cortical dysplasia (FCD) is one important cause of drug-resistant epilepsy potentially curable by epilepsy surgery. We investigated the options of using neuronavigation and intraoperative magnetic-resonance tomographical imaging (MRI) to avoid residual epileptogenic tissue during resection of patients with FCD II to improve seizure outcome. Altogether, 24 patients with FCD II diagnosed by MRI (16 female, 8 male; mean age 34 ± 10 years) suffered from drug-resistant electroclinical and focal epilepsy for a mean of 20.7 ± 5 years. Surgery was performed with preoperative stereoelectroencephalography (in 15 patients), neuronavigation, and intraoperative 1.5T-iopMRI in all 24 investigated patients. In 75% of patients (18/24), a complete resection was performed. In 89% (16/18) of completely resected patients, we documented an Engel I seizure outcome after a mean follow-up of 42 months. All incompletely resected patients had a worse outcome (Engel II-III, P < 0.0002). Patients with FCD IIB had also significant better seizure outcome compared with patients diagnosed as having FCD IIA (82% vs. 28%, P < 0.02). In 46% (11/24) of patients, intraoperative second-look surgeries due to residual lesions detected during the intraoperative MRI were performed. In these 11 patients, there were significant more completely seizure free patients (73% vs. 38% Engel IA), compared with 13 patients who finished surgery after the first intraoperative MRI (P < 0.05). Excellent seizure outcome after surgery of patients with FCD II positively correlated with the amount of resection, histologic subtype, and the use of intraoperative MRI, especially when intraoperative second-look surgeries were performed. Copyright © 2017 Elsevier Inc. All rights reserved.

Initial Treatment for Nonsyndromic Early-Life Epilepsy: An Unexpected Consensus.

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Shellhaas, Renée A; Berg, Anne T; Grinspan, Zachary M; Wusthoff, Courtney J; Millichap, John J; Loddenkemper, Tobias; Coryell, Jason; Saneto, Russell P; Chu, Catherine J; Joshi, Sucheta M; Sullivan, Joseph E; Knupp, Kelly G; Kossoff, Eric H; Keator, Cynthia; Wirrell, Elaine C; Mytinger, John R; Valencia, Ignacio; Massey, Shavonne; Gaillard, William D

2017-10-01

There are no evidence-based guidelines on the preferred approach to treating early-life epilepsy. We examined initial therapy selection in a contemporary US cohort of children with newly diagnosed, nonsyndromic, early-life epilepsy (onset before age three years). Seventeen pediatric epilepsy centers participated in a prospective cohort study of children with newly diagnosed epilepsy with onset under 36 months of age. Details regarding demographics, seizure types, and initial medication selections were obtained from medical records. About half of the 495 enrolled children with new-onset, nonsyndromic epilepsy were less than 12 months old at the time of diagnosis (n = 263, 53%) and about half (n = 260, 52%) had epilepsy with focal features. Of 464 who were treated with monotherapy, 95% received one of five drugs: levetiracetam (n = 291, 63%), oxcarbazepine (n = 67, 14%), phenobarbital (n = 57, 12%), topiramate (n = 16, 3.4%), and zonisamide (n = 13, 2.8%). Phenobarbital was prescribed first for 50 of 163 (31%) infants less than six months old versus seven of 300 (2.3%) of children six months or older (P epilepsy presentation (focal, generalized, mixed/uncertain). Between the first and second treatment choices, 367 (74%) of children received levetiracetam within the first year after diagnosis. Without any specific effort, the pediatric epilepsy community has developed an unexpectedly consistent approach to initial treatment selection for early-life epilepsy. This suggests that a standard practice is emerging and could be utilized as a widely acceptable basis of comparison in future drug studies. Copyright © 2017 Elsevier Inc. All rights reserved.

Excitation in temporal lobe epilepsy : focus on the glutamate-glutamine cycle

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van der Hel, W.S.

2009-01-01

Epilepsy is a common neurological disorder. Temporal lobe epilepsy (TLE) is the most frequent type of human focal epilepsy. Despite ample availability of anti-epileptic dugs, about 30% of TLE patients are pharmaco-resistant. Surgical removal of the epileptogenic focus, which usually includes the

A Functional-genetic Scheme for Seizure Forecasting in Canine Epilepsy.

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Bou Assi, E; Nguyen, D K; Rihana, S; Sawan, M

2017-09-13

The objective of this work is the development of an accurate seizure forecasting algorithm that considers brain's functional connectivity for electrode selection. We start by proposing Kmeans-directed transfer function, an adaptive functional connectivity method intended for seizure onset zone localization in bilateral intracranial EEG recordings. Electrodes identified as seizure activity sources and sinks are then used to implement a seizure-forecasting algorithm on long-term continuous recordings in dogs with naturallyoccurring epilepsy. A precision-recall genetic algorithm is proposed for feature selection in line with a probabilistic support vector machine classifier. Epileptic activity generators were focal in all dogs confirming the diagnosis of focal epilepsy in these animals while sinks spanned both hemispheres in 2 of 3 dogs. Seizure forecasting results show performance improvement compared to previous studies, achieving average sensitivity of 84.82% and time in warning of 0.1. Achieved performances highlight the feasibility of seizure forecasting in canine epilepsy. The ability to improve seizure forecasting provides promise for the development of EEGtriggered closed-loop seizure intervention systems for ambulatory implantation in patients with refractory epilepsy.

Psychiatric comorbidity in patients with two prototypes of focal versus generalized epilepsy syndromes.

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Filho, Gerardo Maria de Araújo; Mazetto, Lenon; da Silva, Joyce Macedo; Caboclo, Luís Otávio Sales Ferreira; Yacubian, Elza Márcia Targas

2011-06-01

The frequency of psychiatric disorders (PD) in a homogeneous series of patients with temporal lobe epilepsy with mesial temporal sclerosis (TLE-MTS) compared to patients with juvenile myoclonic epilepsy (JME) was evaluated, aiming to determine the frequency of PD and possible differences in psychiatric diagnoses between these two epileptic syndromes. Data from 248 patients with refractory TLE-MTS and from 124 JME patients were reviewed and compared. There was a high prevalence of PD in both groups of epilepsy patients, present in 100 TLE-MTS (41%) and in 58 JME patients (46.7%). Mood (23.7%), anxiety (13.7%) and psychotic (11.6%) disorders were the most frequent diagnoses in TLE-MTS group, while mood and anxiety disorders (25% and 21%, respectively) were the most common PD among JME. Psychoses were significantly associated with TLE-MTS (p=0.01). These observations are concordant with our previous study, reforcing the existence of a possible anatomic correlation of PD and brain structures involved in both epilepsy syndromes. Copyright © 2011 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Epilepsy in adults with mitochondrial disease: A cohort study.

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Whittaker, Roger G; Devine, Helen E; Gorman, Grainne S; Schaefer, Andrew M; Horvath, Rita; Ng, Yi; Nesbitt, Victoria; Lax, Nichola Z; McFarland, Robert; Cunningham, Mark O; Taylor, Robert W; Turnbull, Douglass M

2015-12-01

The aim of this work was to determine the prevalence and progression of epilepsy in adult patients with mitochondrial disease. We prospectively recruited a cohort of 182 consecutive adult patients attending a specialized mitochondrial disease clinic in Newcastle upon Tyne between January 1, 2005 and January 1, 2008. We then followed this cohort over a 7-year period, recording primary outcome measures of occurrence of first seizure, status epilepticus, stroke-like episode, and death. Overall prevalence of epilepsy in the cohort was 23.1%. Mean age of epilepsy onset was 29.4 years. Prevalence varied widely between genotypes, with several genotypes having no cases of epilepsy, a prevalence of 34.9% in the most common genotype (m.3243A>G mutation), and 92.3% in the m.8344A>G mutation. Among the cohort as a whole, focal seizures, with or without progression to bilateral convulsive seizures, was the most common seizure type. Conversely, all of the patients with the m.8344A>G mutation and epilepsy experienced myoclonic seizures. Patients with the m.3243A>G mutation remain at high risk of developing stroke-like episodes (1.16% per year). However, although the standardized mortality ratio for the entire cohort was high (2.86), this ratio did not differ significantly between patients with epilepsy (2.96) and those without (2.83). Epilepsy is a common manifestation of mitochondrial disease. It develops early in the disease and, in the case of the m.3243A>G mutation, often presents in the context of a stroke-like episode or status epilepticus. However, epilepsy does not itself appear to contribute to the increased mortality in mitochondrial disease. © 2015 The Authors. Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association.

Changes in the frequency of benign focal spikes accompany changes in central information processing speed : a prospective 2-year follow-up study

NARCIS (Netherlands)

Ebus, S.C.M.; IJff, D.M.; den Boer, J.T.; Debeij-van Hall, M.H.J.A.; Klinkenberg, S.; van der Does, A.; Boon, P.J.; Arends, J.B.A.M.; Aldenkamp, A.P.

We prospectively examined whether changes in the frequency of benign focal spikes accompany changes in cognition. Twenty-six children with benign focal spikes (19 with Rolandic epilepsy) and learning difficulties were examined with repeated 24-hour EEG recordings, three cognitive tests on central

Electrophysiological and pathological study of focal cortical dysplasia

International Nuclear Information System (INIS)

Hodozuka, Akira; Hashizume, Kiyotaka; Hayashi, Yoshimitsu; Tanaka, Tatsuya

2008-01-01

Clinical and experimental studies on focal cortical dysplasia (FCD) were carried out. For the experimental study, an experimental FCD model of rats was developed. Twenty Wistar rats at 0-2 days after birth were used for the study. Kainic acid (KA) solution was injected stereotaxically into medial and lateral sites of the sensori-motor cortex. Bipolar electrodes were inserted. The behavior of the rats and electroencephalography (EEG) were recorded using a digital video-EEG monitoring system. After observation periods of 1, 2 and 6 months, the rats were perfused for pathological study. FCD was observed adjacent to the site of KA injection in all rats more than one month after the injection. EEG recording demonstrated focal spike discharges in and around the site of injection. However, clinical seizure was not observed. Pathological studies showed decrease in gamma aminobutyric acid (GABA)-A receptors and increase in GABA-B receptors not only in the lesion but also in perilesional areas. Fifteen surgical cases of FCD with intractable epilepsy were included in the clinical study. Neuro-imaging studies including high-resolution MRI and single photon emission computed tomography (SPECT) were performed. Conventional EEG studies demonstrated focal EEG abnormalities with epileptic phenomena. At surgery, intraoperative electrocorticography (ECoG) was performed in order to localize epileptic foci under neuroleptanalgesia. Fourteen patients showed epileptiform discharges on preresection ECoG. All foci in non-eloquent areas were resected. Pathological studies including immunohistochemical staining were performed, and characteristics of the FCD in relation to EEG findings were analyzed. Electrophysiological examination revealed epileptogenecity not only in the lesions but also in perilesional areas. In the lesions, immunohistochemical studies showed decrease in GABA-A receptors and increase in GABA-B receptors in both the lesions and perilesional areas, but N

Antithyroid Antibodies Are Implicated in Epileptogenesis of Adult Patients With Epilepsy.

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Tsai, Meng-Han; Fu, Ting-Ying; Chen, Nai-Ching; Shih, Fu-Yuan; Lu, Yan-Ting; Cheng, Mei-Yun; Chuang, Hung-Yi; Chuang, Yao-Chung

2015-07-01

Antithyroid antibodies (Abs) are associated with epilepsy in steroid-responsive encephalopathy, but have been rarely studied in unselected epilepsy patients. This study aimed to characterize the prevalence and associated factors of antithyroid Abs and other auto-Abs in adult patients with epilepsy.Epilepsy patients without autoimmune disorders were surveyed for antinuclear antibody (ANA), anti-β2 glycoprotein 1 antibody (aβ2GP1), anticardiolipin IgG Ab, antimicrosomal antibody (AMA), antithyroglobulin antibody (ATA), and thyroid function test.Of 319 patients, 75 (23.5%) were positive for at least 1 Ab. The most common Ab was anticardiolipin antibody (aCL) (30/319, 9.4%), followed by AMA (24/319, 7.5%), ANA (18/319, 5.6%), aβ2GP1 (18/319, 6.5%), and ATA (6/319, 3.25%). Antimicrosomal Abs were significantly more frequent in patients who were female, older at disease onset, older at the time of study, and had unknown seizure etiology. The presence of aCL was significantly associated with more frequent seizures. Most patients with antithyroid Ab were female and had focal seizures with unknown etiology.The association of different auto-Abs with different factors suggests that they may have different roles in adult patients with epilepsy. Recurrent seizures and certain antiepileptic medications may cause the production of aCL. The role of antithyroid Abs in adult focal epilepsy with unknown cause, especially in females, warrants further evaluation because of the potential implications on treatment.

RBFOX1 and RBFOX3 mutations in rolandic epilepsy

DEFF Research Database (Denmark)

Lal, Dennis; Reinthaler, Eva M; Altmüller, Janine

2013-01-01

Partial deletions of the gene encoding the neuronal splicing regulator RBFOX1 have been reported in a range of neurodevelopmental diseases, including idiopathic generalized epilepsy. The RBFOX1 protein and its homologues (RBFOX2 and RBFOX3) regulate alternative splicing of many neuronal transcripts...... involved in the homeostatic control of neuronal excitability. In this study, we explored if structural microdeletions and exonic sequence variations in RBFOX1, RBFOX2, RBFOX3 confer susceptibility to rolandic epilepsy (RE), a common idiopathic focal childhood epilepsy. By high-density SNP array screening...... that exon deletions and truncating mutations of RBFOX1 and RBFOX3 contribute to the genetic variance of partial and generalized idiopathic epilepsy syndromes....

Comparative effectiveness of eight antiepileptic drugs in adults with focal refractory epilepsy: the influence of age, gender, and the sequence in which drugs were introduced onto the market.

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Mäkinen, Jussi; Peltola, Jukka; Raitanen, Jani; Alapirtti, Tiina; Rainesalo, Sirpa

2017-07-01

The first objective was to determine the long-term retention rate of eight antiepileptic drugs (AEDs) commonly used as adjunctive therapy in adults with focal refractory epilepsy. Second, we assessed the effects of age and gender on retention rates. Third, we examined if the retention rate could be influenced by the sequence in which the AEDs had entered the market. Patients with focal refractory epilepsy treated with any of the eight AEDs in Tampere University Hospital were identified retrospectively (N = 507). Retention rates were evaluated with the Kaplan-Meier method. Follow-up started at the first date of treatment and each individual was followed a maximum of 36 months. We calculated the following 3-year retention rates: lacosamide 77.1% (N = 137), lamotrigine 68.3% (N = 177), levetiracetam 66.7% (N = 319), clobazam 65.6% (N = 130), topiramate 61.6% (N = 178), zonisamide 60.4% (N = 103), pregabalin 54.6% (N = 127), and gabapentin 40.2% (N = 66). Lacosamide, levetiracetam, and clobazam were the most effective AEDs in the elderly. The retention rate for pregabalin was higher in males (65%) than females (51%) whereas females had higher retention rates for both topiramate (72 vs. 58%) and zonisamide (67 vs. 57%). The retention rate was influenced by the sequence in which these AEDs entered the market. We provide important information about practical aspects of these eight AEDs, revealing that there are differences in their effectiveness as adjunctive treatment for focal refractory epilepsy. Most importantly, the retention rate appears to be influenced by the sequence in which these AEDs were introduced onto the market.

Presence of Cognitive Deterioration and Anatomical-Clinical Topography in Patients with Epilepsy in Cienfuegos

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Rolando Lázaro Rivera López

2015-02-01

Full Text Available Background: epilepsy is a pathological condition characterized by a recurrent non-provoked crisis, however, the presence of the crisis is a fraction of the global problem, patients with epilepsy develop a variety of neuropsychiatry problems, as cognitive affection, most of all, in the space of memory. Objective: evaluating the behavior of the cognitive deterioration and focalization according to anatomical- clinical topography in patients with epilepsy. Methods: a descriptive, correlational, cross-section and follow-up study of cases. The techniques used were: structured interview, the Montreal Cognitive Assessment's evaluation, and Luria´s neuropsychological exam. It was used SPDD statical parcel, version 1.5 to process the information that made possible the study of the obtained data, with the aim of expressing the results in chart of frequency and relation of variables in number and percent. Results: the 71.4 % of evaluated patients presented cognitive deterioration in any of its of measurement scales and they focalized according to neuropsychological exam. Conclusions: as the time of evolution of the disease increases, the frequency and duration of the crises, the grade of the cognitive deterioration in patients with epilepsy increases, focalizing with dysfunction majority fronto-temporary level according to anatomical-clinical topography.

Language recovery after epilepsy surgery of the Broca's area

Directory of Open Access Journals (Sweden)

Lilit Mnatsakanyan

Full Text Available Epilepsy surgery is indicated in select patients with drug-resistant focal epilepsy. Seizure freedom or significant reduction of seizure burden without risking new neurological deficits is the expected goal of epilepsy surgery. Typically, when the seizure onset zone overlaps with eloquent cortex, patients are excluded from surgery.We present a patient with drug-resistant frontal lobe epilepsy who underwent successful surgery with resection of Broca's area, primarily involving the pars triangularis (BA 45. We report transient expressive aphasia followed by recovery of speech. This case provides new insights into adult neuroplasticity of the language network. Keywords: Epilepsy surgery, Eloquent cortex, Language and epilepsy, Refractory epilepsy, Electrocorticography

Dostoevsky's epilepsy: a new approach to retrospective diagnosis.

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Rayport, Shirley M Ferguson; Rayport, Mark; Schell, Carolyn A

2011-11-01

There has been considerable debate about Fyodor Dostoevsky's epilepsy. Was his epilepsy generalized or focal? Was the dramatic ecstatic experience an epileptic phenomenon or a literary invention? We compared probable epileptically related behavioral manifestations in The Idiot with experiences of current patients, studied with a modern interdisciplinary approach involving neurosurgery, neurology, and neuropsychiatry. Patients were studied by all disciplines starting with their initial evaluation and trial of antiseizure medication, during hospitalization for long-term monitoring for epilepsy at scalp and depth levels with electrical brain stimulation, during intraoperative interviews, and in long-term postoperative follow-up. Behavioral manifestations, clearly shown to be phenomena of the epilepsy in our patients, were the template for defining the epileptic nature of the behavioral symptomatology described by Dostoevsky in his literary character, Prince Myshkin. We conclude that Dostoevsky had temporolimbic epilepsy and that the ecstatic experience is an epileptic phenomenon. Copyright © 2011 Elsevier Inc. All rights reserved.

Multiplex families with epilepsy

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Afawi, Zaid; Oliver, Karen L.; Kivity, Sara; Mazarib, Aziz; Blatt, Ilan; Neufeld, Miriam Y.; Helbig, Katherine L.; Goldberg-Stern, Hadassa; Misk, Adel J.; Straussberg, Rachel; Walid, Simri; Mahajnah, Muhammad; Lerman-Sagie, Tally; Ben-Zeev, Bruria; Kahana, Esther; Masalha, Rafik; Kramer, Uri; Ekstein, Dana; Shorer, Zamir; Wallace, Robyn H.; Mangelsdorf, Marie; MacPherson, James N.; Carvill, Gemma L.; Mefford, Heather C.; Jackson, Graeme D.; Scheffer, Ingrid E.; Bahlo, Melanie; Gecz, Jozef; Heron, Sarah E.; Corbett, Mark; Mulley, John C.; Dibbens, Leanne M.; Korczyn, Amos D.

2016-01-01

Objective: To analyze the clinical syndromes and inheritance patterns of multiplex families with epilepsy toward the ultimate aim of uncovering the underlying molecular genetic basis. Methods: Following the referral of families with 2 or more relatives with epilepsy, individuals were classified into epilepsy syndromes. Families were classified into syndromes where at least 2 family members had a specific diagnosis. Pedigrees were analyzed and molecular genetic studies were performed as appropriate. Results: A total of 211 families were ascertained over an 11-year period in Israel. A total of 169 were classified into broad familial epilepsy syndrome groups: 61 generalized, 22 focal, 24 febrile seizure syndromes, 33 special syndromes, and 29 mixed. A total of 42 families remained unclassified. Pathogenic variants were identified in 49/211 families (23%). The majority were found in established epilepsy genes (e.g., SCN1A, KCNQ2, CSTB), but in 11 families, this cohort contributed to the initial discovery (e.g., KCNT1, PCDH19, TBC1D24). We expand the phenotypic spectrum of established epilepsy genes by reporting a familial LAMC3 homozygous variant, where the predominant phenotype was epilepsy with myoclonic-atonic seizures, and a pathogenic SCN1A variant in a family where in 5 siblings the phenotype was broadly consistent with Dravet syndrome, a disorder that usually occurs sporadically. Conclusion: A total of 80% of families were successfully classified, with pathogenic variants identified in 23%. The successful characterization of familial electroclinical and inheritance patterns has highlighted the value of studying multiplex families and their contribution towards uncovering the genetic basis of the epilepsies. PMID:26802095

Surgical Treatment of Nonlesional Neocortical Epilepsy: Long-term Longitudinal Study.

Science.gov (United States)

Kim, Dong Wook; Lee, Sang Kun; Moon, Hye-Jin; Jung, Ki-Young; Chu, Kon; Chung, Chun-Ki

2017-03-01

The proportion of surgery for nonlesional neocortical epilepsy has recently increased, with a decrease in surgery for mesial temporal lobe epilepsy. However, there are only a few studies regarding the long-term surgical outcome and the potential prognostic factors for patients with nonlesional neocortical epilepsy. To evaluate the long-term surgical outcome and to identify possible prognostic factors in patients with nonlesional neocortical epilepsy. In a surgical cohort from September 1995 to December 2005 at the Seoul National University Hospital, we included 109 consecutive patients without lesions identifiable by magnetic resonance imaging who underwent focal surgical resection for drug-resistant neocortical epilepsy. Follow-up information for at least 10 years was available for all but 1 patient. Univariate and standard multiple logistic regression analyses were performed to identify the predictors of surgical outcomes, and a generalized estimation equation model was used for the longitudinal multiple logistic regression analysis of up to 21 years of follow-up. The patients consisted of 64 men and 45 women with ages at surgery ranging from 7 to 56 years (mean [SD], 27.1 [7.8] years). At 1 year after surgery, 59 of 109 patients (54.1%) achieved seizure freedom, and 64 of 108 patients (59.3%) achieved seizure freedom at the last follow-up. Only 11 of 108 patients (10.2%) experienced definite changes in postoperative seizure status. Localizing patterns in functional neuroimaging (strongest odds ratio [OR], 0.30 [95% CI, 0.14-0.66] for fluorodeoxyglucose-positron emission tomography; 0.37 [95% CI, 0.15-0.87] for ictal single-photon emission computed tomography), concordant results in presurgical diagnostic evaluations (OR, 3.15 [95% CI, 1.42-7.02]), the presence of aura (OR, 3.49 [95% CI, 1.54-7.92]), and complete resection of areas of ictal onset with frequent interictal spikes during the intracranial electroencephalographic study (OR, 0.37 [95% CI, 0

Identification of a novel idiopathic epilepsy locus in Belgian Shepherd dogs

DEFF Research Database (Denmark)

Seppälä, Eija H.; Koskinen, Lotta L.E.; Gulløv, Christina Hedal

2012-01-01

Epilepsy is the most common neurological disorder in dogs, with an incidence ranging from 0.5% to up to 20% in particular breeds. Canine epilepsy can be etiologically defined as idiopathic or symptomatic. Epileptic seizures may be classified as focal with or without secondary generalization...... collected 159 cases and 148 controls and confirmed the presence of epilepsy through epilepsy questionnaires and clinical examinations. The MRI was normal while interictal EEG revealed abnormalities and variable foci in the clinically examined affected dogs. A genome-wide association study using Affymetrix...... mutation. It would establish the affected breed as a novel therapeutic model, help to develop a DNA test for breeding purposes and introduce a novel candidate gene for human idiopathic epilepsies....

Novel approaches to epilepsy treatment

DEFF Research Database (Denmark)

Sørensen, Andreas T; Kokaia, Merab

2013-01-01

The aim of epilepsy treatment is to achieve complete seizure freedom. Nonetheless, numerous side effects and seizure resistance to antiepileptic drugs (AEDs) affecting about 30-40% of all patients are main unmet needs in today's epileptology. For this reason, novel approaches to treat epilepsy......, and inhibitory neurotransmitters. We also address new molecular-genetic approaches utilizing optogenetic technology. The therapeutic strategies presented herein are predominately aimed toward treatment of partial/focal epilepsies, but could also be envisaged for targeting key seizure propagation areas...... are highly needed. Herein, we highlight recent progress in stem-cell-based and gene transfer-based therapies in epilepsy according to findings in animal models and address their potential clinical application. Multiple therapeutic targets are described, including neuropeptides, neurotrophic factors...

Natural evolution from idiopathic photosensitive occipital lobe epilepsy to idiopathic generalized epilepsy in an untreated young patient.

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Bonini, Francesca; Egeo, Gabriella; Fattouch, Jinan; Fanella, Martina; Morano, Alessandra; Giallonardo, Anna Teresa; di Bonaventura, Carlo

2014-04-01

Idiopathic photosensitive occipital lobe epilepsy (IPOE) is an idiopathic localization-related epilepsy characterized by age-related onset, specific mode of precipitation, occipital photic-induced seizures--frequently consisting of visual symptoms--and good prognosis. This uncommon epilepsy, which usually starts in childhood or adolescence, has rarely been observed in families in which idiopathic generalized epilepsy also affects other members. We describe a nuclear family in which the proband showed electro-clinical features of idiopathic photosensitive occipital lobe epilepsy in childhood, which subsequently evolved into absences and a single generalized tonico-clonic seizure in early adolescence. His mother had features suggestive of juvenile myoclonic epilepsy. This case illustrates a continuum between focal and generalized entities in the spectrum of the so-called idiopathic (genetically determined) epileptic syndromes. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Perampanel for focal epilepsy: insights from early clinical experience.

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Trinka, E; Steinhoff, B J; Nikanorova, M; Brodie, M J

2016-03-01

Perampanel is approved for adjunctive therapy of focal epilepsy with or without secondarily generalized seizures in patients aged >12 years. This narrative review uses real-world and clinical trial data to elucidate perampanel's role in the clinic. Audit data show good tolerability with perampanel and higher freedom-from-seizure rates in elderly vs younger patients. When using perampanel in elderly patients, special attention should be given to comorbidities and co-medication to avoid potential interactions or adverse events. Slower titration is generally recommended, and seizure control should be reassessed at a dose of 4 mg before further dose increases. Perampanel efficacy is similar in adolescents and adults; however, somnolence, nasopharyngitis, and aggression are more frequent in adolescents vs the overall population. Individualized and slow-dose titration can minimize adverse events. Low serum concentrations of perampanel may occur in patients also receiving some enzyme-inducing anti-epileptic drugs; a perampanel dose increase may be required. Adverse events of importance with perampanel include dizziness; anger, aggression, and hostile behavior (particularly in adolescents); and falls (particularly in patients >65 years). An individualized approach to dosing, including slower up-titration and bedtime dosing, reduces dizziness risk. Other drugs may cause or aggravate dizziness; reducing concomitant drugs may be necessary when up-titrating perampanel. It would seem clinically appropriate to give due consideration to avoiding use in patients with a history of anger or hostile/aggressive behavior. The possibility of such behaviors should be discussed with patients before starting perampanel, with monitoring during up-titration. Slower up-titration of perampanel in older patients helps reduce fall risk. © 2015 The Authors. Acta Neurologica Scandinavica Published by John Wiley & Sons Ltd.

Incidence and Classification of New-Onset Epilepsy and Epilepsy Syndromes in Children in Olmsted County, Minnesota from 1980–2004: A population-based study

Science.gov (United States)

Wirrell, Elaine C.; Grossardt, Brandon R.; Wong-Kisiel, Lily C.-L.; Nickels, Katherine C.

2012-01-01

Purpose To determine the incidence and classification of new-onset epilepsy, as well as the distribution of epilepsy syndromes in a population-based group of children, using the newly proposed Report of the ILAE Commission on Classification and Terminology 2005–2009. Methods We identified all children residing in Olmsted County, MN, 1 month through 17 years with newly diagnosed epilepsy from 1980–2004. For each patient, epilepsy was classified into mode of onset, etiology, and syndrome or constellation (if present). Incidence rates were calculated overall and also separately for categories of mode of onset and etiology. Results The adjusted incidence rate of new-onset epilepsy in children was 44.5 cases per 100,000 persons per year. Incidence rates were highest in the first year of life and diminished with age. Mode of onset was focal in 68%, generalized/bilateral in 23%, spasms in 3% and unknown in 5%. Approximately half of children had an unknown etiology for their epilepsy, and of the remainder, 78 (22%) were genetic and 101 (28%) were structural/metabolic. A specific epilepsy syndrome could be defined at initial diagnosis in 99/359 (28%) children, but only 9/359 (3%) had a defined constellation. Conclusion Nearly half of childhood epilepsy is of “unknown” etiology. While a small proportion of this group met criteria for a known epilepsy syndrome, 41% of all childhood epilepsy is of “unknown” cause with no clear syndrome identified. Further work is needed to define more specific etiologies for this group. PMID:21482075

123I-iomazenil brain receptor SPECT in focal epilepsy. In comparison with 99mTc-HMPAO brain SPECT, MRI and Video/EEG monitoring

International Nuclear Information System (INIS)

Xu Hao; Wang Tongge; Huang Li; Michael Cordes

1998-01-01

Purpose: To evaluate the clinical value of 123 I-Iomazenil brain receptor SPECT in diagnosis of focal epilepsy in comparison with 99m Tc-HMPAO brain SPECT, MRI and Video/EEG monitoring. Methods 123 I-Iomazenil brain receptor SPECT was performed on 40 patients with focal epilepsy. The results were compared with those obtained by 99m Tc-HMPAO brain SPECT, MRI and Video/EEG monitoring. Results: In 40 patients, the sensitivity of Video/EEG monitoring for localization of epileptogenic area was 95% (38/40). The sensitivity of 123 I-iomazenil brain receptor SPECT, 99m Tc-HMPAO brain SPECT and MRI for localization of epileptogenic area compared with Video/EEG monitoring ('gold standard') was 65.8%(25/38), 55.3%(21/38) and 47.4%(18/38), respectively. The localization of epileptogenic area with 123 I-Iomazenil brain receptor SPECT was in concordance with Video/EEG monitoring in 20 patients, 99m Tc-HMPAO brain SPECT in 15 patients and MRI in 16 patients, respectively. The sensitivity of 123 I-Iomazenil brain receptor SPECT combined with MRI for localization of epileptogenic area was 84.2%(32/38). Conclusions: 123 I-Iomazenil brain receptor SPECT is a useful method in detecting and localizing epileptogenic area. The combination of 123 I-Iomazenil brain receptor SPECT and MRI has a high sensitivity for detecting epileptogenic area

Genetic models of absence epilepsy: New concepts and insights

NARCIS (Netherlands)

Luijtelaar, E.L.J.M. van; Stein, J.

2017-01-01

The discovery, development and use of genetic rodent models of absence epilepsy have led to a new theory about the origin of absence seizures, which has gained impact within the international epilepsy community. A focal zone has been identified in the perioral region of the somatosensory cortex in

Do people with epilepsy have a different lifestyle?

Science.gov (United States)

Aguirre, Clara; Quintas, Sonia; Ruiz-Tornero, Ana María; Alemán, Guadalupe; Gago-Veiga, Ana Betariz; de Toledo, María; Vivancos, Jose

2017-09-01

Epilepsy is one of the most common neurological diseases. Its high prevalence, economic relevance and impact on daily life make it crucial that we study this condition in further detail. Our study seeks to investigate whether the lifestyle of people diagnosed with epilepsy is different to that of people without epilepsy, in order to better understand our patients. We designed and delivered a questionnaire about quality of life and daily habits to patients from our hospital's Epilepsy Unit. We also delivered the questionnaire to a control group with similar demographic characteristics. Lifestyle differences between patients and control group members were analyzed. Patients were further divided according to the type of epilepsy, time since diagnosis, seizure frequency and pharmacotherapy. A total of 278 people were interviewed (85 patients, 193 controls). There was no difference in educational level, marital status and healthy habits (sports, reading and diet) between the groups. However, patients with epilepsy were more often unemployed (pepilepsy. In terms of the type of epilepsy, patients with focal epilepsy exercised more than those with generalized epilepsy; no other statistically significant differences were found between the individuals studied. Epilepsy diagnosis does not seem to negatively alter the daily life of patients; in fact, many adopt a healthier lifestyle after diagnosis. The risk of antidepressant/anxiolytic intake is, however, higher, which could reflect the impact this chronic condition still has at a social level. Copyright © 2017 Elsevier Inc. All rights reserved.

Unfavorable surgical outcomes in partial epilepsy with secondary bilateral synchrony: Intracranial electroencephalography study.

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Sunwoo, Jun-Sang; Byun, Jung-Ick; Moon, Jangsup; Lim, Jung-Ah; Kim, Tae-Joon; Lee, Soon-Tae; Jung, Keun-Hwa; Park, Kyung-Il; Chu, Kon; Kim, Manho; Chung, Chun-Kee; Jung, Ki-Young; Lee, Sang Kun

2016-05-01

Secondary bilateral synchrony (SBS) indicates bilaterally synchronous epileptiform discharges arising from a focal cortical origin. The present study aims to investigate SBS in partial epilepsy with regard to surgical outcomes and intracranial EEG findings. We retrospectively reviewed consecutive patients who underwent epilepsy surgery following extraoperative intracranial electroencephalography (EEG) study from 2008 to 2012. The presence of SBS was determined based upon the results of scalp EEG monitoring performed for presurgical evaluations. We reviewed scalp EEG, neuroimaging, intracranial EEG findings, and surgical outcomes in patients with SBS. We found 12 patients with SBS who were surgically treated for intractable partial epilepsy. Nine (75%) patients had lateralized ictal semiology and only two (16.6%) patients showed localized ictal onset in scalp EEG. Brain MRI showed epileptogenic lesion in three (25%) patients. Intracranial EEG demonstrated that ictal onset zone was widespread or non-localized in six (50%) patients. Low-voltage fast activity was the most common ictal onset EEG pattern. Rapid propagation of ictal onset was noted in 10 (83.3%) patients. Eleven patients underwent resective epilepsy surgery and only two patients (18.2%) achieved seizure-freedom (median follow-up 56 months). MRI-visible brain lesions were associated with favorable outcomes (p=0.024). Patients with SBS, compared to frontal lobe epilepsy without SBS, showed lesser localization in ictal onset EEG (p=0.029) and more rapid propagation during evolution of ictal rhythm (p=0.015). The present results suggested that resective surgery for partial epilepsy with SBS should be decided carefully, especially in case of nonlesional epilepsy. Poor localization and rapid spread of ictal onset were prominent in intracranial EEG, which might contribute to incomplete resection of the epileptogenic zone and poor surgical outcomes. Copyright © 2016 Elsevier B.V. All rights reserved.

Quality of Life and Fitness in Children and Adolescents with Epilepsy (EpiFit).

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Rauchenzauner, Markus; Hagn, Claudia; Walch, Romana; Baumann, Matthias; Haberlandt, Edda; Frühwirth, Martin; Rostasy, Kevin

2017-06-01

Objective  The objective of this study was to evaluate the correlation between fitness and health-related quality of life (HRQoL) in children with idiopathic epilepsy compared with a healthy matched control group. Methods  In this study, 107 children conducted a 6-minute walk test, anthropometric parameters were measured, and HRQoL was assessed using a standardized questionnaire (KINDL-R). Children were divided into two groups: (1) the patient group ( n  = 48) and (2) the healthy control group ( n  = 59). Results  HRQoL of children with focal epilepsy was greater when compared with healthy children and children with generalized epilepsy. A significant association could be demonstrated for the 6-minute walk distance and mental wellbeing in children with epilepsy but not in healthy children. Furthermore, a negative correlation between the HRQoL and the amount of time spent in front of TV and computer in children with epilepsy and healthy children was seen. In children with focal epilepsy, a significant negative correlation could be shown between school sport and mental wellbeing as well as between school sport and self-esteem. Conclusion  HRQoL in children with idiopathic epilepsy is significantly associated with physical fitness and might be positively influenced by an adequate education of patients and parents, a reduction of consumption of computer and TV in combination with age- and disease-adapted physical activity and sports. Georg Thieme Verlag KG Stuttgart · New York.

Autosomal dominant cortical tremor, myoclonus and epilepsy.

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Striano, Pasquale; Zara, Federico

2016-09-01

The term 'cortical tremor' was first introduced by Ikeda and colleagues to indicate a postural and action-induced shivering movement of the hands which mimics essential tremor, but presents with the electrophysiological findings of cortical reflex myoclonus. The association between autosomal dominant cortical tremor, myoclonus and epilepsy (ADCME) was first recognized in Japanese families and is now increasingly reported worldwide, although it is described using different acronyms (BAFME, FAME, FEME, FCTE and others). The disease usually takes a benign course, although drug-resistant focal seizures or slight intellectual disability occur in some cases. Moreover, a worsening of cortical tremor and myoclonus is common in advanced age. Although not yet recognized by the International League Against Epilepsy (ILAE), this is a well-delineated epilepsy syndrome with remarkable features that clearly distinguishes it from other myoclonus epilepsies. Moreover, genetic studies of these families show heterogeneity and different susceptible chromosomal loci have been identified.

Epilepsy with onset at over 50 years of age: clinical and electroencephalographic characteristics

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Glória Maria Almeida Souza Tedrus

2012-10-01

Full Text Available Epilepsy in older individuals has an elevated incidence. The objective of the present work was to evaluate the clinical, EEG and brain imaging aspects in patients showing late-onset epilepsy. Fifty-five patients with late-onset epilepsy (older than 50 years were evaluated. They were composed of two groups according to the onset age of the epilepsy seizure (ES: 51-60 (G51-60 and over 60 (G60+ years. Focal ES predominated although they were less frequent in G60+. The occurrence of status epilepticus was high and more frequent in G60+ whereas seizures in series predominated in G51-60. Symptomatic epilepsy was more frequent and the vascular etiology predominated. Epileptiform activity was associated with a greater number of ES, and background activity abnormalities were more frequent in G60+. In conclusion, epilepsy with onset at over 50 was predominantly focal and symptomatic, with a high occurrence of status epilepticus and of seizures in series.

Quality of life after surgery for intractable partial epilepsy in children: a cohort study with controls.

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Mikati, Mohamad A; Ataya, Nour; Ferzli, Jessica; Kurdi, Rana; El-Banna, Diana; Rahi, Amal; Shamseddine, Alhan; Sinno, Durriyah; Comair, Youssef

2010-08-01

Investigate if quality of life (QOL) normalizes on long-term follow-up after surgery for partial epilepsy in children. This is a cohort study with controls in which a consecutive cohort of nineteen 2-14-year-old children who underwent focal resections for intractable partial seizures between 1996 and 2006, were matched with 19 non-surgery intractable partial epilepsy patients, and with 19 healthy subjects. The two epilepsy groups were matched for age, sex, socio-economic status (SES), cognitive level, seizure type, and seizure frequency. The healthy group was matched with the two epilepsy groups for age, sex, SES, and cognitive level. QOL was assessed using the QOLCE (Quality of Life in Childhood Epilepsy Questionnaire). In the surgery group (follow-up 3.84+/-2.26 years), 78.9% had Engel class-I versus 21.1% in non-surgery (p=0.01) (follow-up 3.44+/-2.95 years). Surgery patients were similar to healthy subjects in the social, emotional, cognitive, behavioral, and overall QOL (p>0.05) but had lower scores in the total QOL, physical, and health domains (p0.05, power>0.8). Our data indicate that epilepsy surgery for partial seizures in children is associated with better QOL as compared to children with intractable epilepsy who are not operated on, and suggest that in those who achieve seizure freedom normal QOL may at least potentially be possible.

[Tropical causes of epilepsy].

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Carod-Artal, F J

Eighty-five percent of all epileptics live in tropical regions. Prenatal risk factors, traumatic brain injuries and different parasitic infestations of the central nervous system (CNS) are the reasons behind the high prevalence of epilepsy. This work reviews the main parasitic infestations causing epilepsy in the tropics. Neurocysticercosis is the main cause of focal epilepsy in early adulthood in endemic areas (30-50%). All the phases of cysticerci (viable, transitional and calcified) are associated with epileptic seizures. Anti-cysticercus treatment helps get rid of cysticerci faster and reduces the risk of recurrence of seizures in patients with viable cysts. Symptomatic epilepsy can be the first manifestation of neuroschistosomiasis in patients without any systemic symptoms. The pseudotumoral form can trigger seizures secondary to the presence of granulomas and oedemas in the cerebral cortex. The eggs of Schistosoma japonicum are smaller, reach the CNS more easily and trigger epileptic seizures more frequently. Toxocariasis and sparganosis are other parasitic infestations that can give rise to symptomatic seizures. The risk factors for suffering chronic epilepsy after cerebral malaria are a positive familial history of epilepsy and a history of episodes of fever and cerebral malaria that began with coma or which progressed with multiple, prolonged epileptic seizures. About 20% of patients with cerebral infarction secondary to Chagas disease present late vascular epilepsy as a complication. Very few studies have been conducted to examine the prognosis, risk of recurrence and modification of the natural course of seizures associated with tropical parasitic infestations, except for the case of neurocysticercosis.

The clinical value of computerized axial tomography in patients without focal neurological features

International Nuclear Information System (INIS)

Lundorf, E.; Nielson, M.B.

1985-01-01

74 randomly selected patients with non-focal cerebral symptoms and a normal neurologic examination were referred from neurologic departments to CT scan of the brain. 29 patients had generalised epilepsy of long duration. In 26 patients (90%) with epilepsy the Ct scan was normal. 2 patients (7%) had cerebral atrophy, 1 (3%) showed porencephaly (.) 41 (91%) of the patients without epileptic features had a normal CT scan. 4 (9%) presented cerebral atrophy. In this survey, Ct scanning did not contribute to a focal diagnosis in patients with diffuse cerebral features. (orig.) [de

16p11.2 600 kb Duplications confer risk for typical and atypical Rolandic epilepsy

DEFF Research Database (Denmark)

Reinthaler, Eva M; Lal, Dennis; Lebon, Sebastien

2014-01-01

Rolandic epilepsy (RE) is the most common idiopathic focal childhood epilepsy. Its molecular basis is largely unknown and a complex genetic etiology is assumed in the majority of affected individuals. The present study tested whether six large recurrent copy number variants at 1q21, 15q11.2, 15q1...

Long-term applicability of the new ILAE definition of epilepsy. Results from the PRO-LONG study.

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Beretta, Simone; Carone, Davide; Zanchi, Clara; Bianchi, Elisa; Pirovano, Marta; Trentini, Claudia; Padovano, Giada; Colombo, Matteo; Cereda, Diletta; Scanziani, Sofia; Giussani, Giorgia; Gasparini, Sara; Bogliun, Graziella; Ferrarese, Carlo; Beghi, Ettore

2017-09-01

The new epilepsy definition adopted by the International League Against Epilepsy (ILAE) includes patients with one unprovoked seizure with a probability of further seizures, similar to the general recurrence risk after two unprovoked seizures, occurring in a 10-year period. Long-term follow-up of patients diagnosed after a single seizure is needed to assess the applicability of the new epilepsy definition in clinical practice. Patients with newly diagnosed epilepsy were recruited retrospectively with a minimum follow-up of 10 years. Patients were stratified in two groups depending on the occurrence of one (new definition, ND) or two or more unprovoked seizures (traditional definition, TD) at the time of epilepsy diagnosis and compared for disease characteristics and factors predicting seizure recurrence. The primary outcome was the occurrence of a new unprovoked seizure during follow-up in the ND group. The secondary outcome was the achievement of an early remission in both groups. Among 1,006 patients with newly diagnosed epilepsy, 152 (15.1%) were diagnosed after a single seizure. Compared to patients diagnosed using the TD, patients diagnosed according to the ND showed a higher proportion of subjects with an abnormal neurologic examination (19.9% vs. 13.7%, p = 0.0504) and with focal seizures (69.3% vs. 60.4%, p = 0.0021). The two samples differed in the presence of at least one of the factors predicting seizure recurrence (focal seizures or abnormal findings in at least one among the following: neurologic examination, electroencephalography [EEG], and neuroimaging) (94.6% vs. 89.1%, p = 0.0376). Long-term recurrence in patients diagnosed with the new definition was 83.6% at 10 years and 89.1% at 15 years. The probability of early remission did not differ between the two groups. Our results support the applicability of the new epilepsy definition in clinical practice. Individual patient characteristics and a personalized diagnostic approach can justify

Prevalence and risk factors of seizure clusters in adult patients with epilepsy.

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Chen, Baibing; Choi, Hyunmi; Hirsch, Lawrence J; Katz, Austen; Legge, Alexander; Wong, Rebecca A; Jiang, Alfred; Kato, Kenneth; Buchsbaum, Richard; Detyniecki, Kamil

2017-07-01

In the current study, we explored the prevalence of physician-confirmed seizure clusters. We also investigated potential clinical factors associated with the occurrence of seizure clusters overall and by epilepsy type. We reviewed medical records of 4116 adult (≥16years old) outpatients with epilepsy at our centers for documentation of seizure clusters. Variables including patient demographics, epilepsy details, medical and psychiatric history, AED history, and epilepsy risk factors were then tested against history of seizure clusters. Patients were then divided into focal epilepsy, idiopathic generalized epilepsy (IGE), or symptomatic generalized epilepsy (SGE), and the same analysis was run. Overall, seizure clusters were independently associated with earlier age of seizure onset, symptomatic generalized epilepsy (SGE), central nervous system (CNS) infection, cortical dysplasia, status epilepticus, absence of 1-year seizure freedom, and having failed 2 or more AEDs (Pepilepsy (16.3%) and IGE (7.4%; all Pepilepsy type showed that absence of 1-year seizure freedom since starting treatment at one of our centers was associated with seizure clustering in patients across all 3 epilepsy types. In patients with SGE, clusters were associated with perinatal/congenital brain injury. In patients with focal epilepsy, clusters were associated with younger age of seizure onset, complex partial seizures, cortical dysplasia, status epilepticus, CNS infection, and having failed 2 or more AEDs. In patients with IGE, clusters were associated with presence of an aura. Only 43.5% of patients with seizure clusters were prescribed rescue medications. Patients with intractable epilepsy are at a higher risk of developing seizure clusters. Factors such as having SGE, CNS infection, cortical dysplasia, status epilepticus or an early seizure onset, can also independently increase one's chance of having seizure clusters. Copyright © 2017. Published by Elsevier B.V.

Modelling large motion events in fMRI studies of patients with epilepsy

DEFF Research Database (Denmark)

Lemieux, Louis; Salek-Haddadi, Afraim; Lund, Torben E

2007-01-01

-positive activation. Head motion can lead to severe image degradation and result in false-positive activation and is usually worse in patients than in healthy subjects. We performed general linear model fMRI data analysis on simultaneous EEG-fMRI data acquired in 34 cases with focal epilepsy. Signal changes...... associated with large inter-scan motion events (head jerks) were modelled using modified design matrices that include 'scan nulling' regressors. We evaluated the efficacy of this approach by mapping the proportion of the brain for which F-tests across the additional regressors were significant. In 95......% of cases, there was a significant effect of motion in 50% of the brain or greater; for the scan nulling effect, the proportion was 36%; this effect was predominantly in the neocortex. We conclude that careful consideration of the motion-related effects in fMRI studies of patients with epilepsy is essential...

Interictal epileptiform discharges have an independent association with cognitive impairment in children with lesional epilepsy.

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Glennon, Jennifer M; Weiss-Croft, Louise; Harrison, Sue; Cross, J Helen; Boyd, Stewart G; Baldeweg, Torsten

2016-09-01

The relative contribution of interictal epileptiform discharges (IEDs) to cognitive dysfunction in comparison with the underlying brain pathology is not yet understood in children with lesional focal epilepsy. The current study investigated the association of IEDs with intellectual functioning in 103 children with medication-resistant focal epilepsy. Hierarchical multiple regression analyses were used to determine the independent contribution of IED features on intellectual functioning, after controlling for effects of lesional pathology, epilepsy duration, and medication. Exploratory analyses were conducted for language and memory scores as well as academic skills available in a subset of participants. The results reveal that IEDs have a negative association with IQ with independent, additive effects documented for frequent and bilaterally distributed IEDs as well as discharge enhancement in sleep. Left-lateralized IEDs had a prominent effect on verbal intelligence, in excess of the influence of left-sided brain pathology. These effects extended to other cognitive functions, most prominently for sleep-enhanced IEDs to be associated with deficits in expressive and receptive language, reading, spelling and numerical skills. Overall, IED effects on cognition were of a magnitude similar to lesional influences or drug effects (topiramate use). This study demonstrates an association between IEDs and cognitive dysfunction, independent of the underlying focal brain pathology. Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.

Epilepsy surgery in children and adolescents with malformations of cortical development--outcome and impact of the new ILAE classification on focal cortical dysplasia.

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Mühlebner, Angelika; Gröppel, Gudrun; Dressler, Anastasia; Reiter-Fink, Edith; Kasprian, Gregor; Prayer, Daniela; Dorfer, Christian; Czech, Thomas; Hainfellner, Johannes A; Coras, Roland; Blümcke, Ingmar; Feucht, Martha

2014-11-01

To determine long-term efficacy and safety of epilepsy surgery in children and adolescents with malformations of cortical development (MCD) and to identify differences in seizure outcome of the various MCD subgroups. Special focus was set on the newly introduced International League Against Epilepsy (ILAE) classification of focal cortical dysplasia (FCD). This is a single center retrospective cross-sectional analysis of prospectively collected data. age at surgery classification schemes (Barkovich et al., 2012. Brain. 135, 1348-1369; Palmini et al., 2004. Neurology. 62, S2-S8) and the ILAE classification for FCD recently proposed by Blümcke in 2011. Seizure outcome was classified using the ILAE classification proposed by Wieser in 2001. 60 Patients (51.7% male) were included. Follow up was up to 14 (mean 4.4 ± 3.2) years. Mean age at surgery was 8.0 ± 6.0 (median 6.0) years; mean age at epilepsy onset was 2.9 ± 3.2 (median 2.0) years; duration of epilepsy before surgery was 4.8 ± 4.4 (median 3.0) years. 80% of the patients were seizure free at last follow-up. AEDs were successfully withdrawn in 56.7% of all patients. Extended surgery, lesion localization in the temporal lobes and absence of inter-ictal spikes in postsurgical EEG recordings were predictive of favorable seizure outcomes after surgery. However, no association was found between outcome and MCD sub-types. Epilepsy surgery is highly effective in carefully selected drug-resistant children with MCD. Surrogate markers for complete resection of the epileptogenic zone remain the only significant predictors for seizure freedom after surgery. Copyright © 2014 Elsevier B.V. All rights reserved.

Guidelines for imaging infants and children with recent-onset epilepsy

International Nuclear Information System (INIS)

Gaillard, W.D.; Chiron, C.; Cross, H.; Harvey, S.; Kuzniecky, R.; Hertz-Pannier, L.

2009-01-01

The International League Against Epilepsy (ILAE) Subcommittee for Pediatric Neuroimaging examined the usefulness of, and indications for, neuroimaging in the evaluation of children with newly diagnosed epilepsy. The retrospective and prospective published series with n ≥ 30 utilizing computed tomography (CT) and magnetic resonance imaging (MRI) (1.5 T) that evaluated children with new-onset seizure(s) were reviewed. Nearly 50% of individual imaging studies in children with localization-related new-onset seizure(s) were reported to be abnormal; 15-20% of imaging studies provided useful information on etiology or and seizure focus, and 2-4% provided information that potentially altered immediate medical management. A significant imaging abnormality in the absence of a history of a localization-related seizure, abnormal neurologic examination, or focal electro-encephalography (EEG) is rare. Imaging studies in childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and benign childhood epilepsy with centro-temporal spikes (BECTS) do not identify significant structural abnormalities. Imaging provides important contributions to establishing etiology, providing prognostic information, and directing treatment in children with recently diagnosed epilepsy. Imaging is recommended when localization-related epilepsy is known or suspected, when the epilepsy classification is in doubt, or when an epilepsy syndrome with remote symptomatic cause is suspected. When available, MRI is preferred to CT because of its superior resolution, versatility, and lack of radiation. (authors)

Guidelines for imaging infants and children with recent-onset epilepsy

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Gaillard, W.D. [Department of Neuroscience, Children' s National Medical Center, George Washington University, Washington DC (United States); Chiron, C. [Inserm, Hopital Necker-Enfants Malades, Universite Rene Descartes, Paris (France); Cross, H. [Neurosciences Unit, Institute of Child Health, and GreatOrmondStreet Hospital for Children, London (United Kingdom); Harvey, S. [Department of Neurology, Royal Children' s Hospital, University of Melbourne, Melbourne (Australia); Kuzniecky, R. [Department of Neurology, New York University School of Medicine, New York, NY (US); Hertz-Pannier, L. [Department of Radiology, Hopital Necker-Enfants Malades, Universite Descartes, Paris (FR); CEA-DSV-I2BM-Neurospin, 91191 Gif sur Yvette (FR)

2009-07-01

The International League Against Epilepsy (ILAE) Subcommittee for Pediatric Neuroimaging examined the usefulness of, and indications for, neuroimaging in the evaluation of children with newly diagnosed epilepsy. The retrospective and prospective published series with n {>=} 30 utilizing computed tomography (CT) and magnetic resonance imaging (MRI) (1.5 T) that evaluated children with new-onset seizure(s) were reviewed. Nearly 50% of individual imaging studies in children with localization-related new-onset seizure(s) were reported to be abnormal; 15-20% of imaging studies provided useful information on etiology or and seizure focus, and 2-4% provided information that potentially altered immediate medical management. A significant imaging abnormality in the absence of a history of a localization-related seizure, abnormal neurologic examination, or focal electro-encephalography (EEG) is rare. Imaging studies in childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and benign childhood epilepsy with centro-temporal spikes (BECTS) do not identify significant structural abnormalities. Imaging provides important contributions to establishing etiology, providing prognostic information, and directing treatment in children with recently diagnosed epilepsy. Imaging is recommended when localization-related epilepsy is known or suspected, when the epilepsy classification is in doubt, or when an epilepsy syndrome with remote symptomatic cause is suspected. When available, MRI is preferred to CT because of its superior resolution, versatility, and lack of radiation. (authors)

Isolated amygdala enlargement in temporal lobe epilepsy: A systematic review.

Science.gov (United States)

Beh, S M Jessica; Cook, Mark J; D'Souza, Wendyl J

2016-07-01

The objective of this study was to compare the seizure characteristics and treatment outcomes in patient groups with temporal lobe epilepsy (TLE) identified with isolated amygdala enlargement (AE) on magnetic resonance imaging studies. PubMed, Embase, and the Cochrane Library were searched for relevant studies using the keywords 'amygdala enlargement', 'epilepsy', and 'seizures' in April 2015. Human studies, written in English, that investigated cohorts of patients with TLE and AE were included. Of 204 abstracts initially identified using the search strategy, 14 studies met the inclusion criteria (11 epilepsy studies and 3 psychiatry studies). Ultimately, 8 full studies on AE and TLE involving 107 unique patients were analyzed. Gender distribution consisted of 50 males and 57 females. Right amygdala enlargement was seen in 39 patients, left enlargement in 58 patients, and bilateral enlargement in 7 patients. Surgical resection was performed in 28 patients, with the most common finding being dysplasia/hamartoma or focal cortical dysplasia. Most studies involved small samples of less than 12 patients. There was a wide discrepancy in the methods used to measure amygdala volume, in both patients and controls, hindering comparisons. Most TLE with AE studies observed a later age of seizure onset (mean: 32.2years) compared with studies involving TLE with HS (mean of mid- to late childhood). A higher frequency of complex partial seizures compared with that of convulsive seizures is seen in patients with AE (67-100% vs. 26-47%), and they have an excellent response to antiepileptic drugs (81.8%-100% of seizure-free patients). All studies that included controls also found a significant difference in frequency of seizure types between their cases and controls. Reliable assessment of amygdala volume remains a critical issue hindering better understanding of the clinical management and research of this focal epilepsy syndrome. Within these limitations, the literature suggests

International Veterinary Epilepsy Task Force recommendations for a veterinary epilepsy-specific MRI protocol.

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Rusbridge, Clare; Long, Sam; Jovanovik, Jelena; Milne, Marjorie; Berendt, Mette; Bhatti, Sofie F M; De Risio, Luisa; Farqhuar, Robyn G; Fischer, Andrea; Matiasek, Kaspar; Muñana, Karen; Patterson, Edward E; Pakozdy, Akos; Penderis, Jacques; Platt, Simon; Podell, Michael; Potschka, Heidrun; Stein, Veronika M; Tipold, Andrea; Volk, Holger A

2015-08-28

Epilepsy is one of the most common chronic neurological diseases in veterinary practice. Magnetic resonance imaging (MRI) is regarded as an important diagnostic test to reach the diagnosis of idiopathic epilepsy. However, given that the diagnosis requires the exclusion of other differentials for seizures, the parameters for MRI examination should allow the detection of subtle lesions which may not be obvious with existing techniques. In addition, there are several differentials for idiopathic epilepsy in humans, for example some focal cortical dysplasias, which may only apparent with special sequences, imaging planes and/or particular techniques used in performing the MRI scan. As a result, there is a need to standardize MRI examination in veterinary patients with techniques that reliably diagnose subtle lesions, identify post-seizure changes, and which will allow for future identification of underlying causes of seizures not yet apparent in the veterinary literature.There is a need for a standardized veterinary epilepsy-specific MRI protocol which will facilitate more detailed examination of areas susceptible to generating and perpetuating seizures, is cost efficient, simple to perform and can be adapted for both low and high field scanners. Standardisation of imaging will improve clinical communication and uniformity of case definition between research studies. A 6-7 sequence epilepsy-specific MRI protocol for veterinary patients is proposed and further advanced MR and functional imaging is reviewed.

Focal epilepsy presenting as a bath-induced paroxysmal event/breath-holding attack

Directory of Open Access Journals (Sweden)

C.J. Stutchfield

2014-01-01

Conclusion: Water reflex epilepsy can mimic a range of other conditions, and a high index of suspicion is required to establish the diagnosis. Children with water reflex epilepsy can achieve a good quality of life with modified bathing and appropriate antiepileptic medication.

Effects of focal brain cooling on extracellular concentrations of neurotransmitters in patients with epilepsy.

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Nomura, Sadahiro; Inoue, Takao; Imoto, Hirochika; Suehiro, Eiichi; Maruta, Yuichi; Hirayama, Yuya; Suzuki, Michiyasu

2017-04-01

Brain hypothermia controls epileptic discharge and reduces extracellular concentrations of glutamate (Glu), an excitatory neurotransmitter. We aimed to determine the effects of focal brain cooling (FBC) on levels of γ-aminobutyric acid (GABA), which is a major inhibitory neurotransmitter. The relationship between Glu or GABA concentrations and the severity of epileptic symptoms was also analyzed. Patients with intractable epilepsy underwent FBC at lesionectomized (n = 11) or hippocampectomized (n = 8) regions at 15°C for 30 min using custom-made cooling devices. Concentrations of Glu (n = 18) and GABA (n = 12) were measured in extracellular fluid obtained through microdialysis using high-performance liquid chromatography (HPLC). The reduction rate of neurotransmitter levels and its relationship with electrocorticography (ECoG) signal changes in response to FBC were measured. We found no relationship between the concentrations of Glu or GABA and seizure severity. There was a significant decrease in the concentration of Glu to 66.3% of control levels during the cooling period (p = 0.001). This rate of reduction correlated with ECoG power (r 2 = 0.68). Cortical and hippocampal GABA levels significantly (p = 0.02) and nonsignificantly decreased to 47.7% and 32.4% of control levels, respectively. However, the rate of this reduction did not correlate with ECoG (r 2 = 0.11). Although the decrease in hippocampal GABA levels was not significant due to wide variations in its concentration, the levels of cortical GABA and Glu were decreased following FBC. FBC suppresses epileptic discharge and the release of both excitatory and inhibitory neurotransmitters. The reduction in Glu levels further contributes to the reduction in epileptic discharge. However, the reduction in the levels of GABA has no impact on ECoG. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

Epilepsy and radiological investigations

International Nuclear Information System (INIS)

Tomberg, T.

2005-01-01

Epilepsy is a heterogenous group of disorders with multiple causes. Clinical management of epilepsy patients requires knowledge of seizure syndromes, causes, and imaging features. The aim of radiological investigations is to recognize the underlying cause of epilepsy. The main indications for neuroimaging studies are partial and secondarily generalized seizures, patients with neurological signs and intractable seizures, and patients with focal signs on EEG. Partial seizures of any type are more likely to be associated with a focus that may be identified on neuroimaging. MRI is the method of choice for evaluating structural abnormalities of the brain. High resolution MRI and dedicated imaging technique are needed for detection of subtle pathological changes as cortical dysplasias and temporal medial sclerosis. Other lesions that may be detected include neoplasms, vascular malformations, destructive lesions following brain injury, stroke, infection, etc. CT continues to be the technique for the investigation of patients with seizures under certain conditions. New techniques such as functional MRI, MR spectroscopy, SPECT, receptor PET and magnetic source imaging are becoming clinical tools for improving diagnosis [et

Behavioral problems in children with epilepsy in rural Kenya

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Kariuki, Symon M.; Abubakar, Amina; Holding, Penny A.; Mung'ala-Odera, Victor; Chengo, Eddie; Kihara, Michael; Neville, Brian G.; Newton, Charles R.J.C.

2012-01-01

The aims of this study were to record behavioral problems in children with epilepsy (CWE), compare the prevalence with that reported among healthy children without epilepsy, and investigate the risk factors. A child behavioral questionnaire for parents comprising 15 items was administered to the main caregiver of 108 CWE and 108 controls matched for age in Kilifi, Kenya. CWE had a higher mean score for reported behavioral problems than controls (6.9 vs 4.9, t = 4.7, P epilepsy also recorded more behavioral problems than those with inactive epilepsy (8.2 vs 6.2, t = − 2.9, P = 0.005). A significantly greater proportion of CWE (49% vs 26% of controls) were reported to have behavioral problems. Active epilepsy, cognitive impairment, and focal seizures were the most significant independent covariates of behavioral problems. Behavioral problems in African CWE are common and need to be taken into consideration in planning comprehensive clinical services in this region. PMID:22119107

Usefulness of a simple sleep-deprived EEG protocol for epilepsy diagnosis in de novo subjects.

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Giorgi, Filippo S; Perini, Daria; Maestri, Michelangelo; Guida, Melania; Pizzanelli, Chiara; Caserta, Anna; Iudice, Alfonso; Bonanni, Enrica

2013-11-01

In case series concerning the role of EEG after sleep deprivation (SD-EEG) in epilepsy, patients' features and protocols vary dramatically from one report to another. In this study, we assessed the usefulness of a simple SD-EEG method in well characterized patients. Among the 963 adult subjects submitted to SD-EEG at our Center, in the period 2003-2010, we retrospectively selected for analysis only those: (1) evaluated for suspected epileptic seizures; (2) with a normal/non-specific baseline EEG; (3) still drug-free at the time of SD-EEG; (4) with an MRI analysis; (5) with at least 1 year follow-up. SD-EEG consisted in SD from 2:00 AM and laboratory EEG from 8:00 AM to 10:30 AM. We analyzed epileptic interictal abnormalities (IIAs) and their correlations with patients' features. Epilepsy was confirmed in 131 patients. SD-EEG showed IIAs in 41.2% of all patients with epilepsy, and a 91.1% specificity for epilepsy diagnosis; IIAs types observed during SD-EEG are different in generalized versus focal epilepsies; for focal epilepsies, the IIAs yield in SD-EEG is higher than in second routine EEG. This simple SD-EEG protocol is very useful in de novo patients with suspected seizures. This study sheds new light on the role of SD-EEG in specific epilepsy populations. Copyright © 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

Small Lesion Size Is Associated with Sleep-Related Epilepsy in Focal Cortical Dysplasia Type II

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Bo Jin

2018-02-01

Full Text Available ObjectiveTo investigate the neuroimaging and clinical features associated with sleep-related epilepsy (SRE in patients with focal cortical dysplasia (FCD type II.MethodsPatients with histopathologically proven FCD type II were included from three epilepsy centers. SRE was defined according to the video EEG findings and seizure history. Cortical surface reconstruction and volume calculation were performed using FreeSurfer. The lesions were manually delineated on T1 volumetric MRI using the ITK-SNAP software. The lesion volumes were normalized by the intracranial volume of each patient. The lesions were classified as small or large by placing a threshold based on quantitative (whether the lesion was detected on MRI report and qualitative (volume criteria.ResultsA total of 77 consecutive patients were included. Of them, 36 had SRE and 41 had non-SRE. An earlier age of epilepsy onset, high seizure frequency, regional interictal EEG findings, and favorable surgical outcome were characteristic in both groups. Small lesions were defined as those having a volume <3,217 mm3. In total, 60.9% of the patients with SRE (25/41 had small FCD lesion, which was significantly higher than the non-SRE group (9/34, 26.5%, p = 0.005. Small lesion size was the only predictor significantly associated with SRE in the overall type II group by multivariate analyses (p = 0.016. Although the proportion of patients who had frontal FCD and SRE was higher than non-frontal FCD (54.5 vs. 27.3%, p = 0.043, the relationship between SRE and lesion location was not confirmed by multivariate analysis. Thalamic volume and seizure semiology were not statistically different between the SRE and non-SRE group. The significant association between lesion size and SRE was reproducible in type IIb and IIa subgroups.SignificanceSRE is common in patients with FCD type II. Small FCD type II lesions are significantly associated with SRE. Although our findings cannot be applied to

Validation of epilepsy diagnoses in the Danish National Hospital Register

DEFF Research Database (Denmark)

Christensen, Jakob; Vestergaard, Mogens; Olsen, Jørn

2007-01-01

PURPOSE: To validate the diagnosis of epilepsy in the Danish National Hospital Register. METHODS: We randomly selected 200 patients registered with epilepsy in the Danish National Hospital Register between 1977 and 2002 and validated the diagnosis according to the guidelines developed...... by the International League Against Epilepsy. RESULTS: We reviewed the medical records of 188 (94%) persons from 57 departments at 41 hospitals. The epilepsy diagnoses were confirmed in 153 patients, providing a positive predictive value for epilepsy of 81% (95% confidence interval (95% CI): 75-87%). Among the 35...... for syndrome classification was 60% (95% CI: 44-74%) for epilepsy with complex focal seizures and 35% (95% CI: 22-51%) for primary generalized epilepsy. CONCLUSION: The validity of the epilepsy diagnoses in the Danish National Hospital Register has a moderate to high positive predictive value for epilepsy...

[Predictors of epilepsy in children after ischemic stroke].

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Lvova, O A; Shalkevich, L V; Dron, A N; Lukaschuk, M Y; Orlova, E A; Gusev, V V; Suleymanova, E V; Sulimov, A V; Kudlatch, A I

To determine clinical/instrumental predictors of symptomatic epilepsy after ischemic stroke in children. One hundred and thirty-six patients, aged 0-15 years, with the diagnosis of ischemic stroke (ICD-10 I63.0-I63.9) were examined. The duration of the study was 18 months - 12 years. Patients were stratified into post-stroke (n=22) and control (n=114) groups, the latter included patients without epilepsy regardless of the presence of convulsive seizures in the acute stage of stroke. Predictors were determined based on EEG and characteristics of convulsive syndrome in the acute stage of stroke. The following prognostic criteria were found: generalized type of seizures, focal type of seizures with secondary generalization, epileptiform (peak and/or peak-wave) activity, focal character of epileptiform activity, generalized type of seizures in the combination with slow wave background activity on EEG, generalized type of seizures in the combination with slow wave activity and disorganized activity on EEG.

Investigation of GRIN2A in common epilepsy phenotypes

NARCIS (Netherlands)

Lal, Dennis; Steinbrücker, Sandra; Schubert, Julian; Sander, Thomas; Becker, Felicitas; Weber, Yvonne; Lerche, Holger; Thiele, Holger; Krause, Roland; Lehesjoki, Anna Elina; Nürnberg, Peter; Palotie, Aarno; Neubauer, Bernd A.; Muhle, Hiltrud; Stephani, Ulrich; Helbig, Ingo; Becker, Albert J.; Schoch, Susanne; Hansen, Jörg; Dorn, Thomas; Hohl, Christin; Lüscher, Nicole; von Spiczak, Sarah; Lemke, Johannes R.; Zimprich, Fritz; Feucht, Martha; Suls, Arvid; Weckhuysen, Sarah; Claes, Lieve; Deprez, Liesbet; Smets, Katrien; Dyck, Tine Van; Deconinck, Tine; De Jonghe, Peter; Møller, Rikke S.; Klitten, Laura L.; Hjalgrim, Helle; Campus, Kiel; Ostertag, Philipp; Trucks, Hol ger; Elger, Christian E.; Kleefuß-Lie, Ailing A.; Kunz, Wolfram S.; Surges, Rainer; Gaus, Verena; Janz, Dieter; Schmitz, Bettina; Klein, Karl Martin; Reif, Philipp S.; Oertel, Wolfgang H.; Hamer, Hajo M.; Rosenow, Felix; Kapser, Claudia; Schankin, Christoph J.; Koeleman, Bobby P C; de Kovel, Carolien; Lindhout, Dick; Reinthaler, Eva M.; Steinboeck, Hannelore; Neo-phytou, Birgit; Geldner, Julia; Gruber-Sedlmayr, Ursula; Haberlandt, Edda; Ronen, Gabriel M.; Altmueller, Janine; Nuernberg, Peter; Neubauer, Bernd; Sirén, Auli

2015-01-01

Recently, mutations and deletions in the GRIN2A gene have been identified to predispose to benign and severe idiopathic focal epilepsies (IFE), revealing a higher incidence of GRIN2A alterations among the more severe phenotypes. This study aimed to explore the phenotypic boundaries of GRIN2A

Indices of resective surgery effectiveness for intractable nonlesional focal epilepsy.

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Blume, Warren T; Ganapathy, Gobi R; Munoz, David; Lee, Donald H

2004-01-01

Among 70 patients with intractable focal epilepsy and no specific lesion, as determined by both MRI (magnetic resonance imaging) and histopathology, outcome after resective surgery was polarized: 26 (37%) became seizure free (SF), and 27 (39%) were not helped. Eighteen (42%) of 43 standard temporal resections rendered patients SF, somewhat more than eight (30%) of 27 other procedures. To seek reliable prognostic factors, the subsequent correlative data compared features of the 26 SF patients with those of the 27 not helped. Although ictal semiology guided the site of surgical resection, it and other aspects of seizure and neurologic history failed to predict surgical outcome. However, two aspects of preoperative scalp EEGs correlated with SF outcomes: (a) among 25 patients in whom >50% of clinical seizures arose from the later resected lobe and no other origins, 18 (72%) became SF compared with seven (28%) of 25 with other ictal profiles; (b) 13 (93%) of 14 temporal lobe patients whose interictal and ictal EEGs lacked features indicative of multifocal epileptogenesis became SF compared with five (33%) of 15 with such components. The considered need for subdural (SD) EEG reduced SF outcome from 18 (90%) of 20 patients without SD to eight (24%) of 33 with SD; this likely reflected an insufficient congruity of ictal semiology and interictal and ictal scalp EEG for localizing epileptogenesis. Within this SD group, >50% of clinical seizure origins from a later resected lobe increased SF outcome somewhat: from two (14%) of 14 without this attribute to six (40%) of 15 with it; 100% of such origins increased SF outcome from two (12%) of 16 to six (46%) of 13.

Localization, correlation, and visualization of electroencephalographic surface electrodes and brain anatomy in epilepsy studies

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Brinkmann, Benjamin H.; O'Brien, Terence J.; Robb, Richard A.; Sharbrough, Frank W.

1997-05-01

Advances in neuroimaging have enhanced the clinician's ability to localize the epileptogenic zone in focal epilepsy, but 20-50 percent of these cases still remain unlocalized. Many sophisticated modalities have been used to study epilepsy, but scalp electrode recorded electroencephalography is particularly useful due to its noninvasive nature and excellent temporal resolution. This study is aimed at specific locations of scalp electrode EEG information for correlation with anatomical structures in the brain. 3D position localizing devices commonly used in virtual reality systems are used to digitize the coordinates of scalp electrodes in a standard clinical configuration. The electrode coordinates are registered with a high- resolution MRI dataset using a robust surface matching algorithm. Volume rendering can then be used to visualize the electrodes and electrode potentials interpolated over the scalp. The accuracy of the coordinate registration is assessed quantitatively with a realistic head phantom.

Relación entre electroencefalograma y neuroimagen en niños con epilepsia focal de difícil control Relation between electroencephalogram and neuroimaging present in children with epilepsy of difficult control

Directory of Open Access Journals (Sweden)

Ileana Valdivia Álvarez

2009-09-01

Full Text Available INTRODUCCIÓN. Las epilepsias focales son las más frecuentes en los niños, y la resistencia al tratamiento farmacológico puede estar presente hasta en el 30 % de los pacientes. Se realizó este estudio con el objetivo de dirigir la atención hacia la coincidencia topográfica de los paroxismos electroencefalográficos, con lesiones estructurales demostrables por neuroimagen, para facilitar el diseño de estrategias terapéuticas futuras. MÉTODOS. Se realizó un estudio descriptivo, longitudinal prospectivo, con 44 niños con diagnóstico de epilepsia focal de difícil control, ingresados en el Servicio de Neuropediatría del Hospital Pediátrico Docente «Juan M. Márquez», entre enero de 2003 y junio de 2007. Se realizaron estudios por electroencefalograma (EEG al ingreso y videoelectroencefalograma, además de estudios de neuroimagen por tomografía axial o resonancia magnética nuclear. RESULTADOS. Los paroxismos en EEG involucraron el lóbulo frontal hasta en el 68 % de los pacientes. En el 48 % de los pacientes, los paroxismos electroencefalográficos coinciden con zonas de alteración estructural según neuroimagen, más frecuentes en el lóbulo frontal. En el 25 % no hay coincidencia topográfica y en el 27 % no se precisan alteraciones estructurales. CONCLUSIONES. En las epilepsias focales de difícil control se debe prestar especial atención a las zonas elocuentes con coincidencia entre el EEG y la neuroimagen, para evaluar de forma temprana las alternativas quirúrgicas de tratamiento.INTRODUCTION: Focal epilepsies are the more frequent conditions in children and a pharmacologic treatment resistance could be present up to 30% of patients. Aim of present paper was to direct the attention to topographic coincidence of electroencephalographic paroxysms with structural lesions by neuroimaging facilitating the future therapeutical strategies design. METHODS: A descriptive, longitudinal, prospective study was conducted in 44 children

Noninvasive treatment alternative for intractable startle epilepsy

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Sylvia Klinkenberg

2014-01-01

Full Text Available We describe a treatment alternative for intractable, startle-provoked, epileptic seizures in four children aged between 8 and 14. Three of the four children had symptomatic localization-related epilepsy. They all suffered from intractable epilepsy precipitated by sudden sounds. The fact that seizures tended to occur with high frequency – more than one seizure a day – had a clear impact on daily life. Clinical seizure pattern demonstrated asymmetric tonic posturing in all four children. Three children experienced several seizure types including focal seizure onset. All children had focal neurological signs or learning disabilities or a combination of both. Our noninvasive treatment method using psychoeducational counseling and sound generators was applied in four children, resulting in a seizure frequency reduction of ≥50% in two of them.

Electrocardiographic features of sudden unexpected death in epilepsy.

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Chyou, Janice Y; Friedman, Daniel; Cerrone, Marina; Slater, William; Guo, Yu; Taupin, Daniel; O'Rourke, Sean; Priori, Silvia G; Devinsky, Orrin

2016-07-01

Sudden unexpected death in epilepsy (SUDEP) is the most common cause of epilepsy-related mortality. We hypothesized that electrocardiography (ECG) features may distinguish SUDEP cases from living subjects with epilepsy. Using a matched case-control design, we compared ECG studies of 12 consecutive cases of SUDEP over 10 years and 22 epilepsy controls matched for age, sex, epilepsy type (focal, generalized, or unknown/mixed type), concomitant antiepileptic, and psychotropic drug classes. Conduction intervals and prevalence of abnormal ventricular conduction diagnosis (QRS ≥110 msec), abnormal ventricular conduction pattern (QRS <110 msec, morphology of incomplete right or left bundle branch block or intraventricular conduction delay), early repolarization, and features of inherited cardiac channelopathies were assessed. Abnormal ventricular conduction diagnosis and pattern distinguished SUDEP cases from matched controls. Abnormal ventricular conduction diagnosis was present in two cases and no controls. Abnormal ventricular conduction pattern was more common in cases than controls (58% vs. 18%, p = 0.04). Early repolarization was similarly prevalent in cases and controls, but the overall prevalence exceeded that of published community-based cohorts. Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.

Investigation of GRIN2A> in common epilepsy phenotypes

DEFF Research Database (Denmark)

Lal, Dennis; Steinbrücker, Sandra; Schubert, Julian

2015-01-01

Recently, mutations and deletions in the GRIN2A gene have been identified to predispose to benign and severe idiopathic focal epilepsies (IFE), revealing a higher incidence of GRIN2A alterations among the more severe phenotypes. This study aimed to explore the phenotypic boundaries of GRIN2A muta...

Focal cortical dysplasia – review

International Nuclear Information System (INIS)

Kabat, Joanna; Król, Przemysław

2012-01-01

Focal cortical dysplasia is a malformation of cortical development, which is the most common cause of medically refractory epilepsy in the pediatric population and the second/third most common etiology of medically intractable seizures in adults. Both genetic and acquired factors are involved in the pathogenesis of cortical dysplasia. Numerous classifications of the complex structural abnormalities of focal cortical dysplasia have been proposed – from Taylor et al. in 1971 to the last modification of Palmini classification made by Blumcke in 2011. In general, three types of cortical dysplasia are recognized. Type I focal cortical dysplasia with mild symptomatic expression and late onset, is more often seen in adults, with changes present in the temporal lobe. Clinical symptoms are more severe in type II of cortical dysplasia usually seen in children. In this type, more extensive changes occur outside the temporal lobe with predilection for the frontal lobes. New type III is one of the above dysplasias with associated another principal lesion as hippocampal sclerosis, tumor, vascular malformation or acquired pathology during early life. Brain MRI imaging shows abnormalities in the majority of type II dysplasias and in only some of type I cortical dysplasias. The most common findings on MRI imaging include: focal cortical thickening or thinning, areas of focal brain atrophy, blurring of the gray-white junction, increased signal on T2- and FLAIR-weighted images in the gray and subcortical white matter often tapering toward the ventricle. On the basis of the MRI findings, it is possible to differentiate between type I and type II cortical dysplasia. A complete resection of the epileptogenic zone is required for seizure-free life. MRI imaging is very helpful to identify those patients who are likely to benefit from surgical treatment in a group of patients with drug-resistant epilepsy. However, in type I cortical dysplasia, MR imaging is often normal, and also in both

Anti-Neuronal Autoantibodies in Both Drug Responsive and Resistant Focal Seizures with Unknown Cause.

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Gozubatik-Celik, Gokcen; Ozkara, Cigdem; Ulusoy, Canan; Gunduz, Aysegul; Delil, Sakir; Yeni, Naz; Tuzun, Erdem

2017-09-01

and Objective Autoimmunity is an emerging field of research in the etiology of different neurological disorders including epilepsy. We aimed to investigate the presence of neuronal autoantibodies in focal epilepsy with unknown cause and their clinical correlates in both drug-responsive and resistant patients. Between 2009 and 2010 94 patients were prospectively enrolled, had their antibodies tested and clinically followed." An additional 50 age- and gender-matched controls were also tested for antibodies. Age at examination, gender, age at onset, seizure frequency, risk factors, seizure precipitants, and type of seizures were noted. Plasma obtained from patients was frozen at -80°C and analysed for autoantibodies against VGKC-complex, VGCC, GAD, LGI1, CASPR2, NMDA, AMPA and GABAB receptors with immunocytochemistry and radioimmunoassay as required. Thirteen (13.8%) patients, but none of the controls, had antibodies (p=0.003). Antibodies were directed against the uncharacterized components of VGKC-complex in 5 patients (5.3%), GAD in 4 patients (4.2%), NMDA-R in 1 patient (1%), AMPA-R in 1 patient (1%) and both GAD and VGKC-complex in 2 patients (2.1%). Prognosis of epilepsy, in subsequent follow-up, did not correlate to general presence of anti-neuronal antibodies with slightly more patients with antibodies epilepsy control than without (76.9% vs. 69.1%, not-statistically significant. Three patients with suspected active autoimmunity and epilepsy who were treated, showed a response to treatment with a reduction in the seizure frequency. Although most clinical features were identical between seropositive and seronegative patient groups, seropositive patients were more likely to have inflammatory/autoimmune disorders in their medical history. In keeping with previous studies, we have shown anti-neuronal antibodies in a proportion of focal epilepsy patients. Although autoimmunity might merely occur as a bystander effect in many chronic neurological disorders

Amygdala enlargement: Temporal lobe epilepsy subtype or nonspecific finding?

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Reyes, Anny; Thesen, Thomas; Kuzniecky, Ruben; Devinsky, Orrin; McDonald, Carrie R; Jackson, Graeme D; Vaughan, David N; Blackmon, Karen

2017-05-01

Amygdala enlargement (AE) is observed in patients with temporal lobe epilepsy (TLE), which has led to the suggestion that it represents a distinct TLE subtype; however, it is unclear whether AE is found at similar rates in other epilepsy syndromes or in healthy controls, which would limit its value as a marker for focal epileptogenicity. We compared rates of AE, defined quantitatively from high-resolution T1-weighted MRI, in a large multi-site sample of 136 patients with nonlesional localization related epilepsy (LRE), including TLE and extratemporal (exTLE) focal epilepsy, 34 patients with idiopathic generalized epilepsy (IGE), and 233 healthy controls (HCs). AE was found in all groups including HCs; however, the rate of AE was higher in LRE (18.4%) than in IGE (5.9%) and HCs (6.4%). Patients with unilateral LRE were further evaluated to compare rates of concordant ipsilateral AE in TLE and exTLE, with the hypothesis that rates of ipsilateral AE would be higher in TLE. Although ipsilateral AE was higher in TLE (19.4%) than exTLE (10.5%), this difference was not significant. Furthermore, among the 25 patients with unilateral LRE and AE, 13 (52%) had either bilateral AE or AE contralateral to seizure onset. Results suggest that AE, as defined with MRI volumetry, may represent an associated feature of nonlesional localization related epilepsy with limited seizure onset localization value. Copyright © 2017 Elsevier B.V. All rights reserved.

Automatic multimodal detection for long-term seizure documentation in epilepsy.

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Fürbass, F; Kampusch, S; Kaniusas, E; Koren, J; Pirker, S; Hopfengärtner, R; Stefan, H; Kluge, T; Baumgartner, C

2017-08-01

This study investigated sensitivity and false detection rate of a multimodal automatic seizure detection algorithm and the applicability to reduced electrode montages for long-term seizure documentation in epilepsy patients. An automatic seizure detection algorithm based on EEG, EMG, and ECG signals was developed. EEG/ECG recordings of 92 patients from two epilepsy monitoring units including 494 seizures were used to assess detection performance. EMG data were extracted by bandpass filtering of EEG signals. Sensitivity and false detection rate were evaluated for each signal modality and for reduced electrode montages. All focal seizures evolving to bilateral tonic-clonic (BTCS, n=50) and 89% of focal seizures (FS, n=139) were detected. Average sensitivity in temporal lobe epilepsy (TLE) patients was 94% and 74% in extratemporal lobe epilepsy (XTLE) patients. Overall detection sensitivity was 86%. Average false detection rate was 12.8 false detections in 24h (FD/24h) for TLE and 22 FD/24h in XTLE patients. Utilization of 8 frontal and temporal electrodes reduced average sensitivity from 86% to 81%. Our automatic multimodal seizure detection algorithm shows high sensitivity with full and reduced electrode montages. Evaluation of different signal modalities and electrode montages paces the way for semi-automatic seizure documentation systems. Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

Corpus callosotomy in a patient with startle epilepsy.

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Gómez, Nicolás Garófalo; Hamad, Ana Paula; Marinho, Murilo; Tavares, Igor M; Carrete, Henrique; Caboclo, Luís Otávio; Yacubian, Elza Márcia; Centeno, Ricardo

2013-03-01

Startle epilepsy is a syndrome of reflex epilepsy in which the seizures are precipitated by a sudden and surprising, usually auditory, stimulus. We describe herein a girl who had been suffering with startle-induced seizures since 2 years of age. She had focal, tonic and tonic-clonic seizures, refractory to antiepileptic treatment. Daily tonic seizures led to very frequent falls and morbidity. Neurologically, she had no deficit. Interictal EEG showed slow waves and epileptiform discharges in central and fronto-central regions. Video-polygraphic recordings of seizures, triggered by stimuli, showed generalised symmetric tonic posturing with ictal EEG, characterised by an abrupt and diffuse electrodecremental pattern of fast activity, followed by alpha-theta rhythm superimposed by epileptic discharges predominantly over the vertex and anterior regions. Magnetic resonance imaging showed no abnormalities. Corpus callosotomy was performed when the patient was 17. Since surgery, the patient (one year follow-up) has remained seizure-free. Corpus callosotomy may be considered in patients with startle epilepsy and tonic seizures, in the absence of focal lesions amenable to surgery. [Published with video sequences].

Etiologic features and utilization of antiepileptic drugs in people with chronic epilepsy in China: Report from the Epilepsy Cohort of Huashan Hospital (ECoH).

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Ge, Yan; Yu, Peimin; Ding, Ding; Wang, Ping; Shi, Yunbo; Zhao, Ting; Tang, Xinghua; Hong, Zhen

2015-10-01

Chronic epilepsy is estimated to affect more than 2 million people in China. However, data of its clinical characteristics was rarely reported in China. In the present study, we summarized the etiologic features and utilization patterns of antiepileptic drugs (AEDs) in people with chronic epilepsy in a tertiary medical center in China. We prospectively recruited people with chronic epilepsy treated at the Epilepsy Outpatient Clinic of Huashan Hospital during October 2009 to August 2013. Demographic data, clinical characteristics, AED treatment, epilepsy-associated risk factors and medical history, and results of supplementary examinations of each participant were collected retrospectively via an interviewer-administered questionnaire and confirmed by the medical records. Among 554 people with chronic epilepsy, 58.0% of them were male, 66.8% had focal seizure, and 29.2% had symptomatic cause. Developmental anomalies of cerebral structure (16.7%) and cerebral trauma (16.7%) shared the leading cause of symptomatic epilepsy among children with epilepsy. While cerebral trauma (29.1%) and cerebrovascular disorder (36.4%) were the most common causes in groups of adults and elderly. Fifty percent of participants were taking AED monotherapy. Proportions of people with idiopathic, cryptogenic and symptomatic epilepsy treated by multitherapy were 35%, 46% and 45.6%, respectively. Valproic acid (VPA) was the most frequently utilized AED as monotherapy (32.7%) and within multitherapy (62.5%). This hospital-based study reported that etiologic features were diverse in different age groups of people with chronic epilepsy. VPA was widely utilized to treat chronic epilepsy in mainland China. Copyright © 2015 Elsevier B.V. All rights reserved.

Dual Pathology in Rasmussen's Encephalitis: A Report of Coexistent Focal Cortical Dysplasia and Review of the Literature.

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Prayson, Richard A

2012-01-01

Rasmussen's encephalitis is a well-established, albeit rare cause of medically intractable epilepsy. In a small number of Rasmussen's cases, a second pathology is identified, which independently can cause medically intractable seizures (dual pathology). This paper documents a case of a 13-year-old male who presented with medically intractable epilepsy. The patient underwent a series of surgical resections, early on resulting in a diagnosis of focal cortical dysplasia and later yielding a diagnosis of coexistent Rasmussen's encephalitis, marked by chronic inflammation, microglial nodules, and focal cortical atrophy, combined with focal cortical dysplasia (Palmini et al. type IIA, ILAE type IIA). The literature on dual pathology in the setting of Rasmussen's encephalitis is reviewed.

Therapy-related change of corpus callosum in a young patient with epilepsy

International Nuclear Information System (INIS)

Feitova, V.; Krupa, P.; Feit, J.

2002-01-01

Focal nonhemorrhagic lesion in the splenium of the corpus callosum in a patient with epilepsy treated with antiepileptic drugs was observed with MRI imaging. We have found only one such case during the past 2 years (series of MRI examinations of approximately 500 patients with various forms of epilepsy). (orig.)

Familial epilepsy in Algeria: Clinical features and inheritance profiles.

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Chentouf, Amina; Dahdouh, Aïcha; Guipponi, Michel; Oubaiche, Mohand Laïd; Chaouch, Malika; Hamamy, Hanan; Antonarakis, Stylianos E

2015-09-01

To document the clinical characteristics and inheritance pattern of epilepsy in multigeneration Algerian families. Affected members from extended families with familial epilepsy were assessed at the University Hospital of Oran in Algeria. Available medical records, neurological examination, electroencephalography and imaging data were reviewed. The epilepsy type was classified according to the criteria of the International League Against Epilepsy and modes of inheritance were deduced from pedigree analysis. The study population included 40 probands; 23 male (57.5%) and 17 female subjects (42.5%). The mean age of seizure onset was 9.5 ± 6.1 years. According to seizure onset, 16 patients (40%) had focal seizures and 20 (50%) had generalized seizures. Seizure control was achieved for two patients (5%) for 10 years, while 28 (70%) were seizure-free for 3 months. Eleven patients (27.5%) had prior febrile seizures, 12 were diagnosed with psychiatric disorders and four families had syndromic epilepsy. The consanguinity rate among parents of affected was 50% with phenotypic concordance observed in 25 families (62.5%). Pedigree analysis suggested autosomal dominant (AD) inheritance with or without reduced penetrance in 18 families (45%), probable autosomal recessive (AR) inheritance in 14 families (35%), and an X-linked recessive inheritance in one family. This study reveals large Algerian families with multigenerational inheritance of epilepsy. Molecular testing such as exome sequencing would clarify the genetic basis of epilepsy in some of our families. Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

The occurrence and characteristics of auras in a large epilepsy cohort

DEFF Research Database (Denmark)

Nakken, K O; Solaas, M H; Kjeldsen, M J

2009-01-01

= 1897) and 39% of those with active epilepsy (n = 765) had experienced an aura. Six percent reported more than one type. Non-specified auras were most frequently reported (35%), followed by somatosensory (11%) and vertiginous (11%). While the majority of those reporting auras (59%) had focal epilepsies...... is underestimated, especially in children and those with learning disabilities....

Classification of childhood epilepsies in a tertiary pediatric neurology clinic using a customized classification scheme from the international league against epilepsy 2010 report.

Science.gov (United States)

Khoo, Teik-Beng

2013-01-01

In its 2010 report, the International League Against Epilepsy Commission on Classification and Terminology had made a number of changes to the organization, terminology, and classification of seizures and epilepsies. This study aims to test the usefulness of this revised classification scheme on children with epilepsies aged between 0 and 18 years old. Of 527 patients, 75.1% only had 1 type of seizure and the commonest was focal seizure (61.9%). A specific electroclinical syndrome diagnosis could be made in 27.5%. Only 2.1% had a distinctive constellation. In this cohort, 46.9% had an underlying structural, metabolic, or genetic etiology. Among the important causes were pre-/perinatal insults, malformation of cortical development, intracranial infections, and neurocutaneous syndromes. However, 23.5% of the patients in our cohort were classified as having "epilepsies of unknown cause." The revised classification scheme is generally useful for pediatric patients. To make it more inclusive and clinically meaningful, some local customizations are required.

Network Alterations Supporting Word Retrieval in Patients with Medial Temporal Lobe Epilepsy

Science.gov (United States)

Protzner, Andrea B.; McAndrews, Mary Pat

2011-01-01

Although the hippocampus is not considered a key structure in semantic memory, patients with medial-temporal lobe epilepsy (mTLE) have deficits in semantic access on some word retrieval tasks. We hypothesized that these deficits reflect the negative impact of focal epilepsy on remote cerebral structures. Thus, we expected that the networks that…

Resting-State Functional MR Imaging for Determining Language Laterality in Intractable Epilepsy.

Science.gov (United States)

DeSalvo, Matthew N; Tanaka, Naoaki; Douw, Linda; Leveroni, Catherine L; Buchbinder, Bradley R; Greve, Douglas N; Stufflebeam, Steven M

2016-10-01

Purpose To measure the accuracy of resting-state functional magnetic resonance (MR) imaging in determining hemispheric language dominance in patients with medically intractable focal epilepsies against the results of an intracarotid amobarbital procedure (IAP). Materials and Methods This study was approved by the institutional review board, and all subjects gave signed informed consent. Data in 23 patients with medically intractable focal epilepsy were retrospectively analyzed. All 23 patients were candidates for epilepsy surgery and underwent both IAP and resting-state functional MR imaging as part of presurgical evaluation. Language dominance was determined from functional MR imaging data by calculating a laterality index (LI) after using independent component analysis. The accuracy of this method was assessed against that of IAP by using a variety of thresholds. Sensitivity and specificity were calculated by using leave-one-out cross validation. Spatial maps of language components were qualitatively compared among each hemispheric language dominance group. Results Measurement of hemispheric language dominance with resting-state functional MR imaging was highly concordant with IAP results, with up to 96% (22 of 23) accuracy, 96% (22 of 23) sensitivity, and 96% (22 of 23) specificity. Composite language component maps in patients with typical language laterality consistently included classic language areas such as the inferior frontal gyrus, the posterior superior temporal gyrus, and the inferior parietal lobule, while those of patients with atypical language laterality also included non-classical language areas such as the superior and middle frontal gyri, the insula, and the occipital cortex. Conclusion Resting-state functional MR imaging can be used to measure language laterality in patients with medically intractable focal epilepsy. (©) RSNA, 2016 Online supplemental material is available for this article.

Tegretol no tratamento da epilepsia Tegretol in the treatment of epilepsy

Directory of Open Access Journals (Sweden)

Jorge Armbrust-Figueiredo

1968-09-01

Full Text Available O Tegretol (5-carbamil-5H-dibenzo-b,L-azepina foi usado no tratamento de 68 pacientes com epilepsia; 54 apresentavam epilepsia temporal (42 com crises psicomotoras e 12 com crises complexas, 11 epilepsia focal de projeção não temporal e 3 epilepsia centrencefálica com crises P.M. Resultados satisfatórios foram observados em 82% dos 65 pacientes com epilepsia focal; os 3 pacientes com crises P.M. não foram influenciados pelo tratamento. A ação sobre as manifestações psíquicas que acompanhavam o quadro crítico ou intercrítico das crises ficou bem evidenciada. Os pacientes melhoraram de seus sintomas na grande maioria dos casos, conseguindo adaptar-se melhor no ambiente familiar, profissional e escolar. O efeito sobre as alterações eletrencefalográficas é moderado, e a evolução clínica não acompanha as modificações dos traçados. As doses usadas, em geral de 400 a 600 mg diários, são suficientes para o controle das crises na maioria dos casos e estão muito abaixo dos limites considerados como de segurança. As reações secundárias foram observadas em pequeno número de casos e foram sempre transitórias. O Tegretol representa, em nossa opinião, recurso de valor no trataemnto das epilepsias focais, particularmente nas crises psicomotoras.Tegretol (5-carbanyl-5H-dibenzo-b,L-azepina was used experimentally in the theatment of 68 epileptic patients; 54 had temporal lobe epilepsy (42 had psychomotor and 12 had complex seizures, 11 had focal non-temporal lobe epilepsy and 3 had P.M. epilepsy. Satisfactory results were obtained in 82 per cent of the 65 patients with focal epilepsy, but failure of the treatment was observed in all cases of P.M. epilepsy. The drug has a very good influence on the critical and intercritical mental phenomena which are frequently present in these patients. The personality of the patient improves and he becomes better adjusted to his family, his work or school. The action of the electroencephalogram

Sleep disturbances in children with epilepsy compared with their nearest-aged siblings.

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Wirrell, Elaine; Blackman, Marlene; Barlow, Karen; Mah, Jean; Hamiwka, Lorie

2005-11-01

The aim of the study was to compare sleep patterns in children with epilepsy with those of their non-epileptic siblings and to determine which epilepsy-specific factors predict greater sleep disturbance. We conducted a case-control study of 55 children with epilepsy (mean age 10y, range 4 to 16y; 27 males, 28 females) and their nearest-aged non-epileptic sibling (mean age 10y, range 4 to 18y; 26 males, 29 females). Epilepsy was idiopathic generalized in eight children (15%), symptomatic generalized in seven (13%), and focal in 40 (73%); the mean duration was 5 years 8 months. Parents or caregivers completed the Sleep Behavior Questionnaire (SBQ) and Child Behavior Checklist (CBCL) for patients and controls, and the Quality of Life in Childhood Epilepsy (QOLCE) for patients. Patients had a higher (more adverse) Total Sleep score (p<0.001) and scored worse than controls on nearly all subscales of the SBQ. In patients, higher Total Sleep scores were correlated with higher scores on the Withdrawn, Somatic complaints, Social problems, and Attention subscales of the CBCL, and significantly lower Total Quality of Life Scores. Refractory epilepsy, mental retardation, and remote symptomatic etiology predicted greater sleep problems in those with epilepsy. We conclude that children with epilepsy in this current study had significantly greater sleep problems than their non-epileptic siblings.

Computational model of neuron-astrocyte interactions during focal seizure generation

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Davide eReato

2012-10-01

Full Text Available Empirical research in the last decade revealed that astrocytes can respond to neurotransmitters with Ca2+ elevations and generate feedback signals to neurons which modulate synaptic transmission and neuronal excitability. This discovery changed our basic understanding of brain function and provided new perspectives for how astrocytes can participate not only to information processing, but also to the genesis of brain disorders, such as epilepsy. Epilepsy is a neurological disorder characterized by recurrent seizures that can arise focally at restricted areas and propagate throughout the brain. Studies in brain slice models suggest that astrocytes contribute to epileptiform activity by increasing neuronal excitability through a Ca2+-dependent release of glutamate. The underlying mechanism remains, however, unclear. In this study, we implemented a parsimonious network model of neurons and astrocytes. The model consists of excitatory and inhibitory neurons described by Izhikevich's neuron dynamics. The experimentally observed Ca2+ change in astrocytes in response to neuronal activity was modeled with linear equations. We considered that glutamate is released from astrocytes above certain intracellular Ca2+ concentrations thus providing a non-linear positive feedback signal to neurons. Propagating seizure-like ictal discharges (IDs were reliably evoked in our computational model by repeatedly exciting a small area of the network, which replicates experimental results in a slice model of focal ID in entorhinal cortex. We found that the threshold of focal ID generation was lowered when an excitatory feedback-loop between astrocytes and neurons was included. Simulations show that astrocytes can contribute to ID generation by directly affecting the excitatory/inhibitory balance of the neuronal network. Our model can be used to obtain mechanistic insights into the distinct contributions of the different signaling pathways to the generation and

Clinical features, proximate causes, and consequences of active convulsive epilepsy in Africa

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Kariuki, Symon M; Matuja, William; Akpalu, Albert; Kakooza-Mwesige, Angelina; Chabi, Martin; Wagner, Ryan G; Connor, Myles; Chengo, Eddie; Ngugi, Anthony K; Odhiambo, Rachael; Bottomley, Christian; White, Steven; Sander, Josemir W; Neville, Brian G R; Newton, Charles R J C

2014-01-01

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

Ictal cerebral perfusion patterns in partial epilepsy: SPECT subtraction

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Lee, Hyang Woon; Hong, Seung Bong; Tae, Woo Suk; Kim, Sang Eun; Seo, Dae Won; Jeong, Seung Cheol; Yi, Ji Young; Hong, Seung Chyul

2000-01-01

To investigate the various ictal perfusion patterns and find the relationships between clinical factors and different perfusion patterns. Interictal and ictal SPECT and SPECT subtraction were performed in 61 patients with partial epilepsy. Both positive images showing ictal hyperperfusion and negative images revealing ictal hypoperfusion were obtained by SPECT subtraction. The ictal perfusion patterns of subtracted SPECT were classified into focal hyperperfusion, hyperperfusion-plus, combined hyperperfusion-hypoperfusion, and focal hypoperfusion only. The concordance rates with epileptic focus were 91.8% in combined analysis of ictal hyperperfusion and hypoperfusion images of subtracted SPECT, 85.2% in hyperperfusion images only of subtracted SPECT, and 68.9% in conventional ictal SPECT analysis. Ictal hypoperfusion occurred less frequently in temporal lobe epilepsy (TLE) than extratemporal lobe epilepsy. Mesial temporal hyperperfusion alone was seen only in mesial TLE while lateral temporal hyperperfusion alone was observed only in neocortical TLE. Hippocampal sclerosis had much lower incidence of ictal hypoperfusion than any other pathology. Some patients showed ictal hypoperfusion at epileptic focus with ictal hyperperfusion in the neighboring brain regions where ictal discharges propagated. Hypoperfusion as well as hyperperfusion in ictal SPECT should be considered for localizing epileptic focus. Although the mechanism of ictal hypoperfusion could be an intra-ictal early exhaustion of seizure focus or a steal phenomenon by the propagation of ictal discharges to adjacent brain areas, further study is needed to elucidate it.=20

Ictal cerebral perfusion patterns in partial epilepsy: SPECT subtraction

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Lee, Hyang Woon; Hong, Seung Bong; Tae, Woo Suk; Kim, Sang Eun; Seo, Dae Won; Jeong, Seung Cheol; Yi, Ji Young; Hong, Seung Chyul [Sungkyunkwan Univ. School of Medicine, Seoul (Korea, Republic of)

2000-06-01

To investigate the various ictal perfusion patterns and find the relationships between clinical factors and different perfusion patterns. Interictal and ictal SPECT and SPECT subtraction were performed in 61 patients with partial epilepsy. Both positive images showing ictal hyperperfusion and negative images revealing ictal hypoperfusion were obtained by SPECT subtraction. The ictal perfusion patterns of subtracted SPECT were classified into focal hyperperfusion, hyperperfusion-plus, combined hyperperfusion-hypoperfusion, and focal hypoperfusion only. The concordance rates with epileptic focus were 91.8% in combined analysis of ictal hyperperfusion and hypoperfusion images of subtracted SPECT, 85.2% in hyperperfusion images only of subtracted SPECT, and 68.9% in conventional ictal SPECT analysis. Ictal hypoperfusion occurred less frequently in temporal lobe epilepsy (TLE) than extratemporal lobe epilepsy. Mesial temporal hyperperfusion alone was seen only in mesial TLE while lateral temporal hyperperfusion alone was observed only in neocortical TLE. Hippocampal sclerosis had much lower incidence of ictal hypoperfusion than any other pathology. Some patients showed ictal hypoperfusion at epileptic focus with ictal hyperperfusion in the neighboring brain regions where ictal discharges propagated. Hypoperfusion as well as hyperperfusion in ictal SPECT should be considered for localizing epileptic focus. Although the mechanism of ictal hypoperfusion could be an intra-ictal early exhaustion of seizure focus or a steal phenomenon by the propagation of ictal discharges to adjacent brain areas, further study is needed to elucidate it.

Incidence, Risk Factors and Consequences of Epilepsy-Related Injuries and Accidents: A Retrospective, Single Center Study

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Laurent M. Willems

2018-06-01

Full Text Available Introduction: This study was designed to evaluate risk factors and incidence of epilepsy-related injuries and accidents (ERIA at an outpatient clinic of a German epilepsy center providing healthcare to a mixed urban and rural population of over one million inhabitants.Methods: Data acquisition was performed between 10/2013 and 09/2014 using a validated patient questionnaire on socioeconomic status, course of epilepsy, quality of life (QoL, depression, injuries and accidents associated with seizures or inadequate periictal patterns of behavior concerning a period of 3 months. Univariate analysis, multiple testing and regression analysis were performed to identify possible variables associated with ERIA.Results: A total of 292 patients (mean age 40.8 years, range 18–86; 55% female were enrolled and analyzed. Focal epilepsy was diagnosed in 75% of the patients. The majority was on an antiepileptic drug (AEDs polytherapy (mean number of AEDs: 1.65. Overall, 41 patients (14.0% suffered from epilepsy-related injuries and accidents in a 3-month period. Besides lacerations (n = 18, 6.2%, abrasions and bruises (n = 9, 3.1%, fractures (n = 6, 2.2% and burns (n = 3, 1.0%, 17 mild injuries (5.8% were reported. In 20 (6.8% of the total cohort cases, urgent medical treatment with hospitalization was necessary. Epilepsy-related injuries and accidents were related to active epilepsy, occurrence of generalized tonic-clonic seizures (GTCS and drug-refractory course as well as reported ictal falls, ictal loss of consciousness and abnormal peri-ictal behavior in the medical history. In addition, patients with ERIA had significantly higher depression rates and lower QoL.Conclusion: ERIA and their consequences should be given more attention and standardized assessment for ERIA should be performed in every outpatient visit.

Dual Pathology in Rasmussen’s Encephalitis: A Report of Coexistent Focal Cortical Dysplasia and Review of the Literature

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Richard A. Prayson

2012-01-01

Full Text Available Rasmussen’s encephalitis is a well-established, albeit rare cause of medically intractable epilepsy. In a small number of Rasmussen's cases, a second pathology is identified, which independently can cause medically intractable seizures (dual pathology. This paper documents a case of a 13-year-old male who presented with medically intractable epilepsy. The patient underwent a series of surgical resections, early on resulting in a diagnosis of focal cortical dysplasia and later yielding a diagnosis of coexistent Rasmussen’s encephalitis, marked by chronic inflammation, microglial nodules, and focal cortical atrophy, combined with focal cortical dysplasia (Palmini et al. type IIA, ILAE type IIA. The literature on dual pathology in the setting of Rasmussen’s encephalitis is reviewed.

Visual and auditory socio-cognitive perception in unilateral temporal lobe epilepsy in children and adolescents: a prospective controlled study.

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Laurent, Agathe; Arzimanoglou, Alexis; Panagiotakaki, Eleni; Sfaello, Ignacio; Kahane, Philippe; Ryvlin, Philippe; Hirsch, Edouard; de Schonen, Scania

2014-12-01

A high rate of abnormal social behavioural traits or perceptual deficits is observed in children with unilateral temporal lobe epilepsy. In the present study, perception of auditory and visual social signals, carried by faces and voices, was evaluated in children or adolescents with temporal lobe epilepsy. We prospectively investigated a sample of 62 children with focal non-idiopathic epilepsy early in the course of the disorder. The present analysis included 39 children with a confirmed diagnosis of temporal lobe epilepsy. Control participants (72), distributed across 10 age groups, served as a control group. Our socio-perceptual evaluation protocol comprised three socio-visual tasks (face identity, facial emotion and gaze direction recognition), two socio-auditory tasks (voice identity and emotional prosody recognition), and three control tasks (lip reading, geometrical pattern and linguistic intonation recognition). All 39 patients also benefited from a neuropsychological examination. As a group, children with temporal lobe epilepsy performed at a significantly lower level compared to the control group with regards to recognition of facial identity, direction of eye gaze, and emotional facial expressions. We found no relationship between the type of visual deficit and age at first seizure, duration of epilepsy, or the epilepsy-affected cerebral hemisphere. Deficits in socio-perceptual tasks could be found independently of the presence of deficits in visual or auditory episodic memory, visual non-facial pattern processing (control tasks), or speech perception. A normal FSIQ did not exempt some of the patients from an underlying deficit in some of the socio-perceptual tasks. Temporal lobe epilepsy not only impairs development of emotion recognition, but can also impair development of perception of other socio-perceptual signals in children with or without intellectual deficiency. Prospective studies need to be designed to evaluate the results of appropriate re

Epilepsy and violence: case series concerning physical trauma in children of persons with epilepsy

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Gauffin H

2014-11-01

Full Text Available Helena Gauffin1,2 Anne-Marie Landtblom1–4 1Department of Neurology, Linköping University, Linköping, Sweden; 2Department of Clinical and Experimental Medicine, Linköping University, Linköping, Sweden; 3Neurology Unit, Department of Medical Specialist, General Hospital, Department of Medicine and Health Sciences, IMM, County Council, Linköping University, Motala, Sweden; 4Department of Neuroscience, Uppsala University, Uppsala, Sweden Abstract: Historically, epilepsy has been associated with violence, but more recent studies have emphasized genetic and psychosocial factors as more important. The case series presented here aim to highlight the difficult situation the affected children are in. We report on three cases when children have been traumatized and, in one case, even been killed by their parent who was diagnosed with epilepsy. In the first case, we describe a woman with juvenile myoclonic epilepsy who was sentenced to forensic psychiatry care for killing her child. She lived under difficult psychosocial circumstances and a suicide attempt contributed to what happened. The second case describes a man with post-traumatic seizures who was sentenced for child abuse. Ictal or postictal violence was considered in these two cases but a causal link between the violence and epilepsy has not been established. In the third case, we describe a woman with focal epilepsy and psychogenic non-epileptic seizures (PNESs. Her child was hurt and frightened in relation to violent seizures, which were regarded as PNESs. This case series demonstrates that children of parents with epilepsy can be in a vulnerable situation. No causality has been established between the seizures and these events, so consequently other factors such as psychosocial stress, low cognitive function, and a suicide attempt must also be considered as important. When a child is hurt by a parent with epilepsy the patient must be closely examined to determine the role of the seizures

Global Health: Epilepsy.

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Ali, Amza

2018-04-01

Epilepsy is a frequently misunderstood and highly stigmatized condition. Major treatment gaps exist across the world, most so in areas of financial constraint. Classification permits the best approaches to treatment and to ascertaining prognosis. The International League Against Epilepsy's new classification system emphasizes clinical aspects and utilizes all available resources to determine whether it is a focal or generalized epilepsy. The most important tools are a careful history, clinical examination, electroencephalography, and appropriate neuroimaging. Inadequate, delayed, and incomplete evaluation may lead to misdiagnosis and costly mismanagement. Treatment is generally pharmacological, with approximately 20 to 30% of patients eventually proving refractory to medications and thus becoming potential surgical candidates. The type of epilepsy, age, gender, comorbidities, drug interactions, and drug cost are important factors in choosing an antiepileptic drug (AED). The teratogenic potential of some AEDs, weight gain, and menstrual hormone-related issues are important considerations in women. The impact of AEDs on bone health is critical in all age groups, particularly in the elderly. Psychiatric problems, mostly depression and anxiety, can have a great impact on seizure control and overall quality of life. Finally, effective partnerships and collaborations can bring resources, both human and financial, to regions that would otherwise find it impossible to effect change on their own. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Lacosamide in the treatment of patients with epilepsy and intellectual disabilities: A long-term study of 136 patients.

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Böttcher, Stefan; Lutz, Martin T; Mayer, Thomas

2017-10-01

This study aimed to analyze the retention rate of lacosamide (LCM) in patients with epilepsy and intellectual disabilities (IDs), to identify factors influencing retention rate, and to investigate the LCM retention rate with and without concomitant sodium channel blocker (SCB). We hypothesized that the retention rate of LCM with concomitant SCB would be lower than without SCB. Using the Kaplan-Meier estimator, we conducted a monocentric, retrospective, observational, open-label study to evaluate LCM retention rates in patients with IDs and drug-resistant epilepsy. In addition, the impact of therapy-related variables on the long-term retention of LCM was evaluated. One hundred thirty-six subjects with IDs and drug-resistant epilepsy were included (age 2-66 years); most patients had focal epilepsy. Long-term retention rates were 62.0% at 1 year, 43.7% at 2 years, and 29.1% at 3 and 4 years. Reasons for LCM discontinuation included insufficient therapeutic benefits (69%), adverse events (11%), or a combination of both factors (8%). The LCM retention rate was influenced by the number of background antiepileptic drugs (AEDs). An additional and independent influence of concomitant therapy with SCB on retention rate could not be confirmed. One of the major challenges in medically caring for patients with epilepsy and IDs is the high rate of drug resistance. However, there is a lack of evidence-based information about the efficacy and tolerability of AEDs in this population. It has been shown that concomitant SCB use is a key factor in increasing the risk of LCM failure in children with epilepsy. This finding has not been replicated in our predominantly adult sample of patients with IDs. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

FOCAL EPILEPTIC MYOCLONUS IN KOZHEVNIKOV–RASMUSSEN SYNDROME

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N. E. Kvaskova

2016-01-01

Full Text Available The article presents the clinical, electroencephalographic (EEG and neuroimaging features, as well as the results of treatment of patients with focal epileptic myoclonus (FEM with the Kozhevnikov–Rasmussen syndrome (KRS. FEM in KRS-patients was identified in 11 cases, accounting for 0.9 % of all the cases of epilepsy with the onset of seizures up to 18 years (n = 1261. The age at onset of KRS ranged from 3 to 21 years (average – 9.2 ± 5.7 years. In the active period of the disease of all patients in the clinical picture the active FEM appeared and increasing frequency of the secondary generalized seizures (SGS. In addition SGS and FEM, the clinical picture of the disease in most patients (91 % the focal motor (clonic and the somatosensory focal seizures were observed. As the disease progressed, the FEM became more pronounced in frequency and intensity, seized more muscle groups, localizing mainly in the muscles of the trunk and limbs. The typical EEG pattern of FEM patients with KRS was regional epileptiform activity that occurs in the structure of the continued regional slowing localizing maximum of the fronto-central-temporal region. During the magnetic resonance tomography of the brain in dynamics all the patients observed the increase in total cortical hemiatrophy. In all the cases, the appointment of antiepileptic therapy resulted in a slowing of the FEM, however, a complete remission was reached at none of the patients. Two patients were made surgical treatment of epilepsy. In one case remission of epileptic seizures was observed after right-side hemispherotomy. Our study showed that FEM is very resistant type of epileptic seizures. This fact calls for the identification of the FEM at the early stages of the disease with the purpose to improve the prognosis, as well as for an earlier surgical treatment.

Clinical features, proximate causes, and consequences of active convulsive epilepsy in Africa.

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Kariuki, Symon M; Matuja, William; Akpalu, Albert; Kakooza-Mwesige, Angelina; Chabi, Martin; Wagner, Ryan G; Connor, Myles; Chengo, Eddie; Ngugi, Anthony K; Odhiambo, Rachael; Bottomley, Christian; White, Steven; Sander, Josemir W; Neville, Brian G R; Newton, Charles R J C; Twine, Rhian; Gómez Olivé, F Xavier; Collinson, Mark; Kahn, Kathleen; Tollman, Stephen; Masanja, Honratio; Mathew, Alexander; Pariyo, George; Peterson, Stefan; Ndyomughenyi, Donald; Bauni, Evasius; Kamuyu, Gathoni; Odera, Victor Mung'ala; Mageto, James O; Ae-Ngibise, Ken; Akpalu, Bright; Agbokey, Francis; Adjei, Patrick; Owusu-Agyei, Seth; Kleinschmidt, Immo; Doku, Victor C K; Odermatt, Peter; Nutman, Thomas; Wilkins, Patricia; Noh, John

2014-01-01

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

The value of CT and EEG in cases of posttraumatic epilepsy

International Nuclear Information System (INIS)

Reisner, T.; Zeiler, K.; Wessely, P.

1979-01-01

The results of the clinical neurological investigation were compared with those of electroencephalography (EEG) and computed tomography (CT) in 64 patients suffering from verified posttraumatic epilepsy. Only 18 patients (28%) showed central neurological features with corresponding focal disorders on CT (15 cases) and EEG (11 cases). EEG results were normal in 12 cases (19%), CT in 14 cases (22%). The combined application of both methods led to positive results in 94% on the part of at least one accessory examination. The clinical neurological investigation as well as the EEG and CT were normal in only 3 cases, although the traumatic etiology of epilepsy was beyond doubt. In addition to the clinical neurological investigation, EEG and CT are most important accessory examinations for the diagnosis and followup studies in cases of posttraumatic epilepsy. (orig.) [de

Prevalence of epilepsy in children from a Brazilian area of high deprivation.

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Sampaio, Letícia P B; Caboclo, Luis Otávio S F; Kuramoto, Karina; Reche, Angela; Yacubian, Elza Márcia T; Manreza, Maria Luiza G

2010-02-01

This study assessed the prevalence rate of epilepsy and its causes in children and adolescents in one area of high deprivation in São Paulo, São Paulo, in Southeast Brazil. Between July 2005 and June 2006, 4947 families from a population of 22,013 inhabitants (including 10,405 children and adolescents between the ages of 0 and 16 years) living in the shantytown of Paraisópolis, were interviewed. In the first phase, a validated questionnaire was administered, to identify the occurrence of seizures. In the second phase, clinical history, neurologic examination, electroencephalography, and structural neuroimaging were performed. The diagnosis of epilepsy, including etiology, seizure types, and epileptic syndrome classification, was according to criteria of the International League Against Epilepsy. The screening phase identified 353 presumptive cases. In the second phase, 101 of these cases (33.8%) received the diagnosis of epilepsy. Crude prevalence of epilepsy was 9.7/1000 and prevalence of active epilepsy was 8.7/1000. Partial seizures were the most frequent seizure type (62/101). Symptomatic focal epilepsy was the most common form, and hypoxic-ischemic encephalopathy the most common etiology, reflecting the socioeconomic conditions of this specific population. Adequate public policies regarding perinatal assistance could help reduce the prevalence of epilepsy.

Trends in pediatric epilepsy surgery.

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Shah, Ritesh; Botre, Abhijit; Udani, Vrajesh

2015-03-01

Epilepsy surgery has become an accepted treatment for drug resistant epilepsy in infants and children. It has gained ground in India over the last decade. Certain epilepsy surgically remediable syndromes have been delineated and should be offered surgery earlier rather than later, especially if cognitive/behavioral development is being compromised. Advances in imaging, particularly in MRI has helped identify surgical candidates. Pre-surgical evaluation includes clinical assessment, structural and functional imaging, inter-ictal EEG, simultaneous video -EEG, with analysis of seizure semiology and ictal EEG and other optional investigations like neuropsychology and other newer imaging techniques. If data are concordant resective surgery is offered, keeping in mind preservation of eloquent cortical areas subserving motor, language and visual functions. In case of discordant data or non-lesional MRI, invasive EEG maybe useful using a two-stage approach. With multi-focal / generalized disease, palliative surgery like corpus callosotomy and vagal nerve stimulation maybe useful. A good outcome is seen in about 2/3rd of patients undergoing resective surgery with a low morbidity and mortality. This review outlines important learning aspects of pediatric epilepsy surgery for the general pediatrician.

The molecular and phenotypic spectrum of IQSEC2-related epilepsy.

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Zerem, Ayelet; Haginoya, Kazuhiro; Lev, Dorit; Blumkin, Lubov; Kivity, Sara; Linder, Ilan; Shoubridge, Cheryl; Palmer, Elizabeth Emma; Field, Michael; Boyle, Jackie; Chitayat, David; Gaillard, William D; Kossoff, Eric H; Willems, Marjolaine; Geneviève, David; Tran-Mau-Them, Frederic; Epstein, Orna; Heyman, Eli; Dugan, Sarah; Masurel-Paulet, Alice; Piton, Ame'lie; Kleefstra, Tjitske; Pfundt, Rolph; Sato, Ryo; Tzschach, Andreas; Matsumoto, Naomichi; Saitsu, Hirotomo; Leshinsky-Silver, Esther; Lerman-Sagie, Tally

2016-11-01

IQSEC2 is an X-linked gene associated with intellectual disability (ID) and epilepsy. Herein we characterize the epilepsy/epileptic encephalopathy of patients with IQSEC2 pathogenic variants. Forty-eight patients with IQSEC2 variants were identified worldwide through Medline search. Two patients were recruited from our early onset epileptic encephalopathy cohort and one patient from personal communication. The 18 patients who have epilepsy in addition to ID are the subject of this study. Information regarding the 18 patients was ascertained by questionnaire provided to the treating clinicians. Six affected individuals had an inherited IQSEC2 variant and 12 had a de novo one (male-to-female ratio, 12:6). The pathogenic variant types were as follows: missense (8), nonsense (5), frameshift (1), intragenic duplications (2), translocation (1), and insertion (1). An epileptic encephalopathy was diagnosed in 9 (50%) of 18 patients. Seizure onset ranged from 8 months to 4 years; seizure types included spasms, atonic, myoclonic, tonic, absence, focal seizures, and generalized tonic-clonic (GTC) seizures. The electroclinical syndromes could be defined in five patients: late-onset epileptic spasms (three) and Lennox-Gastaut or Lennox-Gastaut-like syndrome (two). Seizures were pharmacoresistant in all affected individuals with epileptic encephalopathy. The epilepsy in the other nine patients had a variable age at onset from infancy to 18 years; seizure types included GTC and absence seizures in the hereditary cases and GTC and focal seizures in de novo cases. Seizures were responsive to medical treatment in most cases. All 18 patients had moderate to profound intellectual disability. Developmental regression, autistic features, hypotonia, strabismus, and white matter changes on brain magnetic resonance imaging (MRI) were prominent features. The phenotypic spectrum of IQSEC2 disorders includes epilepsy and epileptic encephalopathy. Epileptic encephalopathy is a main clinical

Functional brain networks and prediction models in childhood epilepsy

NARCIS (Netherlands)

Diessen, E.G.A.L. van

2015-01-01

Modern network science revolutionized the field of neuroscience and revealed significant insights into the organization of the brain. Throughout this thesis we applied a network analytical approach to improve our understanding of the pathological mechanisms underlying focal epilepsy. The presented

Memory Functioning in Children with Epilepsy: Frontal Lobe Epilepsy, Childhood Absence Epilepsy, and Benign Epilepsy with Centrotemporal Spikes

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Ana Filipa Lopes

2014-01-01

Full Text Available Specific cognitive deficits have been identified in children with epilepsy irrespective of results on intelligence tests. Memory deficits are traditionally attributed to temporal lobe epilepsy, whereas the impact of frontal lobe epilepsy on memory functions has remained controversial. The aim of this study was the examination of memory abilities in other childhood common epilepsy syndromes (frontal lobe epilepsy (FLE, childhood absence epilepsy (CAE, and benign epilepsy with centrotemporal spikes (BECTS and the influence of epilepsy-related variables. Memory was examined in 90 children with epilepsy (each epilepsy group consisted of 30 children, aged 6–15, and compared with 30 control children. Children with FLE showed significant deficits in verbal and visual memory. In addition, type of epilepsy, earlier age at epilepsy onset, and longer active duration of epilepsy were associated with memory problems. Seizure frequency and treatment, however, did not influence memory performance. This study indicates that children with FLE show greater risk of developing memory deficits than children with CAE or BECTS, thus highlighting the importance of assessing also memory functions in frontal lobe epilepsy.

Mortality risk in children with epilepsy : The Dutch Study of Epilepsy in Childhood

NARCIS (Netherlands)

Callenbach, PMC; Westendorp, RGJ; Geerts, AT; Arts, WFM; Peeters, EAJ; van Donselaar, VA; Stroink, H; Brouwer, O.F.

Objective. Long-term follow-up studies of patients with epilepsy have revealed an increased mortality risk compared with the general population. Mortality of children who have epilepsy in modern times is as yet unknown. Therefore, the objective of this study was to determine mortality of children

ILAE Classification of the Epilepsies Position Paper of the ILAE Commission for Classification and Terminology

Science.gov (United States)

Scheffer, Ingrid E; Berkovic, Samuel; Capovilla, Giuseppe; Connolly, Mary B; French, Jacqueline; Guilhoto, Laura; Hirsch, Edouard; Jain, Satish; Mathern, Gary W.; Moshé, Solomon L; Nordli, Douglas R; Perucca, Emilio; Tomson, Torbjörn; Wiebe, Samuel; Zhang, Yue-Hua; Zuberi, Sameer M

2017-01-01

Summary The ILAE Classification of the Epilepsies has been updated to reflect our gain in understanding of the epilepsies and their underlying mechanisms following the major scientific advances which have taken place since the last ratified classification in 1989. As a critical tool for the practising clinician, epilepsy classification must be relevant and dynamic to changes in thinking, yet robust and translatable to all areas of the globe. Its primary purpose is for diagnosis of patients, but it is also critical for epilepsy research, development of antiepileptic therapies and communication around the world. The new classification originates from a draft document submitted for public comments in 2013 which was revised to incorporate extensive feedback from the international epilepsy community over several rounds of consultation. It presents three levels, starting with seizure type where it assumes that the patient is having epileptic seizures as defined by the new 2017 ILAE Seizure Classification. After diagnosis of the seizure type, the next step is diagnosis of epilepsy type, including focal epilepsy, generalized epilepsy, combined generalized and focal epilepsy, and also an unknown epilepsy group. The third level is that of epilepsy syndrome where a specific syndromic diagnosis can be made. The new classification incorporates etiology along each stage, emphasizing the need to consider etiology at each step of diagnosis as it often carries significant treatment implications. Etiology is broken into six subgroups, selected because of their potential therapeutic consequences. New terminology is introduced such as developmental and epileptic encephalopathy. The term benign is replaced by the terms self-limited and pharmacoresponsive, to be used where appropriate. It is hoped that this new framework will assist in improving epilepsy care and research in the 21st century. PMID:28276062

Disrupted intrinsic and remote functional connectivity in heterotopia-related epilepsy.

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Liu, W; Hu, X; An, D; Gong, Q; Zhou, D

2018-01-01

Several neuroimaging studies have examined neural interactions in patients with periventricular nodular heterotopia (PNH). However, features of the underlying functional network remain poorly understood. In this study, we examined alterations in the local (regional) and remote (interregional) cerebral networks in this disorder. Twenty-eight subjects all having suffered from PNH with epilepsy, as well as 28 age- and sex- matched healthy controls, were enrolled in this study. Amplitude of low-frequency fluctuation (ALFF) and seed-based functional connectivity (FC) were calculated to detect regional neural function and functional network integration, respectively. Compared with healthy controls, patients with PNH-related epilepsy showed decreased ALFF in the ventromedial prefrontal cortex (vmPFC) and precuneus areas. ALFF values in both areas were negative correlated with epilepsy duration (P < .05, Bonferroni-corrected). Furthermore, patients with PNH-related epilepsy had increased remote interregional FC mainly in bilateral prefrontal and parietal cortices, supramarginal gyrus, dorsal cingulate gyrus, and right insula; lower FC was found in posterior brain regions including bilateral parahippocampal gyrus and inferior temporal gyrus. Focal spontaneous hypofunction, as assessed by ALFF, correlates with epilepsy duration in patients with PNH-related epilepsy. Abnormalities existed both within the default-mode network and then across the whole brain, demonstrating that intrinsic brain dysfunction may be related to specific network interactions. Our findings provide novel understanding of the connectivity-based pathophysiological mechanisms of PNH. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

History of epilepsy: nosological concepts and classification.

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Wolf, Peter

2014-09-01

The purpose of this review is to provide insight into the development of the nosological views of the epilepsies, from prehistoric times to the present, and highlight how these views are reflected by terminology and classification. Even the earliest written documents reveal awareness that there are multiple forms of epilepsy, and it is surprising that they should be included under the same disease concept, perhaps because the generalised tonic-clonic seizure served as a common denominator. The Hippocratic doctrine that the seat of epilepsy is in the brain may be rooted in earlier knowledge of traumatic seizures. Galenus differentiated cases where the brain was the primary site of origin from others where epilepsy was concomitant with illness in other parts of the body. This laid the fundament for the distinction between idiopathic and symptomatic epilepsies, the definition of which changed considerably over time. The description of the multiple seizure types as they are known at present started in the late 18th century. Attempts to classify seizure types began in the late 19th century, when Jackson formulated a comprehensive pathophysiological definition of epilepsy. Electroencephalography supported a second dichotomy, between seizures with localised onset and others with immediate involvement of both hemispheres which became known as "generalised". In recent years, advanced methods of studying brain function in vivo, including the generation of both spontaneous and reflex epileptic seizures, have revolutionised our understanding of focal and "generalised" human ictogenesis. Both involve complex neuronal networks which are currently being investigated.

Clinical characteristics in focal cortical dysplasia: a retrospective evaluation in a series of 120 patients.

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Fauser, Susanne; Huppertz, Hans-Juergen; Bast, Thomas; Strobl, Karl; Pantazis, Georgios; Altenmueller, Dirk-Matthias; Feil, Bertram; Rona, Sabine; Kurth, Christoph; Rating, Dietz; Korinthenberg, Rudolf; Steinhoff, Bernhard J; Volk, Benedikt; Schulze-Bonhage, Andreas

2006-07-01

Focal cortical dysplasias (FCDs) are increasingly diagnosed as a cause of symptomatic focal epilepsy in paediatric and adult patients. However, little is known about the clinical characteristics of epilepsy in these patients. In order to elucidate the clinical characteristics of their epilepsy, 120 pharmacoresistant patients including children and adults with histologically proven FCD were studied retrospectively. Age at seizure onset was analysed in the total group and compared between subgroups with different localization and different histological subtypes of FCD. The role of febrile seizures with respect to dual pathology was investigated. Seizure semiology was analysed focusing on initial seizure type and change of seizure semiology during the course of disease. Finally, transient responsiveness to antiepileptic drug therapy was studied. In the majority of patients, epilepsy began in the first 5 years of life. However, onset of epilepsy could also occur in the second or third decade until the age of 60. Age at epilepsy onset was not significantly different between temporal, extratemporal and multilobar localization of FCD. Patients without cytoarchitectural abnormalities (mild malformations of cortical development, FCD 1a according to Palmini) had significantly later epilepsy onset (P= 0.001) compared with patients with cytoarchitectural abnormalities (FCD 1b, 2a and 2b according to Palmini). In patients with additional hippocampal sclerosis (dual pathology) febrile seizures were significantly more frequently reported (P = 0.02) than in patients without dual pathology. Moreover, patients with dual pathology and febrile seizures significantly more frequently presented with severe hippocampal sclerosis (Wyler Grade 3-4) as compared with patients with dual pathology in the absence of febrile seizures (P = 0.03). First observed seizures were mainly tonic or generalized tonic-clonic. A change of seizure semiology seemed to be age-dependent and occurred between the

Temporal Lobe Epilepsy in Children

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Nickels, Katherine C.; Wong-Kisiel, Lily C.; Moseley, Brian D.; Wirrell, Elaine C.

2012-01-01

The temporal lobe is a common focus for epilepsy. Temporal lobe epilepsy in infants and children differs from the relatively homogeneous syndrome seen in adults in several important clinical and pathological ways. Seizure semiology varies by age, and the ictal EEG pattern may be less clear cut than what is seen in adults. Additionally, the occurrence of intractable seizures in the developing brain may impact neurocognitive function remote from the temporal area. While many children will respond favorably to medical therapy, those with focal imaging abnormalities including cortical dysplasia, hippocampal sclerosis, or low-grade tumors are likely to be intractable. Expedient workup and surgical intervention in these medically intractable cases are needed to maximize long-term developmental outcome. PMID:22957247

A young infant with musicogenic epilepsy.

Science.gov (United States)

Lin, Kuang-Lin; Wang, Huei-Shyong; Kao, Pan-Fu

2003-05-01

Musicogenic epilepsy is a relatively rare form of epilepsy. In its pure form, it is characterized by epileptic seizures that are provoked exclusively by listening to music. The usual type of seizure is partial complex or generalized tonic-clonic. Precipitating factors are quite specific, such as listening to only one composition or the actual playing of music on an instrument. However, simple sound also can be a trigger. We report a 6-month-old infant with musicogenic epilepsy. She manifested right-sided focal seizures with occasional generalization. The seizures were frequently triggered by loud music, especially that by the Beatles. The interictal electroencephalography results were normal. Ictal spikes were present throughout the left temporal area during continuous electroencephalograpic monitoring. Brain magnetic resonance imaging results were normal, whereas single-photon emission computed tomography of the brain revealed hypoperfusion of the left temporal area. The young age and epileptogenic left temporal lobe lesion in this patient with musicogenic epilepsy were unusual characteristics. Theoretically, three levels of integration are involved in music processing in the brain. The involved integration of this infant's brain may be the sensory level rather than the emotional level. Nevertheless, the personal musicality and musical style of the Beatles might play an important role in this patient's epilepsy.

Definition and classification of epilepsy. Classification of epileptic seizures 2016

Directory of Open Access Journals (Sweden)

K. Yu. Mukhin

2017-01-01

Full Text Available Epilepsy is one of the most common neurological diseases, especially in childhood and adolescence. The incidence varies from 15 to 113 cases per 100 000 population with the maximum among children under 1 year old. The prevalence of epilepsy is high, ranging from 5 to 8 cases (in some regions – 10 cases per 1000 children under 15 years old. Classification of the disease has great importance for diagnosis, treatment and prognosis. The article presents a novel strategy for classification of epileptic seizures, developed in 2016. It contains a number of brand new concepts, including a very important one, saying that some seizures, previously considered as generalized or focal only, can be, in fact, both focal and generalized. They include tonic, atonic, myoclonic seizures and epileptic spasms. The term “secondarily generalized seizure” is replace by the term “bilateral tonic-clonic seizure” (as soon as it is not a separate type of epileptic seizures, and the term reflects the spread of discharge from any area of cerebral cortex and evolution of any types of focal seizures. International League Against Epilepsy recommends to abandon the term “pseudo-epileptic seizures” and replace it by the term “psychogenic non-epileptic seizures”. If a doctor is not sure that seizures have epileptic nature, the term “paroxysmal event” should be used without specifying the disease. The conception of childhood epileptic encephalopathies, developed within this novel classification project, is one of the most significant achievements, since in this case not only the seizures, but even epileptiform activity can induce severe disorders of higher mental functions. In addition to detailed description of the new strategy for classification of epileptic seizures, the article contains a comprehensive review of the existing principles of epilepsy and epileptic seizures classification.

Focal status epilepticus as a manifestation of idiopathic hypertrophic cranial pachymeningitis.

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Navalpotro-Gómez, Irene; Vivanco-Hidalgo, Rosa María; Cuadrado-Godia, Elisa; Medrano-Martorell, Santiago; Alameda-Quitllet, Francisco; Villalba-Martínez, Gloria; Roquer, Jaume

2016-08-15

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is an uncommon disease of unknown etiology characterized by thickening of the cerebral dura mater with possible associated inflammation. The most frequently described clinical symptoms include headache, cranial nerve palsy, and cerebellar dysfunction. Epilepsy and/or status epilepticus as main presentation is very uncommon. Two consecutive cases are presented of patients manifesting focal status epilepticus secondary to IHCP, with clinical, laboratory [blood test and cerebrospinal fluid (CSF) analysis], neuroradiologic [magnetic resonance imaging (MRI) at 3 Tesla and digital subtraction angiography (DSA)], and therapeutic data. One patient underwent meningeal biopsy; pathology findings are also included. Corticosteroid therapy resulted in clinical improvement in both cases, and neuroimaging showed decreased abnormal morphology, compared to initial findings. In the diagnostic approach to focal status epilepticus or epilepsy, IHCP must be considered a potential, although extremely infrequent, cause. Anti-inflammatory treatment is an effective addition to antiepileptic drug therapy in patients with IHCP. Copyright © 2016 Elsevier B.V. All rights reserved.

Memory Functioning in Children with Epilepsy: Frontal Lobe Epilepsy, Childhood Absence Epilepsy, and Benign Epilepsy with Centrotemporal Spikes

OpenAIRE

Lopes, Ana Filipa; Monteiro, José Paulo; Fonseca, Maria José; Robalo, Conceição; Simões, Mário Rodrigues

2014-01-01

Specific cognitive deficits have been identified in children with epilepsy irrespective of results on intelligence tests. Memory deficits are traditionally attributed to temporal lobe epilepsy, whereas the impact of frontal lobe epilepsy on memory functions has remained controversial. The aim of this study was the examination of memory abilities in other childhood common epilepsy syndromes (frontal lobe epilepsy (FLE), childhood absence epilepsy (CAE), and benign epilepsy with centrotemporal ...

Combined study of 99mTc-HMPAO SPECT and computerized electroencephalographic topography (CET) in patients with medically refractory complex partial epilepsy

International Nuclear Information System (INIS)

Rodrigues, M.; Botelho, M.M.; Fonseca, A.T.; Peter, J.P.; Pimentel, T.; Vieira, M.R.

1996-01-01

For successful surgery for drug-resistant partial epilepsy the site of the seizure focus needs to be known exactly. The purpose of this study was to compare the evaluation of the regional cerebral blood flow (rCBF) (localization and degree of disturbances) by 99m Tc-hexamethylpropylene-amineoxime (HMPAO) single photon emission computed tomography (SPECT) with computerized electroencephalographic topography (CET) and transmission computed X-ray tomography (CT) in partial epilepsy. The study included 20 patients with medically refractory complex partial seizures. Of the 20 patients included, 15 were studied interictally, four ictally and one in both states, interictally and ictally. 99m Tc-HMPAO SPECT detected rCBF changes in 95% of the patients. Interictal studies demonstrated focal areas of hypoperfusion in 93% of the patients. Ictal studies demonstrated an area of hyperperfusion in all patients. Blood flow disturbances in deeper structures of the brain, such as basal ganglia, could be detected. The areas with abnormal 99m Tc-HMPAO uptake were concordant, in localization, with CET in 85% of the patients. Abnormal data with CT scans were found in only 45% of the patients. Focal lesions were found in 20% of the patients by CT scans. 99m Tc-HMPAO SPECT combined with CET may be a useful screening procedure prior to referral for invasive diagnostic procedures in future management of patients with medically refractory complex partial seizures. (author)

Epileptiform discharges in EEG and seizure risk in adolescent children of women with epilepsy.

Science.gov (United States)

Samuel, Joseph; Jose, Manna; Nandini, V S; Thomas, Sanjeev V

2017-09-01

We aimed to study the epileptiform discharges (ED) and seizure risk in EEG of 12-18-year-old children of women with epilepsy (WWE). Children of WWE who were prospectively followed up in the Kerala registry of epilepsy and pregnancy (KREP), aged 12-16years (n=92; males 48, females 44) underwent clinical evaluation and a 30-min digital 18-channel EEG. The EEG showed epileptiform discharges in 13 children (5 males and 8 females). The EDs were generalized in 9 and focal in 4 (occipital 2, frontal 1, and centroparietal 1). They had significantly higher risk of ED (odds ratio 4.02, 95% CI 1.04-15.51) when compared to published prevalence of ED in healthy children. There were 2 children with epilepsy (one with localization-related epilepsy and the other generalized epilepsy). The children under study had a trend towards higher prevalence of epilepsy (odds ratio 3.39, 95% CI 0.82-13.77) when compared to age specific prevalence of epilepsy from community surveys in same region. Children of WWE showed increased risk of ED in EEG and trend towards increased seizure risk when compared to healthy children. Copyright © 2017 Elsevier Inc. All rights reserved.

Epileptic Networks in Focal Cortical Dysplasia Revealed Using Electroencephalography–Functional Magnetic Resonance Imaging

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Thornton, Rachel; Vulliemoz, Serge; Rodionov, Roman; Carmichael, David W; Chaudhary, Umair J; Diehl, Beate; Laufs, Helmut; Vollmar, Christian; McEvoy, Andrew W; Walker, Matthew C; Bartolomei, Fabrice; Guye, Maxime; Chauvel, Patrick; Duncan, John S; Lemieux, Louis

2011-01-01

Objective Surgical treatment of focal epilepsy in patients with focal cortical dysplasia (FCD) is most successful if all epileptogenic tissue is resected. This may not be evident on structural magnetic resonance imaging (MRI), so intracranial electroencephalography (icEEG) is needed to delineate the seizure onset zone (SOZ). EEG-functional MRI (fMRI) can reveal interictal discharge (IED)-related hemodynamic changes in the irritative zone (IZ). We assessed the value of EEG-fMRI in patients with FCD-associated focal epilepsy by examining the relationship between IED-related hemodynamic changes, icEEG findings, and postoperative outcome. Methods Twenty-three patients with FCD-associated focal epilepsy undergoing presurgical evaluation including icEEG underwent simultaneous EEG-fMRI at 3T. IED-related hemodynamic changes were modeled, and results were overlaid on coregistered T1-weighted MRI scans fused with computed tomography scans showing the intracranial electrodes. IED-related hemodynamic changes were compared with the SOZ on icEEG and postoperative outcome at 1 year. Results Twelve of 23 patients had IEDs during recording, and 11 of 12 had significant IED-related hemodynamic changes. The fMRI results were concordant with the SOZ in 5 of 11 patients, all of whom had a solitary SOZ on icEEG. Four of 5 had >50% reduction in seizure frequency following resective surgery. The remaining 6 of 11 patients had widespread or discordant regions of IED-related fMRI signal change. Five of 6 had either a poor surgical outcome (<50% reduction in seizure frequency) or widespread SOZ precluding surgery. Interpretation Comparison of EEG-fMRI with icEEG suggests that EEG-fMRI may provide useful additional information about the SOZ in FCD. Widely distributed discordant regions of IED-related hemodynamic change appear to be associated with a widespread SOZ and poor postsurgical outcome. ANN NEUROL 2011 PMID:22162063

Temporal anteroinferior encephalocele: An underrecognized etiology of temporal lobe epilepsy?

Science.gov (United States)

Saavalainen, Taavi; Jutila, Leena; Mervaala, Esa; Kälviäinen, Reetta; Vanninen, Ritva; Immonen, Arto

2015-10-27

To report the increasing frequency with which temporal anteroinferior encephalocele is a cause of adult temporal lobe epilepsy, to illustrate the clinical and imaging characteristics of this condition, and to report its surgical treatment in a series of 23 adult patients. Epilepsy patients diagnosed with temporal anteroinferior encephalocele from January 2006 to December 2013 in a national epilepsy reference center were included in this noninterventional study. Twenty-three epilepsy patients (14 female, mean age 43.8 years) were diagnosed with temporal anteroinferior encephalocele in our institute. Thirteen patients had ≥2 encephaloceles; 7 cases presented bilaterally. The estimated frequency of this condition was 0.3% among MRI examinations performed due to newly diagnosed epilepsy (n = 6) and 1.9% among drug-resistant patients referred to our center (n = 17). Nine patients with local encephalocele disconnection (n = 4) or anterior temporal lobectomy and amygdalohippocampectomy (n = 5) have become seizure-free (Engel 1) for a mean 2.8 years (range 3 months-6.2 years) of follow-up. Three patients with local encephalocele disconnection were almost seizure-free or exhibited worthwhile improvement. Histologically, all 12 surgical patients had gliosis at the base of the encephalocele; some had cortical laminar disorganization (n = 5) or mild hippocampal degeneration (n = 1). The possibility of a temporal encephalocele should be considered when interpreting MRI examinations of patients with medically intractable focal epilepsy. These patients can significantly benefit from unitemporal epilepsy surgery, even in cases with bilateral encephaloceles. © 2015 American Academy of Neurology.

Survival in 76 cats with epilepsy of unknown cause: a retrospective study.

Science.gov (United States)

Szelecsenyi, Arlette Cornelia; Giger, Urs; Golini, Lorenzo; Mothersill, Ian; Torgerson, Paul R; Steffen, Frank

2017-11-01

Survival of cats with epilepsy of unknown cause (EUC) has not been reported. Seizure semiology and its relationship to treatment outcome and survival was studied in a population of 76 cats. A questionnaire for seizure semiology was developed based on experimental data. Seizure semiology was characterised by owner interviews at least one year after discharge. Seizures were classified as (1) primary generalised and (2) focal without and (3) with secondary generalisation. Median age at seizure onset was four (range 0.3-18) years. One-third of cats with EUC presented with primary generalised seizures and 78 per cent of those with initially focal seizures progressed to secondary generalised seizures. Clinical signs of generalised seizures included sudden onset of loss of consciousness and tonic-clonic seizures, while cats with focal seizures had unilateral signs. Antiepileptic drug (AED) therapy was initiated in 62 cats. Complete remission rate was 42 per cent and the median survival time was 3.2 (range 1-11) years with or without AED, and 91 per cent were still alive at the time of interview. Neither semiology nor seizure type predicted survival, response to treatment and outcome in cats with EUC. A seizure-free status of more than 12 months was observed in 79 per cent of cats without AED. © British Veterinary Association (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Psychiatric comorbidity in children and youth with epilepsy: An association with executive dysfunction?

Science.gov (United States)

Alfstad, Kristin Å; Torgersen, Halvor; Van Roy, Betty; Hessen, Erik; Hansen, Berit Hjelde; Henning, Oliver; Clench-Aas, Jocelyne; Mowinckel, Petter; Gjerstad, Leif; Lossius, Morten I

2016-03-01

Psychopathology in children and youth with epilepsy has previously been related to executive dysfunction, but the nature of the association is uncertain. We sought to explore risk factors for psychiatric disorders in children and youth with epilepsy, with emphasis on executive dysfunction, along with seizure-related and psychosocial factors. The cohort consisted of one hundred and one consecutive patients aged 10-19 years with focal (n=52) or genetic generalized (n=49) epilepsy. All were screened for psychiatric symptoms, using part of an extensive questionnaire, the Strengths and Difficulties Questionnaire (SDQ) for both patients and their parents. Participants scoring in the borderline or abnormal range on the SDQ received a psychiatric interview (Kiddie-SADS-PL). All participants underwent a neuropsychological examination, and those with general cognitive abilities (IQ)epilepsy-related or psychosocial factors were not significantly associated with psychiatric disorders. Multiple factors are associated with psychiatric problems in children and youth with epilepsy. In this study, executive dysfunction, male gender, and early epilepsy onset were independent risk factors for having a psychiatric disorder. An evaluation of psychiatric and cognitive problems is important to enable a positive long-term outcome in childhood epilepsy. Copyright © 2016 Elsevier Inc. All rights reserved.

Bicuculline methiodide in the blood-brain barrier-epileptogen model of epilepsy

Energy Technology Data Exchange (ETDEWEB)

Remler, M.P.; Marcussen, W.H.

Focal epilepsy can be produced by a blood-brain barrier (BBB)-excluded systemic convulsant (penicillin, folic acid, etc.) in the presence of a focal BBB lesion. Bicuculline methiodide, a gamma-aminobutyric acid blocking epileptogen, crosses the normal BBB of rats poorly and produces no consistent abnormality behaviorally or on EEG at 36 mg/kg. When the BBB is opened in 0.25 ml of cortex by 6,000 rad of alpha particles, by a pin trauma lesion, or by a heat lesion, the rats are normal clinically and on EEG. When these lesioned rats are challenged with bicuculline methiodide, 36 mg/kg, an intense, highly localized epileptiform discharge results that begins approximately 20 min after injection and lasts 30-90 min. The plausibility and experimental utility of the BBB-epileptogen model of epilepsy are enhanced by these observations.

Clinical characteristics, risk factors and pre-surgical evaluation of post-infectious epilepsy.

Science.gov (United States)

Sellner, J; Trinka, E

2013-03-01

Epilepsy is a frequent complication of central nervous system (CNS) infections. Post-infectious epilepsy is commonly refractory to medical treatment and plays a pivotal role for the poor long-term outcome of CNS infections. To provide an overview of clinical characteristics and risk factors of seizures associated with CNS infections. In addition, to summarize the state of the art of anticonvulsive treatment and the pre-surgical evaluation process in refractory cases. A comprehensive literature search for articles published between January 1970 and December 2011 was carried out. The occurrence of seizures during the acute course of meningitis, encephalitis and brain abscess is the main risk factor for the development of post-infectious epilepsy. There is a shortage of trials evaluating the efficacy of prophylactic and symptomatic treatment during the course of acute infection. Moreover, there are no randomized-controlled trials studying anticonvulsive drugs and their combinations for the management of post-infectious epilepsy. In a selected group of patients, however, medically refractory focal epilepsy is potentially curable by surgery. Further studies are required to improve the pathogenetic understanding of post-infectious epilepsy in order to develop preventive measures as well as to evaluate additional medical and surgical treatment strategies for the patients currently not considered for surgery. © 2012 The Author(s) European Journal of Neurology © 2012 EFNS.

Social cognition dysfunctions in patients with epilepsy: Evidence from patients with temporal lobe and idiopathic generalized epilepsies.

Science.gov (United States)

Realmuto, Sabrina; Zummo, Leila; Cerami, Chiara; Agrò, Luigi; Dodich, Alessandra; Canessa, Nicola; Zizzo, Andrea; Fierro, Brigida; Daniele, Ornella

2015-06-01

Despite an extensive literature on cognitive impairments in focal and generalized epilepsy, only a few number of studies specifically explored social cognition disorders in epilepsy syndromes. The aim of our study was to investigate social cognition abilities in patients with temporal lobe epilepsy (TLE) and in patients with idiopathic generalized epilepsy (IGE). Thirty-nine patients (21 patients with TLE and 18 patients with IGE) and 21 matched healthy controls (HCs) were recruited. All subjects underwent a basic neuropsychological battery plus two experimental tasks evaluating emotion recognition from facial expression (Ekman-60-Faces test, Ek-60F) and mental state attribution (Story-based Empathy Task, SET). In particular, the latter is a newly developed task that assesses the ability to infer others' intentions (i.e., intention attribution - IA) and emotions (i.e., emotion attribution - EA) compared with a control condition of physical causality (i.e., causal inferences - CI). Compared with HCs, patients with TLE showed significantly lower performances on both social cognition tasks. In particular, all SET subconditions as well as the recognition of negative emotions were significantly impaired in patients with TLE vs. HCs. On the contrary, patients with IGE showed impairments on anger recognition only without any deficit at the SET task. Emotion recognition deficits occur in patients with epilepsy, possibly because of a global disruption of a pathway involving frontal, temporal, and limbic regions. Impairments of mental state attribution specifically characterize the neuropsychological profile of patients with TLE in the context of the in-depth temporal dysfunction typical of such patients. Impairments of socioemotional processing have to be considered as part of the neuropsychological assessment in both TLE and IGE in view of a correct management and for future therapeutic interventions. Copyright © 2015 Elsevier Inc. All rights reserved.

Prevalance and characteristics of epilepsy in the Belgian shepherd variants Groenendael and Tervueren born in Denmark 1995-2004

DEFF Research Database (Denmark)

Berendt, Mette; Gulløv, Christina Hedal; Christensen, Stine Louise Krogh

2008-01-01

1995 and 2004. Furthermore, it was the intention to describe the clinical manifestation (seizure types and phenomenology) of epilepsy and to identify risk factors for euthanasia once the dog was diagnosed as having epilepsy. METHODS: All owners of Groenendael and Tervueren dogs born between January...... seizures. In four percent seizures were unclassifiable. The most commonly reported focal seizure phenomenology included ataxia, crawling, swaying, fearful behavior, salivation, excessive attention seeking and disorientation. In 16% of the cases, epilepsy led to euthanasia. Intact dogs with epilepsy had...

Ictal 18F-FDG PET/MRI in a Patient With Cortical Heterotopia and Focal Epilepsy.

Science.gov (United States)

Calabria, Ferdinando F; Cascini, Giuseppe Lucio; Gambardella, Antonio; Labate, Angelo; Cherubini, Andrea; Gullà, Domenico; Tafuri, Benedetta; Sabatini, Umberto; Vescio, Virginia; Quattrone, Aldo

2017-10-01

A 19-year-old man with epilepsy underwent ictal F-FDG PET/MRI, showing a 5 mm heterotopic nodule in the periventricular white matter, adjacent to the frontal horn of the left lateral ventricle (SUVmax, 5.5; glucidic cerebral metabolic rate, 0.317 μmol/mL). A repeated F-FDG PET/MRI, during seizure freedom, showed, at visual analysis, subtle decrease of the nodule metabolism. SUVmax and glucidic cerebral metabolic rate were clearly reduced to 3.7 and 0.226, respectively. Ictal F-FDG PET/MRI could be useful in epilepsy because of the added value of SUVmax and cerebral metabolic rate of glucose analysis to understand the relationship between heterotopy and epilepsy.

Surgical treatment of polymicrogyria-related epilepsy.

Science.gov (United States)

Cossu, Massimo; Pelliccia, Veronica; Gozzo, Francesca; Casaceli, Giuseppe; Francione, Stefano; Nobili, Lino; Mai, Roberto; Castana, Laura; Sartori, Ivana; Cardinale, Francesco; Lo Russo, Giorgio; Tassi, Laura

2016-12-01

The role of resective surgery in the treatment of polymicrogyria (PMG)-related focal epilepsy is uncertain. Our aim was to retrospectively evaluate the seizure outcome in a consecutive series of patients with PMG-related epilepsy who received, or did not receive, surgical treatment, and to outline the clinical characteristics of patients who underwent surgery. We evaluated 64 patients with epilepsy associated with magnetic resonance imaging (MRI)-documented PMG. After presurgical evaluation, 32 patients were excluded from surgical treatment and 32 were offered surgery, which was declined by 8 patients. Seizure outcome was assessed in the 40 nonsurgical and 24 surgical patients. Of 40 nonsurgical patients, 8 (20%) were seizure-free after a mean follow-up of 91.7 ± (standard deviation) 59.5 months. None of the eight patients who declined surgical treatment was seizure-free (mean follow-up: 74.3 ± 60.6 months). These seizure outcomes differ significantly (p = 0.000005 and p = 0.0003, respectively) from that of the 24 surgical patients, 18 of whom (66.7%) were Engel's class I postoperatively (mean follow-up: 66.5 ± 54.0 months). Of the eight patients excluded from surgery for seizure control at first visit, two had seizure recurrence at last contact. At last contact, antiepileptic drugs (AEDs) had been withdrawn in 6 of 24 surgical and in one of 40 nonsurgical cases (p = 0.0092). The present study indicates that, at least in a subset of adequately selected patients with PMG-related epilepsy, surgery may provide excellent seizure outcomes. Furthermore, it suggests that surgery is superior to AEDs for achieving seizure freedom in these cases. Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.

Epilepsy and non-organic non-affective psychosis. National epidemiologic study

DEFF Research Database (Denmark)

Bredkjaer, S R; Mortensen, P B; Parnas, Josef

1998-01-01

BACKGROUND: This study tests the hypothesis that epilepsy increases the risk of developing schizophrenia and other non-affective functional psychoses using a nationwide sample of people with epilepsy. METHOD: A record linkage study between a sample from the National Patient Register, consisting...... of 67,116 people with epilepsy, and the Danish Psychiatric Register identified all people with non-affective psychoses with onset after the first epilepsy diagnosis. The relation between risk of psychiatric disorder in people with epilepsy and the general Danish population was estimated. RESULTS...... of an association between epilepsy and the risk of subsequent non-affective psychosis....

PECULIARITIES OF TREATMENT OF EPILEPSY AT GIRLS AND WOMEN

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O. A. Pylaeva

2015-01-01

Full Text Available The epilepsy treatment is to be based on existing general principles and standards of therapy with differential approach to each patient. Besides peculiarities of treatment of different types of seizures and forms of epilepsy there are also differential approaches to special groups of patients. To one of such groups are referred to women of reproductive age. These patients are referred to special group of risk due to the development of certain side effects of antiepileptic drugs (АED. This article focuses in details on peculiarities of treatment of women of reproductive age with epilepsy with accent made on tolerability and safety of the antiepileptic therapy. It is necessary to take into consideration, that at women neuroendocrinal disorders can be caused both by the disease itself – epilepsy (in such case disorders depend on the starting age, form of epilepsy, focal localization, duration of disorder and other factors, referred to the disease, as well as by the undertaken therapy. The articlehereunder considers only issues, referred to the treatment, i. e. AED side effects and its input in the decrease of life quality of women with epilepsy. As women’s reproductive function starts forming long ago before childbearing age, it is necessary for this category to comprise not only women and adolescents, but girls as well. Notwithstanding the fact that so called benign forms of epilepsy pass before the pubescence period (idiopathic focal epilepsies, several forms of idiopathic generalized epilepsy, in many cases the epilepsy, which has started in childhood, continues in the adult age as well. In the same time there can be possible remote negative consequences of the antiepileptic therapy, which can show at a woman of a reproductive age. The data, given in the article, witnesses the need of the right AED selection at women of reproductive age, suffering from epilepsy. The AED should be selected not only depending on the form of the

Epilepsy secondary to Menkes' disease: five cases report and review of literature

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Pei-yuan ZHANG

2014-12-01

Full Text Available Objective To study the clinical features of patients with Menkes' disease (MD that initiate from seizures, and to reinforce the knowledge of etiological diagnosis of secondary epilepsy.  Methods The clinical and laboratory features of 5 MD patients who were admitted from December 1992 to March 2014 were retrospectively analyzed.  Results All cases were male infants including two brothers, and the age of onset was 1-5 months after birth. Their clinical manifestations started from tonic (N = 3 and focal clonic (N = 2 seizures. The focal clonic seizures became more frequent and progressively worsened. Four cases showed abnormal background EEG rhythm; 3 cases revealed interictal focal or multifocal epileptiform discharges over occipital region predominantly; 2 cases revealed epileptic discharge in fit period EEG. All the cases manifested severe mental retardation accompanied with light complexion and curly hair. Low serum copper and ceruloplasmin levels were found in 4 cases and lead to the diagnosis of MD. The exceptional one was made clinical diagnosis of MD due to his typical manifestation and the diagnosis of his elder brother. Three cases received MRI examination, which showed extensive cerebral atrophy and symmetric encephalomalacia foci. One case revealed tortuosity of main artery and diminution of distal artery by MRA. One case was treated with phenobarbital, 2 cases with topiramate, one case with topiramate and levetiracetam and clonazepam. One case died of respiratory failure at 24 h after admission. The other 4 cases were followed up for 1-9 months after being discharged, 3 of whom died, and the left one converted to intractable infantile spasms and severe mental retardation, with interictal EEG changing to hypsarrhythmia.  Conclusions The onset of patients with epilepsy secondary to MD often occurs in early infancy, manifesting tonic and focal clonic seizures predominantly with interictal epileptiform discharges over the posterior

Autistic spectrum disorder: evaluating a possible contributing or causal role of epilepsy.

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Deonna, Thierry; Roulet, Eliane

2006-01-01

The onset of epilepsy in brain systems involved in social communication and/or recognition of emotions can occasionally be the cause of autistic symptoms or may aggravate preexisting autistic symptoms. Knowing that cognitive and/or behavioral abnormalities can be the presenting and sometimes the only symptom of an epileptic disorder or can even be caused by paroxysmal EEG abnormalities without recognized seizures, the possibility that this may apply to autism has given rise to much debate. Epilepsy and/or epileptic EEG abnormalities are frequently associated with autistic disorders in children but this does not necessarily imply that they are the cause; great caution needs to be exercised before drawing any such conclusions. So far, there is no evidence that typical autism can be attributed to an epileptic disorder, even in those children with a history of regression after normal early development. Nevertheless, there are several early epilepsies (late infantile spasms, partial complex epilepsies, epilepsies with CSWS, early forms of Landau-Kleffner syndrome) and with different etiologies (tuberous sclerosis is an important model of these situations) in which a direct relationship between epilepsy and some features of autism may be suspected. In young children who primarily have language regression (and who may have autistic features) without evident cause, and in whom paroxysmal focal EEG abnormalities are also found, the possible direct role of epilepsy can only be evaluated in longitudinal studies.

Characterizing older adult patients suffering from epilepsy in two hospitals in Bogotá (Colombia

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Gutiérrez-Álvarez AM

2011-12-01

Full Text Available Epilepsy’s overall prevalence in Colombia is 1.13%. Its prevalence in patients aged over 65 could be around 1.5%. Objective: describe demographic and clinical characteristics of patients older than 65 years of age with epilepsy. Materials and methods: A cross-sectional descriptive study was carried out in two high complexity hospitals in Bogotá, Colombia during 2005-2008. Demographic data were compiled and patients characterized regarding the type, frequency and diagnosis of seizures (based on ILAE classification, probable etiology, having a family background of epilepsy, and current pharmacological management. Results: 211 clinical histories were reviewed and 179 of them selected. Mean patient age was 75 (65-98 and average age at onset of epilepsy was 67.5 (7-93. 84% of the seizures were classified as being focal. The most frequently occurring diagnosis was symptomatic focal epilepsy (94.4%. 74 cases (41.3% had an etiological diagnosis. The most important cause was cerebrovascular disease (61 patients. First generation anti-epileptic drugs were the most used ones (99%. 81/104 patients were found not to be free from epileptic episodes. Conclusions: Most seizures have a partial beginning, resulting from symptomatic partial epilepsy as a consequence of a vascular lesion. Pharmacological treatment must be considered following the first seizure. Treatment with second generation anti-epileptic drugs such as Lamotrigine, Gabapentin, Levetiracetam and Topiramate must be begun for minimizing secondary effects and low doses must be maintained from the start of treatment. Costs may limit the use of the above antiepileptic drugs, in such cases Phenytoin and Carbamazepine may be used with extreme caution.

Clinical characteristics and treatment responses in new-onset epilepsy in the elderly.

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Tanaka, Akihiro; Akamatsu, Naoki; Shouzaki, Taisaku; Toyota, Tomoko; Yamano, Mitsuhiko; Nakagawa, Masanori; Tsuji, Sadatoshi

2013-11-01

Epidemiologic studies have shown that the incidence of epilepsy is the highest in the elderly population. Because the elderly constitutes the most rapidly growing population, epilepsy in this group is an important health issue worldwide. To identify the characteristics of epilepsy in the elderly, we reviewed our experience at a tertiary referral center in Japan. We searched all electronic medical records of the past 6 years at the epilepsy clinic of the hospital affiliated to our University-affiliated hospital. We defined an elderly person as an individual aged 65 years and above. All patients underwent history and physical examinations, 3T magnetic resonance imaging and/or computer tomography, and electroencephalogram (EEG). The diagnosis of epilepsy, age of onset, etiology, and antiepileptic medication were recorded. We identified 70 patients who developed epilepsy after the age of 65 years. The mean age of seizure onset was 73.1 years and 52.9% patients were males. Complex partial seizures (CPS) without secondarily generalization (n=33, 47.1%) were most frequent. The most frequent diagnosis was temporal lobe epilepsy (n=50, 71.4%). Etiological diagnosis was possible in nearly 50% patients, including those with cerebrovascular disease. A clear cause of epilepsy was not found (i.e., non-lesional epilepsy) in 52.8% patients. Interictal EEG revealed focal epileptiform discharges in 72.9% (n=51) patients. Of the 54 patients who were followed more than 1 year, 42 patients (77.8%) were on antiepileptic monotherapy and 52 patients (96.3%) had been seizure-free for more than 1 year. The most frequent diagnosis in our cohort of elderly persons with new-onset epilepsy was temporal lobe epilepsy. Non-lesional temporal lobe epilepsy was not uncommon. Epileptogenecity was relatively low in elderly patients and they responded well to antiepileptic medication. Copyright © 2013 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

22q11.2 deletion syndrome lowers seizure threshold in adult patients without epilepsy.

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Wither, Robert G; Borlot, Felippe; MacDonald, Alex; Butcher, Nancy J; Chow, Eva W C; Bassett, Anne S; Andrade, Danielle M

2017-06-01

Previous studies examining seizures in patients with 22q11.2 deletion syndrome (22q11.2DS) have focused primarily on children and adolescents. In this study we investigated the prevalence and characteristics of seizures and epilepsy in an adult 22q11.2DS population. The medical records of 202 adult patients with 22q11.2DS were retrospectively reviewed for documentation of seizures, electroencephalography (EEG) reports, and magnetic resonance imaging (MRI) findings. Epilepsy status was assigned in accordance with 2010 International League Against Epilepsy Classification. Of 202 patients, 32 (15.8%) had a documented history of seizure. Of these 32, 23 (71.8%) had acute symptomatic seizures, usually associated with hypocalcemia and/or antipsychotic or antidepressant use. Nine patients (9/32, 28%; 9/202, 4%) met diagnostic criteria for epilepsy. Two patients had genetic generalized epilepsy; two patients had focal seizures of unknown etiology; two had epilepsy due to malformations of cortical development; in two the epilepsy was due to acquired structural changes; and in one patient the epilepsy could not be further classified. Similarly to children, the prevalence of epilepsy and acute symptomatic seizures in adults with 22q11.2DS is higher than in the general population. Hypocalcemia continues to be a risk factor for adults, but differently from kids, the main cause of seizures in adults with 22q11.2DS is exposure to antipsychotics and antidepressants. Further prospective studies are warranted to investigate how 22q11.2 microdeletion leads to an overall decreased seizure threshold. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

Hyperphosphorylated tau in patients with refractory epilepsy correlates with cognitive decline: a study of temporal lobe resections.

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Tai, Xin You; Koepp, Matthias; Duncan, John S; Fox, Nick; Thompson, Pamela; Baxendale, Sallie; Liu, Joan Y W; Reeves, Cheryl; Michalak, Zuzanna; Thom, Maria

2016-09-01

SEE BERNASCONI DOI101093/AWW202 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Temporal lobe epilepsy, the most prevalent form of chronic focal epilepsy, is associated with a high prevalence of cognitive impairment but the responsible underlying pathological mechanisms are unknown. Tau, the microtubule-associated protein, is a hallmark of several neurodegenerative diseases including Alzheimer's disease and chronic traumatic encephalopathy. We hypothesized that hyperphosphorylated tau pathology is associated with cognitive decline in temporal lobe epilepsy and explored this through clinico-pathological study. We first performed pathological examination on tissue from 33 patients who had undergone temporal lobe resection between ages 50 and 65 years to treat drug-refractory temporal lobe epilepsy. We identified hyperphosphorylated tau protein using AT8 immunohistochemistry and compared this distribution to Braak patterns of Alzheimer's disease and patterns of chronic traumatic encephalopathy. We quantified tau pathology using a modified tau score created specifically for analysis of temporal lobectomy tissue and the Braak staging, which was limited without extra-temporal brain areas available. Next, we correlated tau pathology with pre- and postoperative cognitive test scores and clinical risk factors including age at time of surgery, duration of epilepsy, history of secondary generalized seizures, history of head injury, handedness and side of surgery. Thirty-one of 33 cases (94%) showed hyperphosphorylated tau pathology in the form of neuropil threads and neurofibrillary tangles and pre-tangles. Braak stage analysis showed 12% of our epilepsy cohort had a Braak staging III-IV compared to an age-matched non-epilepsy control group from the literature (8%). We identified a mixture of tau pathology patterns characteristic of Alzheimer's disease and chronic traumatic encephalopathy. We also found unusual patterns of subpial tau deposition, sparing of the hippocampus and

Epilepsy and brain tumors

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ENGLOT, DARIO J.; CHANG, EDWARD F.; VECHT, CHARLES J.

2016-01-01

Seizures are common in patients with brain tumors, and epilepsy can significantly impact patient quality of life. Therefore, a thorough understanding of rates and predictors of seizures, and the likelihood of seizure freedom after resection, is critical in the treatment of brain tumors. Among all tumor types, seizures are most common with glioneuronal tumors (70–80%), particularly in patients with frontotemporal or insular lesions. Seizures are also common in individuals with glioma, with the highest rates of epilepsy (60–75%) observed in patients with low-grade gliomas located in superficial cortical or insular regions. Approximately 20–50% of patients with meningioma and 20–35% of those with brain metastases also suffer from seizures. After tumor resection, approximately 60–90% are rendered seizure-free, with most favorable seizure outcomes seen in individuals with glioneuronal tumors. Gross total resection, earlier surgical therapy, and a lack of generalized seizures are common predictors of a favorable seizure outcome. With regard to anticonvulsant medication selection, evidence-based guidelines for the treatment of focal epilepsy should be followed, and individual patient factors should also be considered, including patient age, sex, organ dysfunction, comorbidity, or cotherapy. As concomitant chemotherapy commonly forms an essential part of glioma treatment, enzyme-inducing anticonvulsants should be avoided when possible. Seizure freedom is the ultimate goal in the treatment of brain tumor patients with epilepsy, given the adverse effects of seizures on quality of life. PMID:26948360

1H MR spectroscopic imaging in patients with MRI-negative extratemporal epilepsy: correlation with ictal onset zone and histopathology

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Krsek, Pavel; Komarek, Vladimir; Hajek, Milan; Dezortova, Monika; Jiru, Filip; Skoch, Antonin; Marusic, Petr; Zamecnik, Josef; Kyncl, Martin; Tichy, Michal

2007-01-01

Proton magnetic resonance spectroscopy ( 1 H MRS) is beneficial in the lateralization of the epileptogenic zone in temporal lobe epilepsy; however, its role in extratemporal and, especially, MRI-negative epilepsy has not been established. This study seeks to verify how 1 H MRS could help in localizing the epileptogenic zone in patients with MRI-negative extratemporal epilepsy. Seven patients (8-23 years) with MRI-negative refractory focal epilepsy were studied using 1 H MRS on a 1.5T MR system. Chemical shift imaging sequence in the transversal plane was directed towards the suspected epileptogenic zone localized by seizure semiology, scalp video/EEG, ictal SPECT and 18 FDG-PET. Spectra were evaluated using the program CULICH, and the coefficient of asymmetry was used for quantitative lateralization. MRS detected lateralization in all patients and was able to localize pathology in five. The most frequent findings were decreased ratios of N-acetylaspartate to choline compounds characterized by increasing choline concentration. The localization of the 1 H MRS abnormality correlated well with ictal SPECT and subdural mapping. In all cases, histopathological analysis revealed MRI-undetected focal cortical dysplasias. 1 H MRS could be more sensitive for the detection of discrete malformations of cortical development than conventional MRI. It is valuable in the presurgical evaluation of patients without MRI-apparent lesions. (orig.)

Altered cortical anatomical networks in temporal lobe epilepsy

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Lv, Bin; He, Huiguang; Lu, Jingjing; Li, Wenjing; Dai, Dai; Li, Meng; Jin, Zhengyu

2011-03-01

Temporal lobe epilepsy (TLE) is one of the most common epilepsy syndromes with focal seizures generated in the left or right temporal lobes. With the magnetic resonance imaging (MRI), many evidences have demonstrated that the abnormalities in hippocampal volume and the distributed atrophies in cortical cortex. However, few studies have investigated if TLE patients have the alternation in the structural networks. In the present study, we used the cortical thickness to establish the morphological connectivity networks, and investigated the network properties using the graph theoretical methods. We found that all the morphological networks exhibited the small-world efficiency in left TLE, right TLE and normal groups. And the betweenness centrality analysis revealed that there were statistical inter-group differences in the right uncus region. Since the right uncus located at the right temporal lobe, these preliminary evidences may suggest that there are topological alternations of the cortical anatomical networks in TLE, especially for the right TLE.

Initial experience in hybrid PET-MRI for evaluation of refractory focal onset epilepsy.

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Shin, Hae W; Jewells, Valerie; Sheikh, Arif; Zhang, Jingwen; Zhu, Hongtu; An, Hongyu; Gao, Wei; Shen, Dinggang; Hadar, Eldad; Lin, Weili

2015-09-01

We aim to evaluate the utility/improved accuracy of hybrid PET/MR compared to current practice separate 3T MRI and PET-CT imaging for localization of seizure foci. In a pilot study, twenty-nine patients undergoing epilepsy surgery evaluation were imaged using PET/MR. This subject group had 29 previous clinical 3T MRI as well as 12 PET-CT studies. Prior clinical PET and MR images were read sequentially while the hybrid PET/MR was concurrently read. The median interval between hybrid PET/MR and prior imaging studies was 5 months (range 1-77 months). In 24 patients, there was no change in the read between the clinical exams and hybrid PET/MR while new anatomical or functional lesions were identified by hybrid PET/MR in 5 patients without significant clinical change. Four new anatomical MR lesions were seen with concordant PET findings. The remaining patient revealed a new abnormal PET lesion without an MR abnormality. All new PET/MR lesions were clinically significant with concordant EEG and/or SPECT results as potential epileptic foci. Our initial hybrid PET-MRI experience increased diagnostic yields for detection of potential epileptic lesions. This may be due to the unique advantage of improved co-registration and simultaneous review of both structural and functional data. Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Benign childhood epilepsy with occipital paroxysms: neuropsychological findings.

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Germanò, Eva; Gagliano, Antonella; Magazù, Angela; Sferro, Caterina; Calarese, Tiziana; Mannarino, Erminia; Calamoneri, Filippo

2005-05-01

Benign childhood epilepsy with occipital paroxysms is classified among childhood benign partial epilepsies. The absence of neurological and neuropsychological deficits has long been considered as a prerequisite for a diagnosis of benign childhood partial epilepsy. Much evidence has been reported in literature in the latest years suggesting a neuropsychological impairment in this type of epilepsy, particularly in the type with Rolandic paroxysms. The present work examines the neuropsychological profiles of a sample of subjects affected by the early-onset benign childhood occipital seizures (EBOS) described by Panayotopulos. The patient group included 22 children (14 males and 8 females; mean age 10.1+/-3.3 years) diagnosed as having EBOS. The patients were examined with a set of tests investigating neuropsychological functions: memory, attention, perceptive, motor, linguistic and academic (reading, writing, arithmetic) abilities. The same instruments have been given to a homogeneous control group as regards sex, age, level of education and socio-economic background. None of the subjects affected by EBOS showed intellectual deficit (mean IQ in Wechsler Full Scale 91.7; S.D. 8.9). Results show a widespread cognitive dysfunction in the context of a focal epileptogenic process in EBOS. In particular, children with EBOS show a significant occurrence of specific learning disabilities (SLD) and other subtle neuropsychological deficits. We found selective dysfunctions relating to perceptive-visual attentional ability (pmemory abilities (psupports the hypothesis that epilepsy itself plays a role in the development of neuropsychological impairment. Supported by other studies that have documented subtle neuropsychological deficits in benign partial epilepsy, we stress the importance of reconsidering its supposed "cognitive benignity", particularly in occipital types.

Clinical features of tuberous sclerosis complex in children with epilepsy

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Dong LI

2014-12-01

Full Text Available Objective To explore the clinical features of tuberous sclerosis complex (TSC in children with epilepsy.  Methods The clinical data of 36 TSC children complicated with epilepsy were analyzed retrospectively.  Results All children had epilepsy as first symptom: 23 cases of seizures happened on age less than one year old (63.89%, 8 cases on age 1-3 years old (22.22%, and 5 cases on age more than 3 years old (13.89% . Main attack types were as follows: 12 cases with focal seizures (33.33% , 5 cases with generalized seizures (13.89% , 7 cases with spasms (19.44% and 12 cases (33.33% with mixed seizures. Mental retardation was found in 19 cases (67.86%. Among 30 cases who received brain MRI examination, all had subependymal nodules, 26 cases (86.67% were complicated with cortical and subcortical nodules, 2 cases (6.67% with subependymal giant cell astrocytoma (SEGA, one case (3.33% with pachygyria. EEG examination showed interictal epileptiform discharges in 34 cases (94.44% , including multifocal discharges in 12 patients (35.29% , generalized discharges in 8 patients (23.53% , focal discharges and hypsarrhythmia in 7 patients (20.59% respectively. Seizures were monitored in 8 patients, and the types included spasms in 4 cases, focal seizures in 3 cases, and myoclonic seizures in one case. Seventeen cases (47.22% took one single antiepileptic drug, 12 cases (33.33% took two drugs, and 7 cases (19.44% took three drugs, while one of them underwent surgical treatment. After 0.50-10 year follow-up, seizures free happened in 7 cases (19.44%, improved in 16 cases (44.44%, and invalid in 13 cases (36.11%.  Conclusions Epilepsy is the most common nervous system performance in children TSC, and most seizures begin to happen in infants. The main types of onset are partial seizures and spasms. Most of the patients get improved with antiepileptic drugs. doi: 10.3969/j.issn.1672-6731.2014.12.011

Epilepsy in Qatar: Causes, treatment, and outcome.

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Haddad, Naim; Melikyan, Gayane; Al Hail, Hassan; Al Jurdi, Ayman; Aqeel, Faten; Elzafarany, Abdullah; Abuhadra, Nour; Laswi, Mujahed; Alsamman, Yasser; Uthman, Basim; Deleu, Dirk; Mesraoua, Boulenouar; Alarcon, Gonzalo; Azar, Nabil; Streletz, Leopold; Mahfoud, Ziyad

2016-10-01

Qatar is a small country on the Eastern coast of the Arabian Peninsula. Its population is a unique mixture of native citizens and immigrants. We aimed to describe the features of epilepsy in Qatar as such information is virtually lacking from the current literature. We summarized information retrospectively collected from 468 patients with epilepsy seen through the national health system adult neurology clinic. Epilepsy was classified as focal in 65.5% of the cases and generalized in 23%. Common causes of epilepsy were as follows: stroke (9%), hippocampal sclerosis (7%), infections (6%), and trauma (6%). Sixty-six percent of patients were receiving a single antiepileptic drug, with levetiracetam being the most frequently prescribed drug (41% of subjects). When the patients were divided by geographical background, remote infections caused the epilepsy in 15% of Asian patients (with neurocysticercosis accounting for 10%) but only in 1% of Qatari and 3% of Middle East/North African subjects (with no reported neurocysticercosis) (pepilepsy in Qatar. The geographical origin of patients adds to the heterogeneity of this disorder. Neurocysticercosis should be in the etiological differential diagnosis of epilepsy in patients coming from Southeast Asian countries, despite the fact that it is not endemic to Qatar. The choice of antiepileptic drugs is influenced by the availability of individual agents in the patients' native countries but had no bearing on the final seizure outcome. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

Reflex epilepsy: triggers and management strategies

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Okudan ZV

2018-01-01

Full Text Available Zeynep Vildan Okudan,1 Çiğdem Özkara2 1Department of Neurology, Bakirkoy Dr Sadi Konuk Education and Research Hospital, 2Department of Neurology and Clinical Neurophysiology, Cerrahpasa Faculty of Medicine, University of Istanbul, Istanbul, Turkey Abstract: Reflex epilepsies (REs are identified as epileptic seizures that are consistently induced by identifiable and objective-specific triggers, which may be an afferent stimulus or by the patient’s own activity. RE may have different subtypes depending on the stimulus characteristic. There are significant clinical and electrophysiologic differences between different RE types. Visual stimuli-sensitive or photosensitive epilepsies constitute a large proportion of the RE and are mainly related to genetic causes. Reflex epilepsies may present with focal or generalized seizures due to specific triggers, and sometimes seizures may occur spontaneously. The stimuli can be external (light flashes, hot water, internal (emotion, thinking, or both and should be distinguished from triggering precipitants, which most epileptic patients could report such as emotional stress, sleep deprivation, alcohol, and menstrual cycle. Different genetic and acquired factors may play a role in etiology of RE. This review will provide a current overview of the triggering factors and management of reflex seizures. Keywords: seizure, reflex epilepsy, photosensitivity, hot water, reading, thinking

Intraoperative hyperventilation vs remifentanil during electrocorticography for epilepsy surgery - a case report

DEFF Research Database (Denmark)

Kjaer, Troels W; Madsen, F F; Moltke, F B

2010-01-01

different brain regions in the same patient. METHODS: Hyperventilation and ultra short acting opioid remifentanil were used separately as intraoperative precipitatants of seizure patterns, while recording from subdural and intraventricular electrodes in a patient with temporal lobe epilepsy. Two different...... ictal onset zones appeared in response to hyperventilation and remifentanil. Both zones were resected and the patient has remained essentially seizure free for 1 year. Furthermore, this is the first description of hyperventilation used as an intraoperative seizure precipitant in human focal epilepsy....

Emotional Prosody Processing in Epilepsy: Some Insights on Brain Reorganization.

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Alba-Ferrara, Lucy; Kochen, Silvia; Hausmann, Markus

2018-01-01

Drug resistant epilepsy is one of the most complex, multifactorial and polygenic neurological syndrome. Besides its dynamicity and variability, it still provides us with a model to study brain-behavior relationship, giving cues on the anatomy and functional representation of brain function. Given that onset zone of focal epileptic seizures often affects different anatomical areas, cortical but limited to one hemisphere, this condition also let us study the functional differences of the left and right cerebral hemispheres. One lateralized function in the human brain is emotional prosody, and it can be a useful ictal sign offering hints on the location of the epileptogenic zone. Besides its importance for effective communication, prosody is not considered an eloquent domain, making resective surgery on its neural correlates feasible. We performed an Electronic databases search (Medline and PsychINFO) from inception to July 2017 for studies about prosody in epilepsy. The search terms included "epilepsy," "seizure," "emotional prosody," and "vocal affect." This review focus on emotional prosody processing in epilepsy as it can give hints regarding plastic functional changes following seizures (preoperatively), resection (post operatively), and also as an ictal sign enabling the assessment of dynamic brain networks. Moreover, it is argued that such reorganization can help to preserve the expression and reception of emotional prosody as a central skill to develop appropriate social interactions.

Factors affecting the employability in people with epilepsy.

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Wo, Monica Chen Mun; Lim, Kheng Seang; Choo, Wan Yuen; Tan, Chong Tin

2016-12-01

People with epilepsy (PWE) are negatively prejudiced in their ability to work. This study aimed to examine demographic, clinical and psychological factors associated with employability in PWE. This study recruited epilepsy patients from a neurology clinic in Malaysia. Employability was measured using employment ratio, with a ratio ≥90% (ER90) classified as high employability. Basic demographic data such as age, gender, marital status, religion, education level and household income was collected. Clinical measures consisted of age of seizure onset, seizure frequency, type of epilepsy, aura, polytherapy, nocturnal seizures and seizure control. Psychological measures included Work Self-Determination Index (WSDI), Rosenberg Self-Esteem Scale (SES), and Multidimensional Scale of Perceived Social Support (MSPSS). Of 146 PWE, 64.4% had high employability. The participants were predominantly female (52%), Chinese (50.7%), single (50%), having tertiary education (55.5%) and focal epilepsy (72.6%). Clinically, only type of epilepsy was significantly correlated to employability of PWE. Employability of PWE was associated with ability to work (indicated by education level, work performance affected by seizures, ability to travel independently and ability to cope with stress at work) and family overprotection. The high employability group was found to have lower self-perceived stigma (ESS), higher self-determined motivation (WSDI), self-esteem (SES) and perceived social support (MSPSS), than the low employability group. Logistic regression analysis showed that tertiary education level (AOR 3.42, CI: 1.46-8.00), higher self-determination (WSDI, AOR 1.09, CI: 1.012-1.17), lower family overprotection (AOR 0.76, CI: 0.61-0.95), and generalised epilepsy (AOR 4.17, CI: 1.37-12.70) were significant predictors for higher employability in PWE. Ability to work (education level), clinical factor (type of epilepsy) and psychological factor (self-determined motivation and family

Ipsiversive ictal eye deviation in inferioposterior temporal lobe epilepsy-Two SEEG cases report.

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Zhang, Wei; Liu, Xingzhou; Zuo, Lijun; Guo, Qiang; Chen, Qi; Wang, Yongjun

2017-02-21

Versive seizure characterized by conjugate eye movement during epileptic seizure has been considered commonly as one of the most valuable semiological signs for epilepsy localization, especially for frontal lobe epilepsy. However, the lateralizing and localizing significance of ictaleye deviation has been questioned by clinical observation of a series of focal epilepsy studies, including frontal, central, temporal, parietal and occipital epilepsy. Two epileptic cases characterized by ipsiversive eye deviation as initial clinical sign during the habitual epileptic seizures are presented in this paper. The localization of the epileptogenic zone of both of the cases has been confirmed as inferioposterior temporal region by the findings of ictalstereoelectroencephalography (SEEG) and a good result after epileptic surgery. Detailed analysis of the exact position of the key contacts of the SEEG electrodes identified the overlap between the location of the epileptogenic zone and human MT/MST complex, which play a crucial role in the control of smooth pursuit eye movement. Ipsiversive eye deviation could be the initial clinical sign of inferioposterior temporal lobe epilepsy and attribute to the involvement of human MT/MST complex, especially human MST whichwas located on the anterior/dorsal bank of the anterior occipital sulcus (AOS).

Epilepsy after cerebral infection: review of the literature and the potential for surgery.

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Ramantani, Georgia; Holthausen, Hans

2017-06-01

The risk of unprovoked seizures in population-based cohorts of cerebral infection survivors is 7-8% in developed countries, rising to considerably higher rates in resource-poor countries. The main risk factors for epilepsy after cerebral infection, besides acute seizures, are infection-associated brain lesions and status epilepticus during the acute phase. Despite the high prevalence of pharmacoresistant epilepsies after cerebral infections, especially in patients with MRI-identifiable lesions, only a small minority undergoes epilepsy surgery. However, excellent surgical candidates are particularly those with a history of meningitis or encephalitis in early childhood, hippocampal sclerosis on MRI, as well as a history, seizure semiology, and EEG-findings compatible with the diagnosis of a mesial temporal lobe epilepsy syndrome. More challenging are patients with neocortical/extratemporal lobe epilepsies post cerebral infection. Finally, patients with a severe hemispheric injury with contralateral hemiparesis are candidates for hemispherectomy/hemispherotomy. This review attempts to shed some light on this frequent cause of symptomatic focal epilepsy, with an emphasis on the chances offered by epilepsy surgery.

Screening LGI1 in a cohort of 26 lateral temporal lobe epilepsy patients with auditory aura from Turkey detects a novel de novo mutation.

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Kesim, Yesim F; Uzun, Gunes Altiokka; Yucesan, Emrah; Tuncer, Feyza N; Ozdemir, Ozkan; Bebek, Nerses; Ozbek, Ugur; Iseri, Sibel A Ugur; Baykan, Betul

2016-02-01

Autosomal dominant lateral temporal lobe epilepsy (ADLTE) is an autosomal dominant epileptic syndrome characterized by focal seizures with auditory or aphasic symptoms. The same phenotype is also observed in a sporadic form of lateral temporal lobe epilepsy (LTLE), namely idiopathic partial epilepsy with auditory features (IPEAF). Heterozygous mutations in LGI1 account for up to 50% of ADLTE families and only rarely observed in IPEAF cases. In this study, we analysed a cohort of 26 individuals with LTLE diagnosed according to the following criteria: focal epilepsy with auditory aura and absence of cerebral lesions on brain MRI. All patients underwent clinical, neuroradiological and electroencephalography examinations and afterwards they were screened for mutations in LGI1 gene. The single LGI1 mutation identified in this study is a novel missense variant (NM_005097.2: c.1013T>C; p.Phe338Ser) observed de novo in a sporadic patient. This is the first study involving clinical analysis of a LTLE cohort from Turkey and genetic contribution of LGI1 to ADLTE phenotype. Identification of rare LGI1 gene mutations in sporadic cases supports diagnosis as ADTLE and draws attention to potential familial clustering of ADTLE in suggestive generations, which is especially important for genetic counselling. Copyright © 2015 Elsevier B.V. All rights reserved.

Obstructive sleep apnea in epilepsy: a preliminary Egyptian study.

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Shaheen, Hala A; Abd El-Kader, Ann A; El Gohary, Amira M; El-Fayoumy, Neveen M; Afifi, Lamia M

2012-09-01

The extent and clinical relevance of the association between epilepsy and sleep apnea are not previously studied in Egypt. What we wanted to know was the frequency of sleep apnea in Egyptian children with epilepsy and its influence on seizure frequency, other seizure characteristics, sleep complaint, and architecture. All patients with epilepsy, aged up to 18 years, who underwent polysomnography were studied. Patients with any neurological disease apart from epilepsy, with psychiatric illness, had hypnotics, or sedatives or those with liver or kidney failure were excluded from the study. The patients were divided into two subgroups according to apnea/hypopnea index: group (1) patients without obstructive sleep apnea (OSA) and group (2) patients with OSA. For control group, we choose 12 healthy individuals, with age and sex matched to that of our patients. We studied the clinical characteristics of epilepsy, sleep history, and polysomnographic recording of the patients with epilepsy and the control. EEG digital and video monitoring was done for all patients. Eleven patients (42.3%) were found to have obstructive sleep apnea. Seizure frequency was significantly higher in the patients with OSA. Apart from apnea and hypopnea indices, all other sleep parameters did not differ between patients' subgroups. Hypopnea index in REM positively correlates with number of awaking. Apnea index in REM positively correlates with latency to deep sleep and to periodic leg movement. Sleep apnea is frequent in patients with epilepsy. OSA may contribute to increase seizure frequency. We recommend investigating sleep apnea in all patients with epilepsy.

Effect of Temporal Neocortical Pathology on Seizure Freeness in Adult Patients with Temporal Lobe Epilepsy.

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Kemerdere, Rahsan; Ahmedov, Merdin Lyutviev; Alizada, Orkhan; Yeni, Seher Naz; Oz, Buge; Tanriverdi, Taner

2018-05-23

Temporal lobe epilepsy (TLE) is the most common form of focal epilepsy. Focal cortical dysplasia is the most common dual pathology found in association with the hippocampal sclerosis. In this study, the effect of dual pathology on freedom from seizure was sought in patients with TLE. This study performed a retrospective analysis of patients with TLE who underwent surgery between 2010 and 2017. Histopathologic analysis was performed on patients with and without dual pathology in the temporal neocortex. Seizure outcomes were compared. A total of 54 patients with TLE were included. The rate of overall favorable seizure outcome was found to be 96.3%. In 53.7%, dual pathology was present in the temporal cortices in addition to the hippocampal sclerosis. Patients without dual pathology showed significantly greater freedom from seizure (P = 0.02). Patients without dual pathology had a significantly higher seizure-free rate after anterior temporal resection than patients with dual pathology. Resection of the temporal cortex in addition to mesial temporal structures seems to be reasonable for better seizure outcome. Copyright © 2018 Elsevier Inc. All rights reserved.

Dual Pathology in Rasmussen's Encephalitis: A Report of Coexistent Focal Cortical Dysplasia and Review of the Literature

OpenAIRE

Prayson, Richard A.

2012-01-01

Rasmussen’s encephalitis is a well-established, albeit rare cause of medically intractable epilepsy. In a small number of Rasmussen's cases, a second pathology is identified, which independently can cause medically intractable seizures (dual pathology). This paper documents a case of a 13-year-old male who presented with medically intractable epilepsy. The patient underwent a series of surgical resections, early on resulting in a diagnosis of focal cortical dysplasia and later yielding a diag...

Genetic models of absence epilepsy: New concepts and insights

NARCIS (Netherlands)

Luijtelaar, E.L.J.M. van; Coenen, A.M.L.; Schwartzkroin, P.A.

2009-01-01

The discovery, development, and use of genetic rodent models of absence epilepsy have led to a new theory about the origin of absence seizures. A focal zone has been identified in the peri-oral region of the somatosensory cortex in WAG/Rij and GAERS – the two most commonly used models – from which

{sup 1}H MR spectroscopic imaging in patients with MRI-negative extratemporal epilepsy: correlation with ictal onset zone and histopathology

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Krsek, Pavel; Komarek, Vladimir [Charles University, Department of Pediatric Neurology, Second Medical School, Motol Hospital, Prague (Czech Republic); Hajek, Milan [Institute for Clinical and Experimental Medicine, MR Unit, Department of Diagnostic and Interventional Radiology, Prague (Czech Republic); Institute for Clinical and Experimental Medicine, MR Spectroscopy, Prague 4 (Czech Republic); Dezortova, Monika; Jiru, Filip; Skoch, Antonin [Institute for Clinical and Experimental Medicine, MR Unit, Department of Diagnostic and Interventional Radiology, Prague (Czech Republic); Marusic, Petr [Charles University, Department of Neurology, Second Medical School, Motol Hospital, Prague (Czech Republic); Zamecnik, Josef [Charles University, Department of Pathology and Molecular Medicine, Second Medical School, Motol Hospital, Prague (Czech Republic); Kyncl, Martin [Charles University, Department of Radiology, Second Medical School, Motol Hospital, Prague (Czech Republic); Tichy, Michal [Charles University, Department of Pediatric Neurosurgery, Second Medical School, Motol Hospital, Prague (Czech Republic)

2007-08-15

Proton magnetic resonance spectroscopy ({sup 1}H MRS) is beneficial in the lateralization of the epileptogenic zone in temporal lobe epilepsy; however, its role in extratemporal and, especially, MRI-negative epilepsy has not been established. This study seeks to verify how {sup 1}H MRS could help in localizing the epileptogenic zone in patients with MRI-negative extratemporal epilepsy. Seven patients (8-23 years) with MRI-negative refractory focal epilepsy were studied using {sup 1}H MRS on a 1.5T MR system. Chemical shift imaging sequence in the transversal plane was directed towards the suspected epileptogenic zone localized by seizure semiology, scalp video/EEG, ictal SPECT and {sup 18}FDG-PET. Spectra were evaluated using the program CULICH, and the coefficient of asymmetry was used for quantitative lateralization. MRS detected lateralization in all patients and was able to localize pathology in five. The most frequent findings were decreased ratios of N-acetylaspartate to choline compounds characterized by increasing choline concentration. The localization of the {sup 1}H MRS abnormality correlated well with ictal SPECT and subdural mapping. In all cases, histopathological analysis revealed MRI-undetected focal cortical dysplasias. {sup 1}H MRS could be more sensitive for the detection of discrete malformations of cortical development than conventional MRI. It is valuable in the presurgical evaluation of patients without MRI-apparent lesions. (orig.)

Benign occipital epilepsy of childhood: Panayiotopoulos syndrome in a 3 year old child

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Menon Narayanankutty Sunilkumar , Vadakut Krishnan Parvathy

2014-11-01

Full Text Available Panayiotopoulos syndrome (PS is a relatively frequent and benign epileptic syndrome seen in children in the age group of 3-6 years and is characterised by predominantly autonomic symptoms and/or simple motor focal seizures followed or not by impairment of consciousness. Although multifocal spikes with high amplitude sharp-slow wave complexes at various locations can be present in the EEG, interictal electroencephalogram (EEG in children with this particular type of epilepsy characteristically shows occipital spikes. This syndrome has known to be a masquerader and can imitate gastroenteritis, encephalitis, syncope, migraine, sleep disorders or metabolic diseases. In the absence of thorough knowledge of types of benign epilepsy syndromes and their various clinical presentations, epilepsy such as PS can be easily missed. The peculiar aspects of this type of epilepsy in children should be known not only by paediatricians but also by general doctors because a correct diagnosis would avoid aggressive interventions and concerns on account of its benign outcome. In this case study, we report a case of PS in a 3 year old child.

Epileptic negative drop attacks in atypical benign partial epilepsy: a neurophysiological study.

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Hirano, Yoshiko; Oguni, Hirokazu; Osawa, Makiko

2009-03-01

We conducted a computer-assisted polygraphic analysis of drop attacks in a child with atypical benign partial epilepsy (ABPE) to investigate neurophysiological characteristics. The patient was a six-year two-month-old girl, who had started to have focal motor seizures, later combined with daily epileptic negative myoclonus (ENM) and drop attacks, causing multiple injuries. We studied episodes of ENM and drop attacks using video-polygraphic and computer-assisted back-averaging analysis. A total of 12 ENM episodes, seven involving the left arm (ENMlt) and five involving both arms (ENMbil), and five drop attacks were captured for analysis. All episodes were time-locked to spike-and-wave complexes (SWC) arising from both centro-temporo-parietal (CTP) areas. The latency between the onset of SWC and ENMlt, ENMbil, and drop attacks reached 68 ms, 42 ms, and 8 ms, respectively. The height of the spike as well as the slow-wave component of SWC for drop attacks were significantly larger than that for both ENMlt and ENMbil (p negative myoclonus involving not only upper proximal but also axial muscles, causing the body to fall. Thus, drop attacks in ABPE are considered to be epileptic negative drop attacks arising from bilateral CTP foci and differ from drop attacks of a generalized origin seen in Lennox-Gastaut syndrome and myoclonic-astatic epilepsy.

De novo 12q22.q23.3 duplication associated with temporal lobe epilepsy.

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Vari, Maria Stella; Traverso, Monica; Bellini, Tommaso; Madia, Francesca; Pinto, Francesca; Minetti, Carlo; Striano, Pasquale; Zara, Federico

2017-08-01

Temporal lobe epilepsy (TLE) is the most common form of focal epilepsy and may be associated with acquired central nervous system lesions or could be genetic. Various susceptibility genes and environmental factors are believed to be involved in the aetiology of TLE, which is considered to be a heterogeneous, polygenic, and complex disorder. Rare point mutations in LGI1, DEPDC5, and RELN as well as some copy number variations (CNVs) have been reported in families with TLE patients. We perform a genetic analysis by Array-CGH in a patient with dysmorphic features and temporal lobe epilepsy. We report a de novo duplication of the long arm of chromosome 12. We confirm that 12q22-q23.3 is a candidate locus for familial temporal lobe epilepsy with febrile seizures and highlight the role of chromosomal rearrangements in patients with epilepsy and intellectual disability. Copyright © 2017 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

Does early ischemic lesion induce cognitive impairment and epilepsy?

Czech Academy of Sciences Publication Activity Database

Kubová, Hana; Máttéffyová, Adéla; Tsenov, Grygoriy; Otáhal, Jakub

-, - (2005), s. 30-30 [Conference of the Czech Neuroscience Society /5./, The Annual Meeting of the Network of European Neuroscience Institutes. 19.11.2005-21.11.2005, Prague] R&D Projects: GA MŠk(CZ) LC554 Institutional research plan: CEZ:AV0Z50110509 Keywords : focal ischemia * cognitive impairment * development of epilepsy Subject RIV: ED - Physiology

Noninvasive transcranial focal stimulation via tripolar concentric ring electrodes lessens behavioral seizure activity of recurrent pentylenetetrazole administrations in rats.

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Makeyev, Oleksandr; Luna-Munguía, Hiram; Rogel-Salazar, Gabriela; Liu, Xiang; Besio, Walter G

2013-05-01

Epilepsy affects approximately 1% of the world population. Antiepileptic drugs are ineffective in approximately 30% of patients and have side effects. We have been developing a noninvasive transcranial focal electrical stimulation with our novel tripolar concentric ring electrodes as an alternative/complementary therapy for seizure control. In this study we demonstrate the effect of focal stimulation on behavioral seizure activity induced by two successive pentylenetetrazole administrations in rats. Seizure onset latency, time of the first behavioral change, duration of seizure, and maximal seizure severity score were studied and compared for focal stimulation treated (n = 9) and control groups (n = 10). First, we demonstrate that no significant difference was found in behavioral activity for focal stimulation treated and control groups after the first pentylenetetrazole administration. Next, comparing first and second pentylenetetrazole administrations, we demonstrate there was a significant change in behavioral activity (time of the first behavioral change) in both groups that was not related to focal stimulation. Finally, we demonstrate focal stimulation provoking a significant change in seizure onset latency, duration of seizure, and maximal seizure severity score. We believe that these results, combined with our previous reports, suggest that transcranial focal stimulation may have an anticonvulsant effect.

Investigation of magnetic resonance imaging texture analysis as an aid tool for characterization of refractory epilepsies

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Mauricio Martins Baldissin

2013-12-01

Full Text Available Refractory epilepsies are syndromes for which therapies that employ two or more antiepileptic drugs, separately or in association, do not result in control of crisis. Patients may present focal cortical dysplasia or diffuse dysplasia and/or hippocampal atrophic alterations that may not be detectable by a simple visual analysis in magnetic resonance imaging. The aim of this study was to evaluate MRI texture in regions of interest located in the hippocampi, limbic association cortex and prefrontal cortex of 20 patients with refractory epilepsy and to compare them with the same areas in 20 healthy individuals, in order to find out if the texture parameters could be related to the presence of the disease. Of the 11 texture parameters calculated, three indicated the existence of statistically significant differences between the studied groups. Such findings suggest the possibility of this technique contributing to studies of refractory epilepsies.

WONOEP appraisal: new genetic approaches to study epilepsy

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Rossignol, Elsa; Kobow, Katja; Simonato, Michele; Loeb, Jeffrey A.; Grisar, Thierry; Gilby, Krista L.; Vinet, Jonathan; Kadam, Shilpa D.; Becker, Albert J.

2014-01-01

Objective New genetic investigation techniques, including next-generation sequencing, epigenetic profiling, cell lineage mapping, targeted genetic manipulation of specific neuronal cell types, stem cell reprogramming and optogenetic manipulations within epileptic networks are progressively unravelling the mysteries of epileptogenesis and ictogenesis. These techniques have opened new avenues to discover the molecular basis of epileptogenesis and to study the physiological impacts of mutations in epilepsy-associated genes on a multilayer level, from cells to circuits. Methods This manuscript reviews recently published applications of these new genetic technologies in the study of epilepsy, as well as work presented by the authors at the genetic session of the XII Workshop on the Neurobiology of Epilepsy in Quebec, Canada. Results Next-generation sequencing is providing investigators with an unbiased means to assess the molecular causes of sporadic forms of epilepsy and have revealed the complexity and genetic heterogeneity of sporadic epilepsy disorders. To assess the functional impact of mutations in these newly identified genes on specific neuronal cell-types during brain development, new modeling strategies in animals, including conditional genetics in mice and in utero knockdown approaches, are enabling functional validation with exquisite cell-type and temporal specificity. In addition, optogenetics, using cell-type specific Cre recombinase driver lines, is enabling investigators to dissect networks involved in epilepsy. Genetically-encoded cell-type labeling is also providing new means to assess the role of the non-neuronal components of epileptic networks such as glial cells. Furthermore, beyond its role in revealing coding variants involved in epileptogenesis, next-generation sequencing can be used to assess the epigenetic modifications that lead to sustained network hyperexcitability in epilepsy, including methylation changes in gene promoters and non

Usefulness of 18FDG-PET in epilepsy. Methods and indications

International Nuclear Information System (INIS)

Chassoux, F.

2009-01-01

Positron emission tomography (PET) using 18-fluorodeoxyglucose ( 18 FDG) is currently used in pre surgical work-up for drug-resistant partial epilepsies in children as in adults, in addition to MRI. Recent cameras with less than 5 mm spatial resolution allow to obtain thin slices (about 2 mm thickness) in 3D planes. 18 FDG is intravenously injected at the mean dose of 3 MBq/kg of body weight in inter-ictal and resting state, in a quiet, dimly lit environment and careful monitoring for head movements and ictal events. In children, sedation may be necessary. Image acquisition starts 30 min after injection and ended 15 to 20 min later. Semiquantitative analysis is visually assessed in clinical practice using colour scales. PET sensibility is improved by superimposition of metabolic imaging on MRI. Statistical analysis with S.P.M. may be useful but comparison with health subjects database is required. In medial temporal lobe epilepsy associated with hippocampal sclerosis, hypo metabolism ipsilateral to the epileptogenic focus is found in 70 to 90% of the cases and is predictive of surgical outcome. In other types of temporal and extra temporal epilepsy with negative MRI, focal hypo metabolism can be detected, allowing identification of minor gyral abnormalities corresponding to focal cortical dysplasia. In such MRI negative cases, PET findings may improve surgical outcome. (author)

Tailored unilobar and multilobar resections for orbitofrontal-plus epilepsy.

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Serletis, Demitre; Bulacio, Juan; Alexopoulos, Andreas; Najm, Imad; Bingaman, William; González-Martínez, Jorge

2014-10-01

Surgery for frontal lobe epilepsy often has poor results, likely because of incomplete resection of the epileptogenic zone. To present our experience with a series of patients manifesting 2 different anatomo-electro-clinical patterns of refractory orbitofrontal epilepsy, necessitating different surgical approaches for resection in each group. Eleven patients with refractory epilepsy involving the orbitofrontal region were consecutively identified over 3 years in whom stereoelectroencephalography identified the epileptogenic zone. All patients underwent preoperative evaluation, stereoelectroencephalography, and postoperative magnetic resonance imaging. Demographic features, seizure semiology, imaging characteristics, location of the epileptogenic zone, surgical resection site, and pathological diagnosis were analyzed. Surgical outcome was correlated with type of resection. Five patients exhibited orbitofrontal plus frontal epilepsy with the epileptogenic zone consistently residing in the frontal lobe; after surgery, 4 patients were free of disabling seizures (Engel I) and 1 patient improved (Engel II). The remaining 6 patients had multilobar epilepsy with the epileptogenic zone located in the orbitofrontal cortex associated with the temporal polar region (orbitofrontal plus temporal polar epilepsy). After surgery, all 6 patients were free of disabling seizures (Engel I). Pathology confirmed focal cortical dysplasia in all patients. We report no complications or mortalities in this series. Our findings highlight the importance of differentiating between orbitofrontal plus frontal and orbitofrontal plus temporal polar epilepsy in patients afflicted with seizures involving the orbitofrontal cortex. For identified cases of orbitofrontal plus temporal polar epilepsy, a multilobar resection including the temporal pole may lead to improved postoperative outcomes with minimal morbidity or mortality.

Seizure outcomes in non-resective epilepsy surgery: An update

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Englot, Dario J.; Birk, Harjus; Chang, Edward F.

2016-01-01

In approximately 30% of patients with epilepsy, seizures are refractory to medical therapy, leading to significant morbidity and increased mortality. Substantial evidence has demonstrated the benefit of surgical resection in patients with drug-resistant focal epilepsy, and in the present journal, we recently reviewed seizure outcomes in resective epilepsy surgery. However, not all patients are candidates for or amenable to open surgical resection for epilepsy. Fortunately, several non-resective surgical options are now available at various epilepsy centers, including novel therapies which have been pioneered in recent years. Ablative procedures such as stereotactic laser ablation and stereotactic radiosurgery offer minimally invasive alternatives to open surgery with relatively favorable seizure outcomes, particularly in patients with mesial temporal lobe epilepsy. For certain individuals who are not candidates for ablation or resection, palliative neuromodulation procedures such as vagus nerve stimulation, deep brain stimulation, or responsive neurostimulation may result in a significant decrease in seizure frequency and improved quality of life. Finally, disconnection procedures such as multiple subpial transections and corpus callosotomy continue to play a role in select patients with an eloquent epileptogenic zone or intractable atonic seizures, respectively. Overall, open surgical resection remains the gold standard treatment for drug-resistant epilepsy, although it is significantly under-utilized. While non-resective epilepsy procedures have not replaced the need for resection, there is hope that these additional surgical options will increase the number of patients who receive treatment for this devastating disorder - particularly individuals who are not candidates for or who have failed resection. PMID:27206422

[Epilepsy and epileptic syndromes during the first year of life].

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Durá-Travé, T; Yoldi-Petri, M E; Hualde-Olascoaga, J; Etayo-Etayo, V

To analyse the epidemiological characteristics and the relative distribution of the different types of epilepsy and epileptic syndromes during the first year of life. An analysis was performed of the patient records of all patients with epilepsy diagnosed during their first year of life who were submitted to a developmental check-up in the year 2007. The sample consisted of 60 patients (27 boys and 33 girls). Epidemiological and clinical data were collected, together with the findings from complementary examinations. The diagnostic criteria applied were those of the International League Against Epilepsy. The mean age at the time of diagnosis was 6.3 months. The mean follow-up time was 7.6 years. The aetiology was symptomatic in 40 cases (66.7%), cryptogenic in 16 (26.7%) and idiopathic in four cases (6.7%). Neuroimaging tests detected abnormalities in 34 patients (56.7%). West's syndrome (30%), symptomatic focal epilepsies (23.3%) and epilepsies linked to specific syndromes (16.7%) were the epileptic syndromes with the highest prevalence. Learning disabilities were observed in 82.5% of the children. Most epilepsies that present during the first year of life are symptomatic and/or cryptogenic, and are accompanied by psychoneurological impairment and/or resistance to therapy, which condition cognitive disorders that are eligible for specialised psycho-pedagogical intervention.

Effect and Safety of Shihogyejitang for Drug Resistant Childhood Epilepsy

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Jinsoo Lee

2016-01-01

Full Text Available Objective. Herbal medicine has been widely used to treat drug resistant epilepsy. Shihogyejitang (SGT has been commonly used to treat epilepsy. We investigated the effect and safety of SGT in children with drug resistant epilepsy. Design. We reviewed medical records of 54 patients with epilepsy, who failed to respond to at least two antiepileptic drugs and have been treated with SGT between April 2006 and June 2014 at the Department of Pediatric Neurology, I-Tomato Hospital, Korea. Effect was measured by the response rate, seizure-free rate, and retention rate at six months. We also checked adverse events, change in antiepileptic drugs use, and the variables related to the outcome. Results. Intent-to-treat analysis showed that, after six months, 44.4% showed a >50% seizure reduction, 24.1% including seizure-free, respectively, and 53.7% remained on SGT. Two adverse events were reported, mild skin rash and fever. Focal seizure type presented significantly more positive responses when compared with other seizure types at six months (p=0.0284, Fisher’s exact test. Conclusion. SGT is an effective treatment with excellent tolerability for drug resistant epilepsy patients. Our data provide evidence that SGT may be used as alternative treatment option when antiepileptic drug does not work in epilepsy children.

Validation of rapid suicidality screening in epilepsy using the NDDIE.

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Mula, Marco; McGonigal, Aileen; Micoulaud-Franchi, Jean-Arthur; May, Theodor W; Labudda, Kirsten; Brandt, Christian

2016-06-01

Standard mortality ratio for suicide in patients with epilepsy is three times higher than in the general population, and such a risk remains high even after adjusting for clinical and socioeconomic factors. It is thus important to have suitable screening instruments and to implement care pathways for suicide prevention in every epilepsy center. The aim of this study is to validate the use of the Neurological Disorder Depression Inventory for Epilepsy (NDDIE) as a suicidality-screening instrument. The study sample included adult patients with epilepsy assessed with the Mini International Neuropsychiatric Interview (MINI) and the NDDIE. A high suicidality risk according to the Suicidality Module of the MINI was considered the gold standard. Receiver operating characteristic analyses for NDDIE total and individual item scores were computed and subsequently compared using a nonparametric approach. The best possible cutoff was identified with the highest Youden index (J). Likelihood ratios were then computed, and specificity, sensitivity, positive, and negative predictive values calculated. The study sample consisted of 380 adult patients with epilepsy: 46.3% male; mean age was 39.4 ± 14.6; 76.7% had a diagnosis of focal epilepsy; mean age at onset of the epilepsy was 23.3 ± 17.5. According to the MINI, 74 patients (19.5%) fulfilled criteria for a major depressive episode and 19 (5%) presented a high suicidality risk. A score >2 (J = 0.751) for item 4 "I'd be better off dead" of the NDDIE displayed excellent psychometric properties with a good to excellent validity (area under the curve [AUC] 0.906; 95% confidence interval [CI] 0.820-0.992; p < 0.001), sensitivity 84.21% (95% CI 60.4-96.6), specificity 90.86% (95% CI 87.4-93.6), likelihood ratio+ 9.21 (95% CI 6.3-13.5), likelihood ratio- 0.17 (95% CI 0.06-0.50). Item 4 of the NDDIE has shown to be an excellent suicidality screening instrument allowing the development of further care pathways for suicide prevention in

Behavioral effects and somnolence due to levetiracetam versus oxcarbazepine - a retrospective comparison study of North Indian patients with refractory epilepsy.

Science.gov (United States)

Shukla, Garima; Gupta, Anupama; Agarwal, Priya; Poornima, Shivani

2016-11-01

Levetiracetam (LEV) is often chosen early in the treatment of refractory epilepsy; however, its adverse effects have largely been studied as part of clinical trials. Oxcarbazepine and valproate (VPA) are the other commonly used AEDs and, hence, serve as good comparators. This study was conducted to evaluate behavioral abnormalities and somnolence among patients with epilepsy being treated with LEV and/or OXC compared with those receiving VPA. Data of consecutive patients attending our intractable epilepsy clinic over a 2 1/2-year period were reviewed, and patients with at least one seizure a month, who had been initiated on either or a combination of LEV, VPA, or OXC, were included for analysis. Data regarding behavioral adverse effects, daytime somnolence (EDS), and weight changes were collected apart from those regarding any major effect necessitating dose reduction or discontinuation of the AED. Among a total of 445 patients screened, 292 (93 F, median age: 21years [range: 8-54]; 237 focal and 55 generalized epilepsy) fulfilled inclusion criteria. Median epilepsy duration was 11years. Levetiracetam had been introduced in 114 patients, VPA in 134, and OXC in 151 during the study period. Twenty-three were on LEV+OXC, 27 on LEV+VPA, and 33 on VPA+OXC. Behavioral disturbances (irritability, obsessive manifestations, aggressiveness, and frank psychosis) were observed in 43 patients; 23 on introduction of LEV (20.2%); LEV was discontinued in 10 (9%). Daytime somnolence was reported by 28 patients, 15 on OXC (10%); 8 received oral modafinil for the same, while none discontinued this AED. Only one patient on LEV and 3 on VPA reported EDS. Menstrual disturbances were reported by 9, weight gain by 3, and severe hair loss by 2 females on VPA. Behavioral disturbances with levetiracetam are common among patients with refractory epilepsy while somnolence is common with oxcarbazepine. Antiepileptic drugs should be selected with this in perspective. Copyright © 2016 Elsevier

Developing the Pediatric Refractory Epilepsy Questionnaire: a pilot study.

Science.gov (United States)

Purusothaman, Vaishnavi; Ryther, Robin C C; Bertrand, Mary; Harker, Lisa A; Jeffe, Donna B; Wallendorf, Michael; Smyth, Matthew D; Limbrick, David D

2014-08-01

Up to 14% of children with epilepsy continue to experience seizures despite having appropriate medical therapy and develop medically refractory epilepsy (MRE). Assessing clinical outcomes and therapeutic efficacy in children with MRE undergoing palliative epilepsy surgery has been challenging because of the lack of a quantitative instrument capable of estimating the clinical status of these patients. The ideal instrument would at once consider seizure control, neurodevelopment, caregiver burden, and quality of life. The purpose of this study was to develop and pilot the Pediatric Refractory Epilepsy Questionnaire (PREQ), a quantitative instrument to assess the severity and individual burden of epilepsy in children with MRE undergoing palliative epilepsy treatments. The caregivers of 25 patients with MRE completed the PREQ and the Quality of Life in Childhood Epilepsy (QOLCE) measure and participated in a semistructured interview. Medical records of the patients were reviewed, an Early Childhood Epilepsy Severity Scale (E-CHESS) score was calculated, and a Global Assessment of Severity of Epilepsy (GASE) score was obtained for each patient. The initial PREQ was modified based on the analysis of responses, association with previously validated scales, comments from caregivers, and expertise of the PREQ panelists. Pediatric Refractory Epilepsy Questionnaire subscale scores were calculated based on clinical paradigm and compared with independent measures of seizure severity and quality of life. Significant correlations were observed between the seizure severity subscale and the GASE score (r=0.55) and between the mood subscale and the well-being score (r=0.61) on the QOLCE. Significant correlations were also observed between the caregiver rating of seizure severity and the GASE score (r=0.53), the social activity score (r=0.57), and the behavior score (r=0.43) on the QOLCE. Correlations between the caregiver rating of quality of life and the quality of life score (r=0

Surgical treatment for medically refractory focal epilepsy in a patient with fragile X syndrome.

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Kenmuir, Cynthia; Richardson, Mark; Ghearing, Gena

2015-10-01

Medication resistant temporal lobe epilepsy occurs in a small population of patients with fragile X syndrome. We present the case of a 24-year-old man with medically refractory temporal lobe epilepsy and fragile X syndrome who underwent left anterior temporal lobectomy resulting in cessation of seizures. Our patient was diagnosed with fragile X syndrome with a fully mutated, fully methylated FMR1 gene resulting in 572 CGG repeats. He developed seizures initially controlled with Depakote monotherapy, but progressed to become medically refractive to combination treatment with Depakote, lamotrigine and zonisamide. Prolonged video EEG monitoring revealed interictal left temporal sharp waves and slowing as well as subclinical and clinical seizures, each with left temporal onset. 3T MRI was consistent with left mesial temporal sclerosis. After discussing the case in our multidisciplinary surgical epilepsy conference, he was referred for presurgical evaluation including neuropsychological testing and Wada testing. He underwent an asleep left anterior temporal lobectomy, sparing the superior temporal gyrus. Pathology showed neuronal loss and gliosis in the hippocampus and amygdala. Twelve months after surgery, the patient has not experienced a seizure. He is described by his parents as less perseverative and less restless. We have presented the case of a 24 year-old-man with fragile X syndrome who underwent successful left anterior temporal lobectomy for the treatment of medically refractory epilepsy who is now seizure free without further functional impairment. This case report demonstrates the feasibility of surgical treatment for a patient with comorbid fragile X syndrome and mesial temporal sclerosis. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Health perception and socioeconomic status following childhood-onset epilepsy : The Dutch study of epilepsy in childhood

NARCIS (Netherlands)

Geerts, Ada; Brouwer, Oebele; van Donselaar, Cees; Stroink, Hans; Peters, Boudewijn; Peeters, Els; Arts, Willem F.

2011-01-01

Purpose: Epilepsy may have far-reaching consequences for patients, other than having seizures and medication. At 15 years after diagnosis, this study investigates health perception, restrictions due to epilepsy, living arrangements (including marital status and offspring), and the educational and

Predictors and Course of Medically Intractable Epilepsy in Young Children Presenting Before 36 Months of Age: A Retrospective, Population-Based Study

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Wirrell, Elaine; Wong-Kisiel, Lily; Mandrekar, Jay; Nickels, Katherine

2012-01-01

Purpose To determine the prevalence and identify predictors of medical intractability in children presenting with epilepsy prior to 36 months of age, and to assess the impact of medical intractability on long-term mortality and intellectual function. Methods Children with newly-diagnosed epilepsy prior to 36 months between 1980–2009 while resident in Olmsted County, MN were identified. Medical records were reviewed to collect epilepsy specific variables and long-term outcome data. Medically intractable epilepsy was defined as either (1) seizures greater than every 6 months at final follow-up and failure of two or more antiepileptic drugs for lack of efficacy, or (2) having undergone epilepsy surgery after failure of two or more antiepileptic drugs. Key Findings One hundred and twenty seven children with new-onset epilepsy were identified and followed for a median of 78 months. Medically intractable seizures occurred in 35%, and significant predictors on multivariate analysis were age ≤12 months at diagnosis (odds ratio [OR] 6.76, 95% confidence interval [CI] 2.00, 22.84, p=0.002), developmental delay at initial diagnosis of epilepsy (OR 20.03, 95% CI 3.49, 114.83, p=0.0008 ), neuroimaging abnormality (OR 6.48, 95% CI 1.96, 21.40, p=0.002) and focal slowing on initial EEG (OR 5.33, 95% CI 1.14, 24.88, p=0.03). Medical intractability occurred early in the course in most children, being seen in 61% by one year, and 93% by five years after initial diagnosis. Mortality was higher (20% vs 0%, p<0.001) and intellectual outcome poorer (p<0.001) if epilepsy was medically intractable. Significance One third of children presenting with epilepsy before 36 months will be medically intractable and significant predictors are identified. Medically intractable epilepsy is associated with increased mortality risk and significant intellectual disability. PMID:22738069

International recommendation for a comprehensive neuropathologic workup of epilepsy surgery brain tissue: A consensus Task Force report from the ILAE Commission on Diagnostic Methods

NARCIS (Netherlands)

Blümcke, Ingmar; Aronica, Eleonora; Miyata, Hajime; Sarnat, Harvey B.; Thom, Maria; Roessler, Karl; Rydenhag, Bertil; Jehi, Lara; Krsek, Pavel; Wiebe, Samuel; Spreafico, Roberto

2016-01-01

Epilepsy surgery is an effective treatment in many patients with drug-resistant focal epilepsies. An early decision for surgical therapy is facilitated by a magnetic resonance imaging (MRI)visible brain lesion congruent with the electrophysiologically abnormal brain region. Recent advances in the

International recommendation for a comprehensive neuropathologic workup of epilepsy surgery brain tissue : A consensus Task Force report from the ILAE Commission on Diagnostic Methods

NARCIS (Netherlands)

Blümcke, I.; Aronica, E.; Miyata, H.; Sarnat, H.B.; Thom, M.; Roessler, K.; Rydenhag, B.; Jehi, L.; Krsek, P.; Wiebe, S.; Spreafico, R.

2016-01-01

Epilepsy surgery is an effective treatment in many patients with drug-resistant focal epilepsies. An early decision for surgical therapy is facilitated by a magnetic resonance imaging (MRI)-visible brain lesion congruent with the electrophysiologically abnormal brain region. Recent advances in the

Factores pronósticos de recurrencia de la epilepsia focal en el niño Prognostic factors of recurrence of focal epilepsy in children

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Albia Pozo Alonso

2009-12-01

Full Text Available INTRODUCCIÓN. El objetivo de este trabajo fue determinar los factores pronósticos de recurrencia de las crisis epilépticas focales a los 2 años del diagnóstico y del inicio del tratamiento. MÉTODOS. Este estudio observacional, analítico y prospectivo incluyó a 207 niños que presentaron dos o más crisis epilépticas focales no provocadas, hospitalizados en el Departamento de Neuropediatría del Hospital «William Soler», entre diciembre de 2001 y diciembre de 2003. Al final de los 2 años de seguimiento, 185 pacientes concluyeron el estudio. RESULTADOS. El 33,5 % de los pacientes presentó recurrencias de las crisis epilépticas focales al finalizar el estudio. Constituyeron factores de riesgo de recurrencia de las crisis epilépticas focales los siguientes: edad menor de un año, etiología sintomática, presencia de antecedentes personales de crisis neonatales sintomáticas y discapacidades neurológicas y la persistencia de descargas en el electroencefalograma (EEG evolutivo. El análisis de regresión logística demostró como variables pronósticas de recurrencia la etiología sintomática (p = 0,000; OR = 3,107, el antecedente personal de crisis neonatales sintomáticas (p = 0,037; OR = 4,623 y la persistencia de descargas en el EEG evolutivo (p = 0,000; OR = 2,109. CONCLUSIONES. El antecedente personal de crisis neonatales sintomáticas constituyó el factor independiente con mayor influencia en las recurrencias de las crisis epilépticas focales.INTRODUCTION: The objective of present paper was to determine the recurrent prognostic factors of focal epileptic crises at 2 years of diagnosis and of treatment onset. METHODS: This prospective, analytical and observational study included 207 children presenting two or more non-provoked epileptic crises, admitted in Neuropediatrics Department of "William Soler" Hospital between December, 2001 and December, 2003. At a two years follow-up, 185 patients concluded the study. RESULTS: The

Familial occurrence of epilepsy in children with newly diagnosed multiple seizures : Dutch study of epilepsy in childhood

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Callenbach, PMC; Geerts, AT; Arts, WFM; van Donselaar, CA; Peters, A.C. Boudewyn; Stroink, H; Brouwer, OF

Purpose: To study the familial occurrence of epilepsy in children with newly diagnosed multiple unprovoked seizures. Methods: Between August 1988 and September 1992, 462 children with two or more unprovoked seizures were included in the prospective Dutch Study of Epilepsy in Childhood. Seizures and

Emotional Prosody Processing in Epilepsy: Some Insights on Brain Reorganization

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Lucy Alba-Ferrara

2018-03-01

Full Text Available Drug resistant epilepsy is one of the most complex, multifactorial and polygenic neurological syndrome. Besides its dynamicity and variability, it still provides us with a model to study brain-behavior relationship, giving cues on the anatomy and functional representation of brain function. Given that onset zone of focal epileptic seizures often affects different anatomical areas, cortical but limited to one hemisphere, this condition also let us study the functional differences of the left and right cerebral hemispheres. One lateralized function in the human brain is emotional prosody, and it can be a useful ictal sign offering hints on the location of the epileptogenic zone. Besides its importance for effective communication, prosody is not considered an eloquent domain, making resective surgery on its neural correlates feasible. We performed an Electronic databases search (Medline and PsychINFO from inception to July 2017 for studies about prosody in epilepsy. The search terms included “epilepsy,” “seizure,” “emotional prosody,” and “vocal affect.” This review focus on emotional prosody processing in epilepsy as it can give hints regarding plastic functional changes following seizures (preoperatively, resection (post operatively, and also as an ictal sign enabling the assessment of dynamic brain networks. Moreover, it is argued that such reorganization can help to preserve the expression and reception of emotional prosody as a central skill to develop appropriate social interactions.

Effect of seizure on hippocampus in mesial temporal lobe epilepsy and neocortical epilepsy: an MRS study

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Lee, S.K.; Kim, D.W.; Kim, K.K.; Chung, C.K.; Song, I.C.; Chang, K.H.

2005-01-01

This study was performed to evaluate the effect of seizures on the bilateral hippocampus in mesial temporal lobe epilepsy (mTLE) and neocortical epilepsy by single voxel proton magnetic resonance spectroscopy (MRS). Forty-one patients with mTLE having unilateral hippocampal sclerosis and 43 patients with a neocortical epilepsy who underwent subsequent epilepsy surgery were recruited. Ninety-five percent confidence intervals of N-acetyl aspartate/choline (NAA/Cho) and NAA/creatine (NAA/Cr) ratios in 20 healthy control subjects were used as threshold values to determine abnormal NAA/Cho and NAA/Cr. NAA/Cho and NAA/Cr were significantly lower in the ipsilateral hippocampus of mTLE and neocortical epilepsy. Using asymmetry indices for patients with bilaterally abnormal ratios of NAA/Cho and NAA/Cr in addition to using unilateral abnormal ratio, the seizure focus was correctly lateralized in 65.9% of patients with mTLE and 48.8% of neocortical epilepsy patients. Bilateral NAA/Cho abnormality was significantly related to a poor surgical outcome in mTLE. No significant relationship was found between the results of NAA/Cho or NAA/Cr and surgical outcome in neocortical epilepsy. The mean contralateral NAA/Cr ratio of the hippocampus in mTLE was significantly lower in patients with a history of secondary generalized tonic-clonic seizure (SGTCS) than in those without. (orig.)

Effect of seizure on hippocampus in mesial temporal lobe epilepsy and neocortical epilepsy: an MRS study

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Lee, S.K.; Kim, D.W.; Kim, K.K. [Seoul National University College of Medicine, Seoul National University Hospital, Department of Neurology, Chongno ku, Seoul (Korea); Chung, C.K. [Seoul National University College of Medicine, Seoul National University Hospital, Department of Neurosurgery, Chongno ku, Seoul (Korea); Song, I.C.; Chang, K.H. [Seoul National University College of Medicine, Seoul National University Hospital, Department of Radiology, Chongno ku, Seoul (Korea)

2005-12-01

This study was performed to evaluate the effect of seizures on the bilateral hippocampus in mesial temporal lobe epilepsy (mTLE) and neocortical epilepsy by single voxel proton magnetic resonance spectroscopy (MRS). Forty-one patients with mTLE having unilateral hippocampal sclerosis and 43 patients with a neocortical epilepsy who underwent subsequent epilepsy surgery were recruited. Ninety-five percent confidence intervals of N-acetyl aspartate/choline (NAA/Cho) and NAA/creatine (NAA/Cr) ratios in 20 healthy control subjects were used as threshold values to determine abnormal NAA/Cho and NAA/Cr. NAA/Cho and NAA/Cr were significantly lower in the ipsilateral hippocampus of mTLE and neocortical epilepsy. Using asymmetry indices for patients with bilaterally abnormal ratios of NAA/Cho and NAA/Cr in addition to using unilateral abnormal ratio, the seizure focus was correctly lateralized in 65.9% of patients with mTLE and 48.8% of neocortical epilepsy patients. Bilateral NAA/Cho abnormality was significantly related to a poor surgical outcome in mTLE. No significant relationship was found between the results of NAA/Cho or NAA/Cr and surgical outcome in neocortical epilepsy. The mean contralateral NAA/Cr ratio of the hippocampus in mTLE was significantly lower in patients with a history of secondary generalized tonic-clonic seizure (SGTCS) than in those without. (orig.)

International recommendation for a comprehensive neuropathologic workup of epilepsy surgery brain tissue: A consensus Task Force report from the ILAE Commission on Diagnostic Methods.

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Blümcke, Ingmar; Aronica, Eleonora; Miyata, Hajime; Sarnat, Harvey B; Thom, Maria; Roessler, Karl; Rydenhag, Bertil; Jehi, Lara; Krsek, Pavel; Wiebe, Samuel; Spreafico, Roberto

2016-03-01

Epilepsy surgery is an effective treatment in many patients with drug-resistant focal epilepsies. An early decision for surgical therapy is facilitated by a magnetic resonance imaging (MRI)-visible brain lesion congruent with the electrophysiologically abnormal brain region. Recent advances in the pathologic diagnosis and classification of epileptogenic brain lesions are helpful for clinical correlation, outcome stratification, and patient management. However, application of international consensus classification systems to common epileptic pathologies (e.g., focal cortical dysplasia [FCD] and hippocampal sclerosis [HS]) necessitates standardized protocols for neuropathologic workup of epilepsy surgery specimens. To this end, the Task Force of Neuropathology from the International League Against Epilepsy (ILAE) Commission on Diagnostic Methods developed a consensus standard operational procedure for tissue inspection, distribution, and processing. The aims are to provide a systematic framework for histopathologic workup, meeting minimal standards and maximizing current and future opportunities for morphofunctional correlations and molecular studies for both clinical care and research. Whenever feasible, anatomically intact surgical specimens are desirable to enable systematic analysis in selective hippocampectomies, temporal lobe resections, and lesional or nonlesional neocortical samples. Correct orientation of sample and the sample's relation to neurophysiologically aberrant sites requires good communication between pathology and neurosurgical teams. Systematic tissue sampling of 5-mm slabs along a defined anatomic axis and application of a limited immunohistochemical panel will ensure a reliable differential diagnosis of main pathologies encountered in epilepsy surgery. Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.

Role of three-dimensional fluid-attenuated inversion recovery (3D FLAIR) and proton density magnetic resonance imaging for the detection and evaluation of lesion extent of focal cortical dysplasia in patients with refractory epilepsy

International Nuclear Information System (INIS)

Saini, Jitender; Kesavadas, Chandrasekharan; Thomas, Bejoy; Singh, Atampreet; Rathore, Chathurbhuj; Radhakrishnan, Ashalatha; Radhakrishnan, Kurupath; Bahuleyan, Biji

2010-01-01

Background: Focal cortical dysplasia (FCD) is often associated with epilepsy. Identification of FCD can be difficult due to subtle magnetic resonance imaging (MRI) changes. Though fluid-attenuated inversion recovery (FLAIR) sequence detects the majority of these lesions, smaller lesions may go unnoticed while larger lesions may be poorly delineated. Purpose: To determine the ability of a specialized epilepsy protocol in visualizing and delineating the extent of FCD. Material and Methods: We compared the imaging findings in nine patients with cortical malformation who underwent routine epilepsy MR imaging as well as a specialized epilepsy protocol. All imaging was done on a 1.5T MR unit. The specialized epilepsy protocol included 3D FLAIR in the sagittal plane as well as proton density (PD) and high-resolution T2-weighted (T2W) images in the transverse plane. Results: In all nine patients, the specialized protocol identified lesion anatomy better. In three patients in whom routine MRI was normal, the specialized epilepsy protocol including 3D FLAIR helped in identifying the lesions. One of these patients underwent surgery, and histo-pathology revealed a cortical dysplasia. In one patient, lesion characterization was improved, while in the remaining patients the extent of the FCD was more clearly demonstrated in the 3D FLAIR and PD images. Statistical analysis of images for cortical thickness, cortical signal intensity, adjacent white matter abnormalities, and gray-white matter junction showed significant statistical difference in the ability of 3D FLAIR to assess these aspects over conventional images. PD images were also found superior to the routine epilepsy protocol in assessment of cortical signal, adjacent white matter, and gray-white matter junction. Conclusion: Specialized MRI sequences and techniques should be performed whenever there is a high suspicion of cortical dysplasia, especially when they remain occult on conventional MR protocols. These techniques

Dilemmas in diagnostics and therapy of rolandic epilepsy

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Škrijelj Fadil

2011-01-01

Full Text Available Introduction. It is considered that around 20%-30% of patients of all ages and in all continents have wrong epilepsy diagnoses. Diagnostic and consequential therapeutic errors appear, most often, when an adequate diagnostics is not applied. Benign focal epilepsy of childhoods with centrotemporal spikes-rolandic epilepsy, brings very often to diagnostic and therapeutic problems because of persistence of epilepticforms changes in an electroencephalography (EEG recording, several years after establishment of good control over seizures. Case report. We presented 8.5 years-old girl, with the first and the only epileptic seizure at the age of 5, during her sleep. With a clear correlation of EEG record, benign rolandic epilepsy was diagnosed, so the therapy with valproate was introduced. There were no seizures after three years of its implementation. Because of epileptic-forms changes that still persisted in EEG record during her sleep, it was suggested to further use valproate. However, after reconsidering all circumstances it was concluded that the AED should bee slowly reduced up to its exclusion. After a complete stoppage of the therapy, the patient did not have any epileptic seizure for nine months, although EEG still remained pathologically changed during her sleep. Conclusion. A changed EEG record in a patient with rolandic epilepsy must not be a predictor of continuation of antiepileptic drugs therapy, after 2-3 years of successful seizures remission.

Usefulness of {sup 18}FDG-PET in epilepsy. Methods and indications; Utilite de la TEP au {sup 18}FDG dans l'epilepsie. Methodes et indications

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Chassoux, F. [Centre hospitalier Sainte-Anne, 1, rue Cabanis, 75014 Paris, (France); Service hospitalier Frederic-Joliot, I2BM-CEA, 4, place du General Leclerc, 91401 Orsay cedex, (France)

2009-05-15

Positron emission tomography (PET) using 18-fluorodeoxyglucose ({sup 18}FDG) is currently used in pre surgical work-up for drug-resistant partial epilepsies in children as in adults, in addition to MRI. Recent cameras with less than 5 mm spatial resolution allow to obtain thin slices (about 2 mm thickness) in 3D planes. {sup 18}FDG is intravenously injected at the mean dose of 3 MBq/kg of body weight in inter-ictal and resting state, in a quiet, dimly lit environment and careful monitoring for head movements and ictal events. In children, sedation may be necessary. Image acquisition starts 30 min after injection and ended 15 to 20 min later. Semiquantitative analysis is visually assessed in clinical practice using colour scales. PET sensibility is improved by superimposition of metabolic imaging on MRI. Statistical analysis with S.P.M. may be useful but comparison with health subjects database is required. In medial temporal lobe epilepsy associated with hippocampal sclerosis, hypo metabolism ipsilateral to the epileptogenic focus is found in 70 to 90% of the cases and is predictive of surgical outcome. In other types of temporal and extra temporal epilepsy with negative MRI, focal hypo metabolism can be detected, allowing identification of minor gyral abnormalities corresponding to focal cortical dysplasia. In such MRI negative cases, PET findings may improve surgical outcome. (author)

Major vault protein (MVP) gene polymorphisms and drug resistance in mesial temporal lobe epilepsy with hippocampal sclerosis.

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Balan, Shabeesh; Radhab, Saradalekshmi Koramannil; Radha, Koramannil; Sathyan, Sanish; Vijai, Joseph; Banerjee, Moinak; Radhakrishnan, Kurupath

2013-09-10

The human major vault protein (MVP) has been implicated in the development of drug resistance in cancer cells. Over expression of MVP has also been reported in brain tissue samples from antiepileptic drug (AED)-resistant human focal epilepsies. To investigate the relationship between single nucleotide polymorphisms (SNPs) involving the MVP gene and AED-resistance, we compared the distribution of three SNPs in the MVP gene, rs4788187, rs3815824 and rs3815823, among 220 patients with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) (prototype of AED-resistant epilepsy syndrome), 201 patients with juvenile myoclonic epilepsy (JME) (prototype of AED-responsive epilepsy syndrome) and 213 ethnically matched non-epilepsy controls. All the patients and controls were residents of the South Indian state of Kerala for more than three generations. We did not find any significant difference in allele and genotypic frequencies of the studied SNPs between AED-resistant and AED-responsive cohorts, and between AED-resistant and AED-responsive cohorts independently and pooled together when compared with the controls. We conclude that rs4788187, rs3815824, rs3815823 variants of the MVP gene are associated neither with predisposition for epilepsy nor with AED-resistance in the population that we have studied. Our results suggest the need for further research into the link between MVP and AED-resistance. Copyright © 2013 Elsevier B.V. All rights reserved.

Peri-ictal water drinking: a rare automatic behaviour in temporal lobe epilepsy.

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Pietrafusa, Nicola; Trivisano, Marina; de Palma, Luca; Serino, Domenico; Moavero, Romina; Benvenga, Antonella; Cappelletti, Simona; Boero, Giovanni; Vigevano, Federico; La Neve, Angela; Specchio, Nicola

2015-12-01

Peri-ictal water drinking (PIWD) has been reported as the action of drinking during or within two minutes of an electroclinical seizure. It is considered a peri-ictal vegetative symptom, evident both during childhood and adulthood epilepsy. The aim of this paper was to describe the clinical and electroencephalographic features of two new adult subjects suffering from symptomatic temporal lobe epilepsy with episodes of PIWD recorded by VIDEO-EEG and to review literature data in order to better define this peculiar event during seizures, a rare and probably underestimated semiological sign. To date, 51 cases with focal epilepsy and seizures associated with PIWD have been reported. All patients presented with temporal lobe epilepsy. All cases but one had symptomatic epilepsy. Most of the patients had an involvement of the right hemisphere. Water drinking was reported as an ictal sign in the majority of patients, and less frequently was reported as postictal. We believe that PIWD might be considered a rare automatic behaviour, like other automatisms. Automatisms are more frequently described in patients with temporal lobe epilepsy. PIWD was reported also to have lateralizing significance in the non-dominant temporal lobe, however, because of its rarity, this finding remains unclear.

Sleep problems in children and adolescents with epilepsy: Associations with psychiatric comorbidity.

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Hansen, Berit Hjelde; Alfstad, Kristin Å; van Roy, Betty; Henning, Oliver; Lossius, Morten I

2016-09-01

Sleep problems are common in pediatric epilepsy and may influence seizure control, daytime functioning, and overall quality of life. Knowledge of factors contributing to sleep problems is likely to improve treatment. The aim of this study was to investigate associations between psychiatric comorbidity and parent-reported and self-reported sleep problems in a sample of children and adolescents with epilepsy. Participants were children and adolescents (N=94), aged 10-19years, with generalized or focal epilepsy who had been referred to a tertiary epilepsy treatment center in Norway. Participants underwent a thorough clinical assessment and 24h of EEG registration. Information on sleep problems was obtained from parents using the Children's Sleep Habit Questionnaire (CSHQ) and from self-reporting using the Sleep Self-Report (SSR) questionnaire. Psychiatric diagnoses were established using the semistructured psychiatric interview Schedule for Affective Disorders and Schizophrenia - Present and Lifetime Version (Kiddie-SADS-PL). Both the total and subdomain CSHQ and SSR scores were high in comparison with scores from population-based samples. Having one or more psychiatric disorder(s) was significantly associated with elevated scores on both the CSHQ and the SSR. With the exception of parent-reported parasomnias, associations between sleep problems and psychiatric disorders remained significant after adjusting for relevant epilepsy variables. Psychiatric comorbidity explained about one-third of the variance of the reported sleep problems in children and adolescents with epilepsy. Copyright © 2016 Elsevier Inc. All rights reserved.

Early life stress as an influence on limbic epilepsy: an hypothesis whose time has come?

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Amelia S Koe

2009-10-01

Full Text Available The pathogenesis of mesial temporal lobe epilepsy (MTLE, the most prevalent form of refractory focal epilepsy in adults, is thought to begin in early life, even though seizures may not commence until adolescence or adulthood. Amongst the range of early life factors implicated in MTLE causation (febrile seizures, traumatic brain injury, etc., stress may be one important contributor. Early life stress is an a priori agent deserving study because of the large amount of neuroscientific data showing enduring effects on structure and function in hippocampus and amygdala, the key structures involved in MTLE. An emerging body of evidence directly tests hypotheses concerning early life stress and limbic epilepsy: early life stressors, such as maternal separation, have been shown to aggravate epileptogenesis in both status epilepticus and kindling models of limbic epilepsy. In addition to elucidating its influence on limbic epileptogenesis itself, the study of early life stress has the potential to shed light on the psychiatric disorder that accompanies MTLE. For many years, psychiatric comorbidity was viewed as an effect of epilepsy, mediated psychologically and/or neurobiologically. An alternative – or complementary – perspective is that of shared causation. Early life stress, implicated in the pathogenesis of several psychiatric disorders, may be one such causal factor. This paper aims to critically review the body of experimental evidence linking early life stress and epilepsy; to discuss the direct studies examining early life stress effects in current models of limbic seizures/epilepsy; and to suggest priorities for future research.

Laser interstitial thermal therapy for the treatment of epilepsy: evidence to date

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Shukla ND

2017-09-01

Full Text Available Navika D Shukla, Allen L Ho, Arjun V Pendharkar, Eric S Sussman, Casey H Halpern Department of Neurosurgery, Stanford University, Stanford, CA, USA Abstract: Medically intractable epilepsy is associated with increased morbidity and mortality. For those with focal epilepsy and correlated electrophysiological or radiographic features, open surgical resection can achieve high rates of seizure control, but can be associated with neurologic deficits and cognitive effects. Recent innovations have allowed for more minimally invasive methods of surgical seizure control such as magnetic resonance-guided laser interstitial therapy (MRgLITT. MRgLITT achieves the goal of ablating seizure foci while preserving neuropsychological function and offering real-time feedback and monitoring of tissue ablation. This review summarizes the utilization of MRgLITT for mesial temporal lobe epilepsy and other seizure disorders. Overall, the efficacy of MRgLITT is comparable to that of open surgery and offers a less invasive approach in patients with significantly less morbidity. Keywords: laser ablation, MRgLITT, mesial temporal lobe epilepsy, epilepsy surgery, corpus callostomy

Chronic Granulomatous Herpes Encephalitis in a Child with Clinically Intractable Epilepsy

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James R. Hackney

2012-01-01

Full Text Available Most patients with herpes simplex virus Type I encephalitis experience an acute, monophasic illness. Chronic encephalitis is much less common, and few late relapses are associated with intractable seizure disorders. A 10-year-old boy was admitted to our institution for intractable epilepsy as part of an evaluation for epilepsy surgery. His history was significant for herpes meningitis at age 4 months. At that time, he presented to an outside hospital with fever for three days, with acyclovir treatment beginning on day 4 of his 40-day hospital course. He later developed infantile spasms and ultimately a mixed seizure disorder. Video electroencephalogram showed a Lennox-Gastaut-type pattern with frequent right frontotemporal spikes. Imaging studies showed an abnormality in the right frontal operculum. Based on these findings, he underwent a right frontal lobectomy. Neuropathology demonstrated chronic granulomatous inflammation with focal necrosis and mineralizations. Scattered lymphocytes, microglial nodules and nonnecrotizing granulomas were present with multinucleated giant cells. Immunohistochemistry for herpes simplex virus showed focal immunoreactivity. After undergoing acyclovir therapy, he returned to baseline with decreased seizure frequency. This rare form of herpes encephalitis has only been reported in children, but the initial presentation of meningitis and the approximate 10-year-time interval in this case are unusual.

Sudden Unexpected Death in Epilepsy Among Patients With Benign Childhood Epilepsy With Centrotemporal Spikes.

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Doumlele, Kyra; Friedman, Daniel; Buchhalter, Jeffrey; Donner, Elizabeth J; Louik, Jay; Devinsky, Orrin

2017-06-01

Children with benign epilepsy with centrotemporal spikes (BECTS) have traditionally been considered to have a uniformly good prognosis. However, benign may be a misnomer because BECTS is linked to cognitive deficits, a more severe phenotype with intractable seizures, and the potential for sudden unexpected death in epilepsy (SUDEP). To determine if cases of BECTS are present in the North American SUDEP Registry (NASR). The NASR is a clinical and biospecimen repository established in 2011 to promote SUDEP research. The NASR database, which includes medical records, results of electroencephalographic tests, and interviews with family members of patients with epilepsy who died suddenly without other identifiable causes of death, was queried from June 3, 2011, to June 3, 2016, for cases of BECTS. The patients with epilepsy had died suddenly without other identifiable causes of death (eg, drowning, trauma, exposure to toxic substances, or suicide); SUDEP classification was determined by the consensus of 2 epileptologists. Cases of SUDEP among children who received a diagnosis of BECTS among patients reported in the NASR. Three boys (median age at death, 12 years; range, 9-13 years) who received a diagnosis of BECTS by their pediatric epileptologist or neurologists were identified among 189 cases reported in the NASR. The median age of epilepsy onset was 5 years (range, 3-11 years), and the median duration of epilepsy was 4 years (range, 1-10 years). Two deaths were definite SUDEP, and 1 was probable SUDEP. Independent review of clinical and electroencephalographic data supported the diagnosis of BECTS in all 3 patients. None of the patients was prescribed antiseizure drugs, either owing to physician recommendation or mutual decision by the physician and parents. All 3 patients were found dead in circumstances typical of SUDEP. The 3 patients spanned the spectrum of BECTS severity: 1 had only a few seizures, 1 had more than 30 focal motor seizures, and 1 had 4 witnessed

Zonisamide in the management of epilepsy in the elderly

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Romigi A

2015-06-01

Full Text Available Andrea Romigi,1,2 Eti A Femia,3 Cinzia Fattore,4 Giuseppe Vitrani,1 Giancarlo Di Gennaro,1 Valentina Franco4 1Istituto Neurologico Mediterraneo, IRCCS Neuromed, Pozzilli (IS, Italy; 2Neurophysiopathology Department, University of Rome Tor Vergata, Rome, Italy; 3Dipartimento di Scienze della Salute, Università degli Studi di Milano, Milano, Italy; 4Clinical Trial Centre & Antiepileptic Drugs, C. Mondino National Neurological Institute, Pavia, Italy Abstract: Zonisamide (ZNS, a second-generation antiepileptic drug, indicated as add-on treatment of focal epilepsy, has been recently approved as monotherapy for the treatment of partial seizures in adults affected by newly diagnosed epilepsy in Europe. Evidence on the efficacy and tolerability of antiepileptic drugs in the elderly is still lacking as these patients are frequently excluded from clinical trials. Here, a comprehensive overview of available data regarding the use of ZNS in the treatment of epilepsy in elderly people is provided. In a pooled analysis conducted in patients aged ≥65 years, no new/unexpected safety findings have emerged. Few data from uncontrolled investigations suggest that ZNS may be effective and well tolerated when administered as monotherapy or adjunctive antiepileptic treatment in the elderly. However, evidence from these observational studies is less than satisfactory, and randomized controlled trials focused on these patients are still needed. Keywords: zonisamide, elderly, epilepsy, safety, efficacy

Family Stigma Associated With Epilepsy: A Qualitative Study

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Reza nabi amjad

2017-03-01

Full Text Available Introduction: Harmful nature of epilepsy can affect the patient and their parent. Stigma, arising from it, affects the patient and their family. To relieve it understanding the experiences of the parent are useful. This study was aimed at understanding the experiences of parent of child with epilepsy in Iran. Methods: In this interpretative phenomenological study, 10 parents who took care of their child with epilepsy were participated. Data were collected through in-depth semi-structured interviews. After transcription, data were analyzed using Van Manen’s method. Results: Family stigma emerged as a main theme in data analysis with three subthemes including becoming verbally abusive, a dull and heavy shadowed look, and associates interference. Conclusion: Family stigma is a major challenge for parents of child with epilepsy need to special attention by health system. Nurses, as a big part of the system, can play an important role to manage this problem.

Prevalence and etiology of epilepsy in a Norwegian county-A population based study.

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Syvertsen, Marte; Nakken, Karl Otto; Edland, Astrid; Hansen, Gunnar; Hellum, Morten Kristoffer; Koht, Jeanette

2015-05-01

Epilepsy represents a substantial personal and social burden worldwide. When addressing the multifaceted issues of epilepsy care, updated epidemiologic studies using recent guidelines are essential. The aim of this study was to find the prevalence and causes of epilepsy in a representative Norwegian county, implementing the new guidelines and terminology suggested by the International League Against Epilepsy (ILAE). Included in the study were all patients from Buskerud County in Norway with a diagnosis of epilepsy at Drammen Hospital and the National Center for Epilepsy at Oslo University Hospital. The study period was 1999-2014. Patients with active epilepsy were identified through a systematic review of medical records, containing information about case history, electroencephalography (EEG), cerebral magnetic resonance imaging (MRI), genetic tests, blood samples, treatment, and other investigations. Epilepsies were classified according to the revised terminology suggested by the ILAE in 2010. In a population of 272,228 inhabitants, 1,771 persons had active epilepsy. Point prevalence on January 1, 2014 was 0.65%. Of the subjects registered with a diagnostic code of epilepsy, 20% did not fulfill the ILAE criteria of the diagnosis. Epilepsy etiology was structural-metabolic in 43%, genetic/presumed genetic in 20%, and unknown in 32%. Due to lack of information, etiology could not be determined in 4%. Epilepsy is a common disorder, affecting 0.65% of the subjects in this cohort. Every fifth subject registered with a diagnosis of epilepsy was misdiagnosed. In those with a reliable epilepsy diagnosis, every third patient had an unknown etiology. Future advances in genetic research will probably lead to an increased identification of genetic and hopefully treatable causes of epilepsy. Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.

Managing Epilepsy Well: Emerging e-Tools for epilepsy self-management.

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Shegog, Ross; Bamps, Yvan A; Patel, Archna; Kakacek, Jody; Escoffery, Cam; Johnson, Erica K; Ilozumba, Ukwuoma O

2013-10-01

The Managing Epilepsy Well (MEW) Network was established in 2007 by the Centers for Disease Control and Prevention Epilepsy Program to expand epilepsy self-management research. The network has employed collaborative research strategies to develop, test, and disseminate evidence-based, community-based, and e-Health interventions (e-Tools) for epilepsy self-management for people with epilepsy, caregivers, and health-care providers. Since its inception, MEW Network collaborators have conducted formative studies (n=7) investigating the potential of e-Health to support epilepsy self-management and intervention studies evaluating e-Tools (n=5). The MEW e-Tools (the MEW website, WebEase, UPLIFT, MINDSET, and PEARLS online training) and affiliated e-Tools (Texting 4 Control) are designed to complement self-management practices in each phase of the epilepsy care continuum. These tools exemplify a concerted research agenda, shared methodological principles and models for epilepsy self-management, and a communal knowledge base for implementing e-Health to improve quality of life for people with epilepsy. © 2013.

MRI segmentation in the diagnosis and clinical correlations of temporal lobe epilepsy

International Nuclear Information System (INIS)

He Huijin; Shen Tianzhen; Chen Xingrong; Feng Xiaoyuan; Jiang Chengchuan

2004-01-01

Objective: To study the different patterns of hippocampal atrophy by MRI segmental analysis and to investigate the etiology and pathogenesis of temporal lobe epilepsy. Methods: GE 1.5 T Signa Horizon LX MRI scanner was used. Oblique coronal T 1 weighted images perpendicular to the long axis of the hippocampus were obtained. The mesial temporal structures were divided into four parts: the amygdala, hippocampal head, body and tail. MRI patterns of atrophy in 50 patients with histologically confirmed hippocampal sclerosis were investigated by MRI volumetric measurement and segmental analysis, and the differences of clinical features and surgical outcome in different groups were compared. Results: Diffuse hippocampal atrophy was found in 22 of 50 patients (44%), 5 of the 50 patients (10%) showed diffuse atrophy involving both the amygdala and hippocampus. 20 of the 50 patients (40%) had hippocampal focal atrophy and 8 of 50 patients (16%) had no obvious atrophy. 38 of 50 (76%) hippocampal sclerosis had atrophy in the hippocampal body, 29 of 50 (58%) had hippocampal head atrophy, 24 of 50 (48%) had hippocampal tail atrophy, and the least involved part was the amygdala (16%, 8/50). 10 patients who had normal hippocampal volume showed focal hippocampal atrophy by segmental analysis. Various patterns of hippocampal atrophy were found to be statistically related to the duration of epilepsy, the frequency of seizure and the outcome of surgery, respectively (P 0.05). Conclusion: MRI segmental analysis can improve the diagnostic sensitivity of temporal lobe epilepsy and help to investigate its etiology and pathogenesis. (author)

The social competence and behavioral problem substrate of new- and recent-onset childhood epilepsy.

Science.gov (United States)

Almane, Dace; Jones, Jana E; Jackson, Daren C; Seidenberg, Michael; Hermann, Bruce P

2014-02-01

This study examined patterns of syndrome-specific problems in behavior and competence in children with new- or recent-onset epilepsy compared with healthy controls. Research participants consisted of 205 children aged 8-18, including youth with recent-onset epilepsy (n=125, 64 localization-related epilepsy [LRE] and 61 idiopathic generalized epilepsy [IGE]) and healthy first-degree cousin controls (n=80). Parents completed the Child Behavior Checklist for children aged 6-18 (CBCL/6-18) from the Achenbach System of Empirically Based Assessment (ASEBA). Dependent variables included Total Competence, Total Problems, Total Internalizing, Total Externalizing, and Other Problems scales. Comparisons of children with LRE and IGE with healthy controls were examined followed by comparisons of healthy controls with those having specific epilepsy syndromes of LRE (BECTS, Frontal/Temporal Lobe, and Focal NOS) and IGE (Absence, Juvenile Myoclonic, and IGE NOS). Children with LRE and/or IGE differed significantly (pcompetence (Total Competence including School and Social). Similarly, children with specific syndromes of LRE and IGE differed significantly (pcompetence (Total Competence including School). Only on the Thought Problems scale were there syndrome differences. In conclusion, children with recent-onset epilepsy present with significant behavioral problems and lower competence compared with controls, with little syndrome specificity whether defined broadly (LRE and IGE) or narrowly (specific syndromes of LRE and IGE). Copyright © 2013 Elsevier Inc. All rights reserved.

Microscopic mild focal cortical dysplasia in temporal lobe dual pathology: an electrocorticography study.

Science.gov (United States)

Morales Chacón, L; Estupiñán, B; Lorigados Pedre, L; Trápaga Quincoses, O; García Maeso, I; Sanchez, A; Bender del Busto, J; Garcia, M E; Baez Martin, M; Zaldivar, M; Gómez, A; Orozco, S; Rocha Arrieta, L

2009-10-01

Associations between electrophysiological and histological findings might provide an insight into the epileptogenicity of mild focal cortical dysplasia (FCD) in patients with temporal lobe epilepsy (TLE) and a dual pathology. A total of 22 patients with pharmacoresistant TLE were included in the study, 16 of them with histologically confirmed hippocampal sclerosis (HS) associated with neocortical temporal mild Palmini Type-I FCD subtypes and 6 with HS. Intraoperative electrocorticography (ECoG) recordings were analysed for epileptiform discharge frequency and morphology. Associations between histological, and electrocorticography pattern findings in these patients were analysed. Electroclinical outcomes in these patients were also evaluated. Neocortical areas with mild Palmini Type-I FCD showed a significantly higher spike frequency (SF) recorded in the inferior temporal gyrus than those neocortical areas in patients with HS. There was a tendency to higher spike frequency and lower amplitude in neocortical areas with histopathologic subtype IB FCD in relation with IA during intraoperative ECoG. Post-SF excision and amplitude were significantly lower during neocortical post-excision intraoperative ECoG than during neocortical pre-excision recording. There was no difference found in the clinical outcome between patients with and without FCD. Intraoperative electrocorticographic interictal spike frequency recorded in the neocortical inferior temporal gyrus may help to characterize the histopathologic subtypes of mild Palmini Type-I FCD in patients with temporal lobe epilepsy (TLE) and a dual pathology. Our data support the epileptogenicity of neocortical mild FCD in TLE and assessments of ECoG patterns are relevant to determine the extent of the resection in these patients which can influence the electroclinical outcome.

Talking about epilepsy: Challenges parents face when communicating with their child about epilepsy and epilepsy-related issues.

Science.gov (United States)

O'Toole, Stephanie; Lambert, Veronica; Gallagher, Pamela; Shahwan, Amre; Austin, Joan K

2016-04-01

The aim of this qualitative study was to explore the challenges that parents of children with epilepsy experienced when engaging in dialog with their child about epilepsy and epilepsy-related issues. Using a qualitative exploratory approach, interviews were conducted with 34 parents of children with epilepsy (aged 6-16 years), consisting of 27 mothers and 7 fathers. Data were transcribed verbatim and thematically analyzed. Findings revealed five main themes: normalizing epilepsy, the invisibility of epilepsy, information concealment, fear of misinforming the child, and difficulty in discussing particular epilepsy-related issues. Many of the communicative challenges experienced by parents impacted on their ability to engage openly in parent-child dialog about epilepsy in the home. Parents face specific challenges when choosing to communicate with their child about epilepsy, relating to creating a sense of normality, reducing fear of causing their child worry, and having a lack of epilepsy-related knowledge. Healthcare professionals who work closely with families living with epilepsy should remain mindful of the importance of discussing family communication surrounding epilepsy and the challenges parents of children with epilepsy face when talking about epilepsy within the home. Copyright © 2016 Elsevier Inc. All rights reserved.

CDKL5 mutations as a cause of severe epilepsy in infancy: clinical and electroencephalographic long-term course in 4 patients.

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Jähn, Johanna; Caliebe, Almuth; von Spiczak, Sarah; Boor, Rainer; Stefanova, Irina; Stephani, Ulrich; Helbig, Ingo; Muhle, Hiltrud

2013-07-01

CDKL5 mutations cause severe epilepsy in infancy with subsequent epileptic encephalopathy. As yet, few studies report on long-term observations in patients with CDKL5-related epileptic encephalopathy. In this study, we describe the evolution of the epilepsy phenotype and the electroencephalographic (EEG) features in 4 patients during a maximum observation period of 22 years. All 4 patients had epilepsy starting with focal seizures in the first 3 months of life, evolving to epileptic spasms between the ages of 2 and 6 years and later on to tonic seizures. In 3 patients, epilepsy was resistant to antiepileptic therapy. Although there was no common EEG pattern in all patients, late hypsarrhythmia until the age of 9 years was observed in 2 patients. CDKL5-related epileptic encephalopathies are a group of refractory seizure disorders starting in early infancy. The phenomenon of late hypsarrhythmia may help define a subgroup of patients with severe and adverse outcomes.

Epilepsy-associated stigma in Bolivia: a community-based study among the Guarani population: an International League Against Epilepsy/International Bureau for Epilepsy/World Health Organization Global Campaign Against Epilepsy Regional Project.

Science.gov (United States)

Bruno, Elisa; Bartoloni, Alessandro; Sofia, Vito; Rafael, Florentina; Magnelli, Donata; Padilla, Sandra; Quattrocchi, Graziella; Bartalesi, Filippo; Segundo, Higinio; Zappia, Mario; Preux, Pierre-Marie; Nicoletti, Alessandra

2012-09-01

Epilepsy is associated with a significant burden of social stigma that appears to be influenced by psychosocial and cultural factors. Stigma has a negative effect on the management of people with epilepsy (PWE), representing one of the major factors that contribute to the burden of epilepsy. To assess stigma perception among the Guarani population, one hundred thirty-two people living in Guaraní communities in Bolivia were invited to complete the Stigma Scale of Epilepsy questionnaire. The main determinants of stigma identified were: the fear linked to loss of control, the feelings of sadness and pity toward PWE, the difficulties faced by PWE in the professional and relationship fields, the level of education and type of seizure. Our study pointed out that, in this population, PWE face difficulties in everyday life because of epilepsy-associated stigma and the results attest to the importance of promoting community-based educational programs aimed at reducing the stigmatization process. Copyright © 2012 Elsevier Inc. All rights reserved.

Neuronal autoantibodies in epilepsy patients with peri-ictal autonomic findings.

Science.gov (United States)

Baysal-Kirac, Leyla; Tuzun, Erdem; Erdag, Ece; Ulusoy, Canan; Vanli-Yavuz, Ebru Nur; Ekizoglu, Esme; Peach, Sian; Sezgin, Mine; Bebek, Nerses; Gurses, Candan; Gokyigit, Aysen; Vincent, Angela; Baykan, Betul

2016-03-01

Autonomic dysfunction has frequently been reported in autoimmune encephalitis associated with seizures and there is growing evidence that epilepsy patients may display neuronal autoantibodies (NAAb). The aim of this study was to investigate the frequency of NAAb in epilepsy patients with peri-ictal autonomic findings. Fifty-eight patients (37 women/21 men; average age of 34.2 ± 9.9 years and epilepsy duration of 19.1 ± 9.6 years) who had at least one video-EEG recorded focal or secondary generalized seizure with clear-cut documented peri-ictal autonomic findings, or consistently reported seizures with autonomic semiology, were included. NAAb were tested by RIA or cell based assays. NAAb were present in 17 of 58 (29.3%) patients. Among seropositive patients, antibodies were directed against N-methyl-D-aspartate receptor (NMDAR) in 5 (29%), contactin-associated protein-like 2 (CASPR2) in 5 (29%), uncharacterized voltage gated potassium channel (VGKC)-complex antigens in 3 (18%), glutamic acid decarboxylase (GAD) in 2 (12%), glycine receptor (GLYR) in one (6%) and type A gamma aminobutyric acid receptor (GABAAR) in one patient (6%). Peri-ictal gastrointestinal manifestations, piloerection, ictal fever, urinary urge, and cough occurred more commonly in the seropositive group. The prevalences of psychotic attacks and status epilepticus were significantly increased in the seropositive group. Seropositivity prevalence in our patient group with peri-ictal autonomic findings is higher than other previously reported epilepsy cohorts. In our study, ictal fever-VGKC-complex antibody and pilomotor seizure-GABAAR antibody associations were documented for the first time. Chronic epilepsy patients with peri-ictal autonomic semiology, history of status epilepticus and psychotic disorder may benefit from autoantibody screening.

Fertility Treatment and Childhood Epilepsy - a Nationwide Cohort Study

DEFF Research Database (Denmark)

Kettner, Laura Ozer; Kesmodel, Ulrik Schiøler; Ramlau-Hansen, Cecilia Høst

2017-01-01

BACKGROUND: Fertility treatment includes hormonal stimulation of the woman and in vitro manipulation of gametes and embryos that may influence prenatal brain development. We aimed to investigate the association between fertility treatment and childhood epilepsy, including specific types of treatm......BACKGROUND: Fertility treatment includes hormonal stimulation of the woman and in vitro manipulation of gametes and embryos that may influence prenatal brain development. We aimed to investigate the association between fertility treatment and childhood epilepsy, including specific types...... of treatment and indications, as well as subtypes of epilepsy. METHODS: In this nationwide birth cohort study, we included all pregnancies in Denmark resulting in live-born singletons, 1995-2003. Children conceived by fertility treatment and children developing epilepsy (until 2013) were identified from Danish...... national registers. RESULTS: A total of 565,116 pregnancies were included; 8,071 children (1.4%) developed epilepsy. Children conceived after ovulation induction or intrauterine insemination had a slightly higher risk of childhood epilepsy (hazard ratio [HR]: 1.15; 95% confidence interval [CI]: 1.00, 1...

[Nootropics and antioxidants in the complex therapy of symptomatic posttraumatic epilepsy].

Science.gov (United States)

Savenkov, A A; Badalian, O L; Avakian, G N

2013-01-01

To study the possibility of application of nootropics and antioxidants in the complex antiepileptic therapy, we examined 75 patients with symptomatic focal posttraumatic epilepsy. A statistically significant reduction in the number of epileptic seizures, improvement of cognitive function and quality of life of the patients as well as a decrease in the severity of depression and epileptic changes in the EEG were identified. The potentiation of antiepileptic activity of basic drugs, normalization of brain's electrical activity and reduction in EEG epileptiform activity, in particular coherent indicators of slow-wave activity, were noted after treatment with the antioxidant mexidol. A trend towards the improvement of neuropsychological performance and quality of life was observed. There was a lack of seizure aggravation typical of many nootropic drugs. Thus, phenotropil and mexidol can be recommended for complex treatment of symptomatic posttraumatic epilepsy.

Epilepsy surgery for epileptic encephalopathy as a sequela of herpes simplex encephalitis: case report.

Science.gov (United States)

Taskin, Birce Dilge; Tanji, Kurenai; Feldstein, Neil A; McSwiggan-Hardin, Maureen; Akman, Cigdem I

2017-07-01

Herpes simplex virus (HSV) encephalitis can manifest with different clinical presentations, including acute monophasic illness and biphasic chronic granulomatous HSV encephalitis. Chronic encephalitis is much less common, and very rare late relapses are associated with intractable epilepsy and progressive neurological deficits with or without evidence of HSV in the cerebrospinal fluid. The authors report on an 8-year-old girl with a history of treated HSV-1 encephalitis when she was 13 months of age and focal epilepsy when she was 2 years old. Although free of clinical seizures, when she was 5, she experienced behavioral and academic dysfunction, which was later attributed to electrographic focal seizures and worsening electroencephalography (EEG) findings with electrical status epilepticus during slow-wave sleep (ESES). Following a right temporal lobectomy, chronic granulomatous encephalitis was diagnosed. The patient's clinical course improved with the resolution of seizures and EEG abnormalities.

Atypical language representation in children with intractable temporal lobe epilepsy.

Science.gov (United States)

Maulisova, Alice; Korman, Brandon; Rey, Gustavo; Bernal, Byron; Duchowny, Michael; Niederlova, Marketa; Krsek, Pavel; Novak, Vilem

2016-05-01

This study evaluated language organization in children with intractable epilepsy caused by temporal lobe focal cortical dysplasia (FCD) alone or dual pathology (temporal lobe FCD and hippocampal sclerosis, HS). We analyzed clinical, neurological, fMRI, neuropsychological, and histopathologic data in 46 pediatric patients with temporal lobe lesions who underwent excisional epilepsy surgery. The frequency of atypical language representation was similar in both groups, but children with dual pathology were more likely to be left-handed. Atypical receptive language cortex correlated with lower intellectual capacity, verbal abstract conceptualization, receptive language abilities, verbal working memory, and a history of status epilepticus but did not correlate with higher seizure frequency or early seizure onset. Histopathologic substrate had only a minor influence on neuropsychological status. Greater verbal comprehension deficits were noted in children with atypical receptive language representation, a risk factor for cognitive morbidity. Copyright © 2016 Elsevier Inc. All rights reserved.

Atypical language laterality is associated with large-scale disruption of network integration in children with intractable focal epilepsy.

Science.gov (United States)

Ibrahim, George M; Morgan, Benjamin R; Doesburg, Sam M; Taylor, Margot J; Pang, Elizabeth W; Donner, Elizabeth; Go, Cristina Y; Rutka, James T; Snead, O Carter

2015-04-01

Epilepsy is associated with disruption of integration in distributed networks, together with altered localization for functions such as expressive language. The relation between atypical network connectivity and altered localization is unknown. In the current study we tested whether atypical expressive language laterality was associated with the alteration of large-scale network integration in children with medically-intractable localization-related epilepsy (LRE). Twenty-three right-handed children (age range 8-17) with medically-intractable LRE performed a verb generation task in fMRI. Language network activation was identified and the Laterality index (LI) was calculated within the pars triangularis and pars opercularis. Resting-state data from the same cohort were subjected to independent component analysis. Dual regression was used to identify associations between resting-state integration and LI values. Higher positive values of the LI, indicating typical language localization were associated with stronger functional integration of various networks including the default mode network (DMN). The normally symmetric resting-state networks showed a pattern of lateralized connectivity mirroring that of language function. The association between atypical language localization and network integration implies a widespread disruption of neural network development. These findings may inform the interpretation of localization studies by providing novel insights into reorganization of neural networks in epilepsy. Copyright © 2015 Elsevier Ltd. All rights reserved.

Adult Prevalence of Epilepsy in Spain: EPIBERIA, a Population-Based Study

Science.gov (United States)

Serrano-Castro, Pedro J.; Mauri-Llerda, Jose Angel; Hernández-Ramos, Francisco José; Sánchez-Alvarez, Juan Carlos; Parejo-Carbonell, Beatriz; Quiroga-Subirana, Pablo; Vázquez-Gutierrez, Fernando; Santos-Lasaosa, Sonia; Mendez-Lucena, Carolina; Redondo-Verge, Luis; Tejero-Juste, Carlos; Morandeira-Rivas, Clara; Sancho-Rieger, Jerónimo; Matías-Guiu, Jorge

2015-01-01

Background. This study assesses the lifetime and active prevalence of epilepsy in Spain in people older than 18 years. Methods. EPIBERIA is a population-based epidemiological study of epilepsy prevalence using data from three representative Spanish regions (health districts in Zaragoza, Almería, and Seville) between 2012 and 2013. The study consisted of two phases: screening and confirmation. Participants completed a previously validated questionnaire (EPIBERIA questionnaire) over the telephone. Results. A total of 1741 valid questionnaires were obtained, including 261 (14.99%) raising a suspicion of epilepsy. Of these suspected cases, 216 (82.75%) agreed to participate in phase 2. Of the phase 2 participants, 22 met the International League Against Epilepsy's diagnostic criteria for epilepsy. The estimated lifetime prevalence, adjusted by age and sex per 1,000 people, was 14.87 (95% CI: 9.8–21.9). Active prevalence was 5.79 (95% CI: 2.8–10.6). No significant age, sex, or regional differences in prevalence were detected. Conclusions. EPIBERIA provides the most accurate estimate of epilepsy prevalence in the Mediterranean region based on its original methodology and its adherence to ILAE recommendations. We highlight that the lifetime prevalence and inactive epilepsy prevalence figures observed here were compared to other epidemiological studies. PMID:26783554

Stress and epilepsy: a population-based cohort study of epilepsy in parents who lost a child

DEFF Research Database (Denmark)

Christensen, Jakob; Li, Jiong; Vestergaard, Mogens

2007-01-01

OBJECTIVE: The goal of the study described here was to study the risk for epilepsy in parents exposed to severe stress caused by loss of a child. METHODS: The risk of being diagnosed with epilepsy (Danish National Hospital Register) in a cohort of parents who had lost a child under the age of 18...... was compared with the risk among parents who had not lost a child. RESULTS: The adjusted relative risk (RR) of epilepsy in parents who had lost a child was 1.50 (95% CI: 1.21-1.86). The RR was modified by time since bereavement and was 2.46 (95% CI: 1.49-4.07) in mothers and 1.92 (95% CI: 1.09-3.36) in fathers...... within the first 3 years of loss of a child, and 2.10 (95% CI: 1.53-2.88) in mothers and 0.66 (95% CI: 0.41-1.06) in fathers 4 to 18 years after loss. CONCLUSIONS: Stress was associated with a moderately increased risk of being diagnosed with epilepsy. Udgivelsesdato: 2007-Nov...

A cohort study of epilepsy among 665,000 insured dogs

DEFF Research Database (Denmark)

Heske, L.; Nødtvedt, A.; Jäderlund, K. Hultin

2014-01-01

The main objective of this study was to estimate the incidence and mortality rates of epilepsy in a large population of insured dogs and to evaluate the importance of a variety of risk factors. Survival time after a diagnosis of epilepsy was also investigated. The Swedish animal insurance database...... used in this study has previously been helpful in canine epidemiological investigations. More than 2,000,000 dog-years at-risk (DYAR) were available in the insurance database. In total, 5013 dogs had at least one veterinary care claim for epilepsy, and 2327 dogs were euthanased or died because...... of epilepsy. Based on veterinary care claims the incidence rate of epilepsy (including both idiopathic and symptomatic cases) was estimated to be 18 per 10,000 DYAR. Dogs were followed up until they were 10 (for life insurance claims) or 12 years of age (veterinary care claims). Among the 35 most common...

Dominant KCNA2 mutation causes episodic ataxia and pharmacoresponsive epilepsy.

Science.gov (United States)

Corbett, Mark A; Bellows, Susannah T; Li, Melody; Carroll, Renée; Micallef, Silvana; Carvill, Gemma L; Myers, Candace T; Howell, Katherine B; Maljevic, Snezana; Lerche, Holger; Gazina, Elena V; Mefford, Heather C; Bahlo, Melanie; Berkovic, Samuel F; Petrou, Steven; Scheffer, Ingrid E; Gecz, Jozef

2016-11-08

To identify the genetic basis of a family segregating episodic ataxia, infantile seizures, and heterogeneous epilepsies and to study the phenotypic spectrum of KCNA2 mutations. A family with 7 affected individuals over 3 generations underwent detailed phenotyping. Whole genome sequencing was performed on a mildly affected grandmother and her grandson with epileptic encephalopathy (EE). Segregating variants were filtered and prioritized based on functional annotations. The effects of the mutation on channel function were analyzed in vitro by voltage clamp assay and in silico by molecular modeling. KCNA2 was sequenced in 35 probands with heterogeneous phenotypes. The 7 family members had episodic ataxia (5), self-limited infantile seizures (5), evolving to genetic generalized epilepsy (4), focal seizures (2), and EE (1). They had a segregating novel mutation in the shaker type voltage-gated potassium channel KCNA2 (CCDS_827.1: c.765_773del; p.255_257del). A rare missense SCN2A (rs200884216) variant was also found in 2 affected siblings and their unaffected mother. The p.255_257del mutation caused dominant negative loss of channel function. Molecular modeling predicted repositioning of critical arginine residues in the voltage-sensing domain. KCNA2 sequencing revealed 1 de novo mutation (CCDS_827.1: c.890G>A; p.Arg297Gln) in a girl with EE, ataxia, and tremor. A KCNA2 mutation caused dominantly inherited episodic ataxia, mild infantile-onset seizures, and later generalized and focal epilepsies in the setting of normal intellect. This observation expands the KCNA2 phenotypic spectrum from EE often associated with chronic ataxia, reflecting the marked variation in severity observed in many ion channel disorders. © 2016 American Academy of Neurology.

Association of ABCB1 C3435T polymorphism with phenobarbital resistance in Thai patients with epilepsy.

Science.gov (United States)

Keangpraphun, T; Towanabut, S; Chinvarun, Y; Kijsanayotin, P

2015-06-01

One-third of patients with epilepsy are resistant to anti-epileptic drugs (AEDs). Drug-resistant epilepsy is believed to be multifactorial involving both genetic and non-genetic factors. Genetic variations in the ABCB1 gene encoding the drug efflux transporter, p-glycoprotein (p-gp), may influence the interindividual variability in AED response by limiting drugs from reaching their target. Phenobarbital (PB), one of the most cost-effective and widely used AEDs in developing countries, has been reported to be transported by p-gp. This study aimed to investigate the association of a genetic variant, ABCB1 3435C>T, and non-genetic factors with phenobarbital response in Thai patients with epilepsy. One hundred and ten Thai patients with epilepsy who were treated with PB maintenance doses were enrolled in this study. Two phenotypic groups, PB-responsive epilepsy and PB-resistant epilepsy, were defined according to the International League Against Epilepsy (ILAE) criteria. Subjects were genotyped for ABCB1 3435C>T (rs1045642). Multiple logistic regression analysis was tested for the association of ABCB1 3435C>T polymorphism and non-genetic factors with PB response. Sixty-two PB-responsive epilepsy subjects and 48 PB-resistant epilepsy subjects were identified. All genotype frequencies of the ABCB1 3435C>T SNP were consistent with the Hardy-Weinberg equilibrium (P > 0·05). The ABCB1 3435C>T polymorphism and type of epilepsy were associated with response to PB. Patients with PB-resistant epilepsy had a significantly higher frequency of ABCB1 3435CC genotype and had focal epilepsy more often than patients with PB-responsive epilepsy (adjusted OR = 3·962, 95% CI = 1·075-14·610, P-value = 0·039; adjusted OR = 5·936, 95% CI = 2·272-15·513, P-value phenobarbital. © 2015 John Wiley & Sons Ltd.

Characteristic phasic evolution of convulsive seizure in PCDH19-related epilepsy.

Science.gov (United States)

Ikeda, Hiroko; Imai, Katsumi; Ikeda, Hitoshi; Shigematsu, Hideo; Takahashi, Yukitoshi; Inoue, Yushi; Higurashi, Norimichi; Hirose, Shinichi

2016-03-01

PCDH19-related epilepsy is a genetic disorder that was first described in 1971, then referred to as "epilepsy and mental retardation limited to females". PCDH19 has recently been identified as the responsible gene, but a detailed characterization of the seizure manifestation based on video-EEG recording is still limited. The purpose of this study was to elucidate features of the seizure semiology in children with PCDH19-related epilepsy. To do this, ictal video-EEG recordings of 26 convulsive seizures in three girls with PCDH19-related epilepsy were analysed. All seizures occurred in clusters, mainly during sleep accompanied by fever. The motor manifestations consisted of six sequential phases: "jerk", "reactive", "mild tonic", "fluttering", "mild clonic", and "postictal". Some phases were brief or lacking in some seizures, whereas others were long or pronounced. In the reactive phase, the patients looked fearful or startled with sudden jerks and turned over reactively. The tonic and clonic components were less intense compared with those of typical tonic-clonic seizures in other types of epilepsy. The fluttering phase was characterised initially by asymmetric, less rhythmic, and less synchronous tremulous movement and was then followed by the subtle clonic phase. Subtle oral automatism was observed in the postictal phase. The reactive, mild tonic, fluttering and mild clonic phases were most characteristic of seizures of PCDH19-related epilepsy. Ictal EEG started bilaterally and was symmetric in some patients but asymmetric in others. It showed asymmetric rhythmic discharges in some seizures at later phases. The electroclinical pattern of the phasic evolution of convulsive seizure suggests a focal onset seizure with secondary generalisation. Based on our findings, we propose that the six unique sequential phases in convulsive seizures suggest the diagnosis of PCDH19-related epilepsy when occurring in clusters with or without high fever in girls. [Published with

Efficacy of the Ketogenic Diet for the Treatment of Refractory Childhood Epilepsy: Cerebrospinal Fluid Neurotransmitters and Amino Acid Levels.

Science.gov (United States)

Sariego-Jamardo, Andrea; García-Cazorla, Angels; Artuch, Rafael; Castejón, Esperanza; García-Arenas, Dolores; Molero-Luis, Marta; Ormazábal, Aida; Sanmartí, Francesc Xavier

2015-11-01

The mechanisms of the ketogenic diet remain unclear, but several predictors of response have been proposed. We aimed is to study the relationship between the etiology of epilepsy, cerebrospinal fluid neurotransmitters, pterins, and amino acids, and response to a ketogenic diet. We studied 60 patients who began classic ketogenic diet treatment for refractory epilepsy. In 24 of 60 individuals, we analyzed cerebrospinal fluid neurotransmitters, pterins, and amino acids in baseline conditions. Mean age at epilepsy onset was 24 months, 83.3% were focal epilepsies, and in 51.7% the etiology of the epilepsy was unknown. Six months after initiating the ketogenic diet, it was effective (greater than a 50% reduction in seizure frequency) in 31.6% of patients. We did not find a link between rate of efficacy for the ketogenic diet and etiologies of epilepsy, nor did we find a link between the rate of efficacy for the ketogenic diet and cerebrospinal fluid pterins and biogenic amines concentrations. However, we found statistically significant differences for lysine and arginine values in the cerebrospinal fluid between ketogenic diet responders and nonresponders, but not for the other amino acids analyzed. The values of some amino acids were significantly different in relationship with the ketogenic diet efficacy; however, the epilepsy etiology and the cerebrospinal fluid biogenic amine and pterin values were not. Copyright © 2015 Elsevier Inc. All rights reserved.

Experimental therapy of epilepsy with transcranial magnetic stimulation: lack of additional benefit with prolonged treatment

Directory of Open Access Journals (Sweden)

Brasil-Neto Joaquim P.

2004-01-01

Full Text Available OBJECTIVE: To investigate the effect of three months of low-frequency repetitive transcranial magnetic stimulation (rTMS treatment in intractable epilepsy. METHODS: Five patients (four males, one female; ages 6 to 50 years, were enrolled in the study; their epilepsy could not be controlled by medical treatment and surgery was not indicated. rTMS was performed twice a week for three months; patients kept records of seizure frequency for an equal period of time before, during, and after rTMS sessions. rTMS was delivered to the vertex with a round coil, at an intensity 5 % below motor threshold. During rTMS sessions, 100 stimuli (five series of 20 stimuli, with one-minute intervals between series were delivered at a frequency of 0.3 Hz. RESULTS: Mean daily number of seizures (MDNS decreased in three patients and increased in two during rTMS- one of these was treated for only one month; the best result was achieved in a patient with focal cortical dysplasia (reduction of 43.09 % in MDNS. In the whole patient group, there was a significant (p<0.01 decrease in MDNS of 22.8 %. CONCLUSION: Although prolonged rTMS treatment is safe and moderately decreases MDNS in a group of patients with intractable epilepsy, individual patient responses were mostly subtle and clinical relevance of this method is probably low. Our data suggest, however, that patients with focal cortical lesions may indeed benefit from this novel treatment. Further studies should concentrate on that patient subgroup.

Clinical and MRI features of supratentorial gliomas with adult-onset epilepsy

International Nuclear Information System (INIS)

Hashimoto, Takahiro; Yamaura, Akira; Watanabe, Osamu.

1992-01-01

Although some patients with supratentorial gliomas develop epilepsy in their clinical course, the details of adult-onset epilepsy with gliomas have not been fully evaluated. This paper reports on 15 cases of supratentorial glioma with the sole symptom of adult-onset epilepsy and characterizes their clinical and MRI features. The patients, 5 males and 10 females, developed the first epilepsy at the mean age of 37 years. Generalized seizure was encountered in all cases and focal seizure alone was never seen. Seizure was satisfactorily controlled with anticonvulsants in all except 2 cases. The tumor was located in the frontal lobe (9 cases) or temporal lobe (6 cases). Histologically, there were 12 astrocytomas, 2 glioblastomas, and 1 oligoastrocytoma. Of these, 12 were benign gliomas. Surprisingly, CT scan and MRI revealed tumors larger than predicted. The abnormal intensity region was delineated most prominently on T 2 -weighted SE image and was broader on T 2 -weighted spin echo image than on T 1 -weighted spin echo and inversion recovery image. The authors conclude that gliomas presenting with epilepsy tend to be histologically benign, are predominantly seen in middle-aged women, and are located in the frontal and temporal lobes. Although a tumor may be large enough to be detected on CT scan or MRI, as in the present study, histological examination is needed to establish the diagnosis. Additionally, gliomas with equivocal abnormalities on CT and MRI do evolve despite further neurological deficits, so meticulous evaluation including stereotactic biopsy is the method of choice. Finally, T 2 -weighted SE image in the coronal plane is advocated for patients with adult-onset epilepsy to achieve accurate diagnosis and to initiate early treatment. (author)

Long-term Effectiveness of Antiepileptic Drug Monotherapy in Partial Epileptic Patients: A 7-year Study in an Epilepsy Center in China

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Zhu, Fei; Lang, Sen-Yang; Wang, Xiang-Qing; Shi, Xiao-Bing; Ma, Yun-Feng; Zhang, Xu; Chen, Ya-Nan; Zhang, Jia-Tang

2015-01-01

Background: It is important to choose an appropriate antiepileptic drug (AED) to manage partial epilepsy. Traditional AEDs, such as carbamazepine (CBZ) and valproate (VPA), have been proven to have good therapeutic effects. However, in recent years, a variety of new AEDs have increasingly been used as first-line treatments for partial epilepsy. As the studies regarding the effectiveness of new drugs and comparisons between new AEDs and traditional AEDs are few, it is determined that these are areas in need of further research. Accordingly, this study investigated the long-term effectiveness of six AEDs used as monotherapy in patients with partial epilepsy. Methods: This is a retrospective, long-term observational study. Patients with partial epilepsy who received monotherapy with one of six AEDs, namely, CBZ, VPA, topiramate (TPM), oxcarbazepine (OXC), lamotrigine (LTG), or levetiracetam (LEV), were identified and followed up from May 2007 to October 2014, and time to first seizure after treatment, 12-month remission rate, retention rate, reasons for treatment discontinuation, and adverse effects were evaluated. Results: A total of 789 patients were enrolled. The median time of follow-up was 56.95 months. CBZ exhibited the best time to first seizure, with a median time to first seizure of 36.06 months (95% confidential interval: 30.64–44.07). CBZ exhibited the highest 12-month remission rate (85.55%), which was significantly higher than those of TPM (69.38%, P = 0.006), LTG (70.79%, P = 0.001), LEV (72.54%, P = 0.005), and VPA (73.33%, P = 0.002). CBZ, OXC, and LEV had the best retention rate, followed by LTG, TPM, and VPA. Overall, adverse effects occurred in 45.87% of patients, and the most common adverse effects were memory problems (8.09%), rashes (7.76%), abnormal hepatic function (6.24%), and drowsiness (6.24%). Conclusion: This study demonstrated that CBZ, OXC, and LEV are relatively effective in managing focal epilepsy as measured by time to first seizure

Long-term Effectiveness of Antiepileptic Drug Monotherapy in Partial Epileptic Patients: A 7-year Study in an Epilepsy Center in China

Directory of Open Access Journals (Sweden)

Fei Zhu

2015-01-01

Full Text Available Background: It is important to choose an appropriate antiepileptic drug (AED to manage partial epilepsy. Traditional AEDs, such as carbamazepine (CBZ and valproate (VPA, have been proven to have good therapeutic effects. However, in recent years, a variety of new AEDs have increasingly been used as first-line treatments for partial epilepsy. As the studies regarding the effectiveness of new drugs and comparisons between new AEDs and traditional AEDs are few, it is determined that these are areas in need of further research. Accordingly, this study investigated the long-term effectiveness of six AEDs used as monotherapy in patients with partial epilepsy. Methods: This is a retrospective, long-term observational study. Patients with partial epilepsy who received monotherapy with one of six AEDs, namely, CBZ, VPA, topiramate (TPM, oxcarbazepine (OXC, lamotrigine (LTG, or levetiracetam (LEV, were identified and followed up from May 2007 to October 2014, and time to first seizure after treatment, 12-month remission rate, retention rate, reasons for treatment discontinuation, and adverse effects were evaluated. Results: A total of 789 patients were enrolled. The median time of follow-up was 56.95 months. CBZ exhibited the best time to first seizure, with a median time to first seizure of 36.06 months (95% confidential interval: 30.64-44.07. CBZ exhibited the highest 12-month remission rate (85.55%, which was significantly higher than those of TPM (69.38%, P = 0.006, LTG (70.79%, P = 0.001, LEV (72.54%, P = 0.005, and VPA (73.33%, P = 0.002. CBZ, OXC, and LEV had the best retention rate, followed by LTG, TPM, and VPA. Overall, adverse effects occurred in 45.87% of patients, and the most common adverse effects were memory problems (8.09%, rashes (7.76%, abnormal hepatic function (6.24%, and drowsiness (6.24%. Conclusion: This study demonstrated that CBZ, OXC, and LEV are relatively effective in managing focal epilepsy as measured by time to first

Methylphenidate, cognition, and epilepsy: A 1-month open-label trial.

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Adams, Jesse; Alipio-Jocson, Valerie; Inoyama, Katherine; Bartlett, Victoria; Sandhu, Saira; Oso, Jemima; Barry, John J; Loring, David W; Meador, Kimford J

2017-12-01

Cognitive difficulties are common in epilepsy. Beyond reducing seizures and adjusting antiepileptic medications, no well-validated treatment exists in adults. Methylphenidate is used effectively in children with epilepsy and attention-deficit/hyperactivity disorder, but its effects in adults have not been systematically evaluated. We hypothesized that methylphenidate can safely improve cognition in adults with epilepsy. We detail here the open-label follow-up to a double-blind, placebo-controlled, single-dose study. Thirty epilepsy patients entered a 1-month open-label methylphenidate trial after a double-blind phase. Doses were titrated according to clinical practice and patient tolerance, ranging 20-40 mg/day. Primary measures included: Conners' Continuous Performance Test (CPT), Symbol-Digit Modalities Test (SDMT), and Medical College of Georgia Memory Test (MCG). Secondary measures were: Beck Depression Inventory, 2nd Edition (BDI-II), Beck Anxiety Inventory, Apathy Evaluation Scale (AES), Stimulant Side-Effect Checklist, Adverse Events Profile, Quality of Life in Epilepsy-89 (QOLIE-89), and seizure frequency. Fourteen healthy, nonmedicated controls were tested concurrently. Twenty-eight participants with epilepsy (13 men/15 women) completed the trial. Withdrawals occurred due to anxiety (n = 1) and fatigue (n = 1). Mean age was 36.4 years (range = 20-60). Epilepsy types were: focal (n = 21), generalized (n = 6), or unclassified (n = 1). Mean epilepsy duration was 12.3 years. Mean baseline seizure frequency was 2.8/month. There were significant improvements on methylphenidate for SDMT, MCG, CPT (the ability to discriminate between targets and nontargets [d'] hits, hit reaction time standard deviation, omissions, and commissions), and QOLIE subscales (energy/fatigue, attention/concentration, memory, and language; paired t tests; p ≤ 0.002). BDI-II and additional subscales also improved, at a lower level of statistical significance. Effect

Serial SPECT in children with partial epilepsy

International Nuclear Information System (INIS)

Hosoya, Machiko; Ushiku, Hideo

1995-01-01

We performed serial single-photon emission CT (SPECT) with N-isopropyl-p-( 123 I)-Iodoamphetamine to measure the regional cerebral blood flow (rCBF) in 15 children with partial epilepsy. SPECT showed focal changes in 14 cases. Ten cases had abnormalities in the initial SPECT and another four cases in the second test. The cases with normal rCBF in initial SPECT had been tested in an early phase after the onset, and then decreased rCBF were observed in the second SPECT. The cases with both abnormal rCBF in the initial SPECT and improved rCBF in the second SPECT showed good prognosis in clinico-electrophysiological evolutions. In cases with abnormal changes of rCBF in the second SPECT, clinical prognosis was found to be not so good. These findings suggest that serial SPECT may be used to follow the course of epilepsy. (author)

MRI-negative temporal lobe epilepsy-What do we know?

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Muhlhofer, Wolfgang; Tan, Yee-Leng; Mueller, Susanne G; Knowlton, Robert

2017-05-01

Temporal lobe epilepsy (TLE) is the most common focal epilepsy in adults. TLE has a high chance of becoming medically refractory, and as such, is frequently considered for further evaluation and surgical intervention. Up to 30% of TLE cases, however, can have normal ("nonlesional" or negative) magnetic resonance imaging (MRI) results, which complicates the presurgical workup and has been associated with worse surgical outcomes. Helped by contributions from advanced imaging techniques and electrical source localization, the number of surgeries performed on MRI-negative TLE has increased over the last decade. Thereby new epidemiologic, clinical, electrophysiologic, neuropathologic, and surgical data of MRI-negative TLE has emerged, showing characteristics that are distinct from those of lesional TLE. This review article summarizes what we know today about MRI-negative TLE, and discusses the comprehensive assessment of patients with MRI-negative TLE in a structured and systematic approach. It also includes a concise description of the most recent developments in structural and functional imaging, and highlights postprocessing imaging techniques that have been shown to add localization value in MRI-negative epilepsies. We evaluate surgical outcomes of MRI-negative TLE, identify prognostic makers of postoperative seizure freedom, and discuss strategies for optimizing the selection of surgical candidates in this group. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

Assessment of knowledge, attitude, and practice related to epilepsy: a community-based study

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Teferi J

2015-05-01

Full Text Available Jalle Teferi,1 Zewdu Shewangizaw2 1Addis Ababa Health Bureau, Zewuditu Specialized Hospital, Addis Ababa, Ethiopia; 2College of Medicine and Health Sciences, Arba Minch University, Arba Minch, Ethiopia Abstract: Religious and sociocultural beliefs influence the nature of treatment and care received by people with epilepsy. Many communities in Africa and other developing nations believe that epilepsy results from evil spirits, and thus, treatment should be through the use of herbaceous plants from traditional doctors and religious leadership. Community-based cross-sectional study designs were used to assess the knowledge, attitude, and practice related to epilepsy and its associated factors by using a pretested, semi-structured questionnaire among 660 respondents living in Sululta Woreda, Oromia, Ethiopia. According to the results of this study, 59.8% of the respondents possessed knowledge about epilepsy, 35.6% had a favorable attitude, and 33.5% of them adopted safe practices related to epilepsy. The following factors had significant association to knowledge, attitude, and practice related to epilepsy: being rural dwellers, living alone, those with more years of formal education, heard information about epilepsy, distance of health facility from the community, had witnessed an epileptic seizure, age range from 46 years to 55 years, had heard about epilepsy, prior knowledge of epilepsy, occupational history of being self-employed or a laborer, history of epilepsy, and history of epilepsy in family member. The findings indicated that the Sululta community is familiar with epilepsy, has an unfavorable attitude toward epilepsy, and unsafe practices related to epilepsy, but has a relatively promising knowledge of epilepsy. Keywords: Oromia, favorable attitude, safe practice, rural

Clinical characteristics of children and young adults with co-occurring autism spectrum disorder and epilepsy.

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El Achkar, Christelle M; Spence, Sarah J

2015-06-01

The association between autism spectrum disorder (ASD) and epilepsy has been described for decades, and yet we still lack the full understanding of this relationship both clinically and at the pathophysiologic level. This review evaluates the available data in the literature pertaining to the clinical characteristics of patients with autism spectrum disorder who develop epilepsy and, conversely, patients with epilepsy who develop autism spectrum disorder. Many studies demonstrate an increased risk of epilepsy in individuals with ASD, but rates vary widely. This variability is likely secondary to the different study methods employed, including the study population and definitions of the disorders. Established risk factors for an increased risk of epilepsy in patients with ASD include intellectual disability and female gender. There is some evidence of an increased risk of epilepsy associated with other factors such as ASD etiology (syndromic), severity of autistic features, developmental regression, and family history. No one epilepsy syndrome or seizure type has been associated, although focal or localization-related seizures are often reported. The age at seizure onset can vary from infancy to adulthood with some evidence of a bimodal age distribution. The severity and intractability of epilepsy in populations with ASD have not been well studied, and there is very little investigation of the role that epilepsy plays in the autism behavioral phenotype. There is evidence of abnormal EEGs (especially epileptiform abnormalities) in children with ASD even in the absence of clinical seizures, but very little is known about this phenomenon and what it means. The development of autism spectrum disorder in patients with epilepsy is less well studied, but there is evidence that the ASD risk is greater in those with epilepsy than in the general population. One of the risk factors is intellectual disability, and there is some evidence that the presence of a particular seizure

Adult Prevalence of Epilepsy in Spain: EPIBERIA, a Population-Based Study

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Pedro J. Serrano-Castro

2015-01-01

Full Text Available Background. This study assesses the lifetime and active prevalence of epilepsy in Spain in people older than 18 years. Methods. EPIBERIA is a population-based epidemiological study of epilepsy prevalence using data from three representative Spanish regions (health districts in Zaragoza, Almería, and Seville between 2012 and 2013. The study consisted of two phases: screening and confirmation. Participants completed a previously validated questionnaire (EPIBERIA questionnaire over the telephone. Results. A total of 1741 valid questionnaires were obtained, including 261 (14.99% raising a suspicion of epilepsy. Of these suspected cases, 216 (82.75% agreed to participate in phase 2. Of the phase 2 participants, 22 met the International League Against Epilepsy’s diagnostic criteria for epilepsy. The estimated lifetime prevalence, adjusted by age and sex per 1,000 people, was 14.87 (95% CI: 9.8–21.9. Active prevalence was 5.79 (95% CI: 2.8–10.6. No significant age, sex, or regional differences in prevalence were detected. Conclusions. EPIBERIA provides the most accurate estimate of epilepsy prevalence in the Mediterranean region based on its original methodology and its adherence to ILAE recommendations. We highlight that the lifetime prevalence and inactive epilepsy prevalence figures observed here were compared to other epidemiological studies.

Efficacy and tolerability of high-dose phenobarbital in children with focal seizures.

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Okumura, Akihisa; Nakahara, Eri; Ikeno, Mitsuru; Abe, Shinpei; Igarashi, Ayuko; Nakazawa, Mika; Takasu, Michihiko; Shimizu, Toshiaki

2016-04-01

We retrospectively reviewed the outcomes of children with focal epilepsy treated with oral high-dose phenobarbital. We reviewed data on children (agedphenobarbital (>5 mg/kg/day to maintain a target serum level >40 μg/mL) for at least 6 months. Seizure frequency was evaluated after phenobarbital titration, and 1 and 2 years after high-dose phenobarbital treatment commenced. Treatment was judged effective when seizure frequencies fell by ⩾75%. Seven boys and eight girls were treated. The median age at commencement of high-dose phenobarbital therapy was 30 months. The maximal serum phenobarbital level ranged from 36.5 to 62.9 μg/mL. High-dose PB was effective in seven. In two patients, treatment was transiently effective, but seizure frequency later returned to the baseline. High-dose PB was ineffective in six. No significant association between effectiveness and any clinical variable was evident. Drowsiness was recorded in nine patients, but no patient developed a behavioral problem or hypersensitivity. Oral high-dose phenobarbital was effective in 7 of 15 patients with focal epilepsy and well tolerated. High-dose PB may be useful when surgical treatment is difficult. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

[Causes of symptomatic epilepsy in two first years of life children hospitalized in 2006-2007 years].

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Kroczka, Sławomir; Skowronek-Bała, Barbara; Zajac, Anna

2008-01-01

Epilepsy in two first years of life needs constant attention due to diagnostic and therapeutic difficulties. The aim of the study was to identify cause of symptomatic epilepsy in two first years of life children from miopolskie and podkarpackie provinces hospitalized in Pediatric Neurology Clinic of Children and Adolescents Neurology Cathedra UJ in Cracow. 102 children with epilepsy aged from 1 week to 24 months hospitalized between 1st of January 2006 and 31st of December 2007. The group included 47 girls and 55 boys. On the basis of clinical characteristics and results of additional examinations idiopathic epilepsy was diagnosed in 16/102 (13.3%) children and in remaining 86 (87.7%) symtopmatic epilepsy was established. Perinatal burdening was cause of epilepsy in 31/86 (33.72%) children. Other causes were identified in 32/54 children (59.3%) and in remaining 231 54 (40.7%) children the cause was not established. In 3/32 epilepsy occured in the course of hydrocephalus and in 3/32 children as one of CNS inflammation complications. Epilepsy as a result of vascular lesions and bleeding to CNS occured in 4 children. Multiple developmental deffects syndrome was diagnosed in 4 children and in 11 specific neurodevelopmental disorders were the cause of epilepsy. In 6 children epilepsy occured in the course of neurometabolic diseases, neurocutaneous syndromes and neoplasms. In children in two first years of life polimorphic seizures were diagnosed the most often (32/86 that is 37.2%) and tonic, tonic-clonic seizures were less often (21/86 that is 24.43%). Focal seizures occured in 20/86 (23.26%) patients, in 4/86 (4.65%) mioclonic jerks were observed and infantile spasms in 9/86 (10.46%). (1) In most hospitalized children in two first years of life symptomatic epilepsy was diagnosed. (2) Epilepsy in two first years of life was more often in boys. (3) The most often cause of symptomatic epilepsy was pathology of perinatal period. (4) Polymorphic seizures were the most

Trends in resource utilization and prescription of anticonvulsants for patients with active epilepsy in Germany.

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Strzelczyk, Adam; Haag, Anja; Reese, Jens P; Nickolay, Tanja; Oertel, Wolfgang H; Dodel, Richard; Knake, Susanne; Rosenow, Felix; Hamer, Hajo M

2013-06-01

This study evaluated trends in the resource use of patients with active epilepsy over a 5-year period at an outpatient clinic of a German epilepsy center. Two cross-sectional cohorts of consecutive adults with active epilepsy were evaluated over a 3-month period in 2003 and 2008. Data on socioeconomic status, course of epilepsy, as well as direct and indirect costs were recorded using validated patient questionnaires. We enrolled 101 patients in 2003 and 151 patients in 2008. In both cohorts, 76% of the patients suffered from focal epilepsy, and the majority was on antiepileptic drug (AED) polytherapy (mean AED number: 1.7 (2003), 1.8 (2008)). We calculated epilepsy-specific costs of € 2955 in 2003 and € 3532 in 2008 per 3 months per patient. Direct medical costs were mainly due to anticonvulsants in 2003 (59.4% of total direct costs, 34.0% in 2008) and to hospitalization in 2008 (46.9% of total direct costs, 27.7% in 2003). The proportion of enzyme-inducing anticonvulsants and 'old' AEDs decreased between 2003 and 2008. Indirect costs of € 1689 and € 1847 were mainly due to early retirement (48.4%; 46.0% of total indirect costs in 2003; 2008), unemployment (26.1%; 24.2%), and days off due to seizures (25.5%; 29.8%). This study showed a shift in distribution of direct cost components with increased hospital costs as well as a cost-neutral increase in the prescription of 'newer' AEDs. The amount and distribution of indirect cost components remained unchanged. Copyright © 2013 Elsevier Inc. All rights reserved.

Depression in Patients with Epilepsy: A Study from Enugu, South ...

African Journals Online (AJOL)

Background: Depression is a common psychiatric disorder in epilepsy patients. A bidirectional interaction is thought to be present between epilepsy and depression. There are few studies of this relationship in Nigerian Africans. Objectives: This was a cross-sectional study of the frequency and pattern of depression in a ...

[The causes of symptomatic epilepsy in children aged 3-18 years hospitalized in the year 2006-2007].

Science.gov (United States)

Gergont, Aleksandra; Kroczka, Sławomir; Kaciński, Marek

2008-01-01

Epilepsy can be one of symptoms of the damage to CNS in children with neurodevelopmental deficits, it is more difficult however to diagnose seizures if they are the first symptom of severe brain damage. This retrospective research was conducted to study causes of symptomatic epilepsy in children aged 3-18 year hospitalized between 2006 and 2007 year in the Department of Pediatric Neurology. 156 children with symptomatic epilepsy occurred after 2 years of life were included. The diagnosis of symptomatic epilepsy was established including clinical picture, neuro-radiological tests and EEG. The information from parents was helpful to analyze the type of seizures. The clinical state of children was analyzed, especially psychomotor development, focal deficits, as well as results of CT and/or MRI, in some children psychological testing was performed, molecular or serological. 156 children with epilepsy were hospitalized, within encephalopathy was diagnosed in 61 children. In 42 children static encephalopathy was associated with birth trauma, in 7 progressive encephalopathy was diagnosed, in 1 child CO intoxication caused encephalopathy, and in 11 cases the cause was not identified. Malformations of nervous system were associated with epilepsy in 37 children, geneticaly determined syndromes in 6, and the head trauma in other 6 children. Disorders of vascular origin caused epilepsy in 16 children, and neuroinfections in 9 children. In 2 children epilepsy was associated with ADEM, and in 11 children nonspecific de/dysmyelination was detected. The brain tumor was detected in 6 children with symptomatic epilepsy. The most common disorder leading to epilepsy in children aged 3-18 years was encephalopathy, within hypoxic-ischemic encephalopathy. The other in sequence were malformations of nervous system and vascular diseases.

TBC1D24 genotype-phenotype correlation: Epilepsies and other neurologic features.

Science.gov (United States)

Balestrini, Simona; Milh, Mathieu; Castiglioni, Claudia; Lüthy, Kevin; Finelli, Mattea J; Verstreken, Patrik; Cardon, Aaron; Stražišar, Barbara Gnidovec; Holder, J Lloyd; Lesca, Gaetan; Mancardi, Maria M; Poulat, Anne L; Repetto, Gabriela M; Banka, Siddharth; Bilo, Leonilda; Birkeland, Laura E; Bosch, Friedrich; Brockmann, Knut; Cross, J Helen; Doummar, Diane; Félix, Temis M; Giuliano, Fabienne; Hori, Mutsuki; Hüning, Irina; Kayserili, Hulia; Kini, Usha; Lees, Melissa M; Meenakshi, Girish; Mewasingh, Leena; Pagnamenta, Alistair T; Peluso, Silvio; Mey, Antje; Rice, Gregory M; Rosenfeld, Jill A; Taylor, Jenny C; Troester, Matthew M; Stanley, Christine M; Ville, Dorothee; Walkiewicz, Magdalena; Falace, Antonio; Fassio, Anna; Lemke, Johannes R; Biskup, Saskia; Tardif, Jessica; Ajeawung, Norbert F; Tolun, Aslihan; Corbett, Mark; Gecz, Jozef; Afawi, Zaid; Howell, Katherine B; Oliver, Karen L; Berkovic, Samuel F; Scheffer, Ingrid E; de Falco, Fabrizio A; Oliver, Peter L; Striano, Pasquale; Zara, Federico; Campeau, Phillipe M; Sisodiya, S M

2016-07-05

To evaluate the phenotypic spectrum associated with mutations in TBC1D24. We acquired new clinical, EEG, and neuroimaging data of 11 previously unreported and 37 published patients. TBC1D24 mutations, identified through various sequencing methods, can be found online (http://lovd.nl/TBC1D24). Forty-eight patients were included (28 men, 20 women, average age 21 years) from 30 independent families. Eighteen patients (38%) had myoclonic epilepsies. The other patients carried diagnoses of focal (25%), multifocal (2%), generalized (4%), and unclassified epilepsy (6%), and early-onset epileptic encephalopathy (25%). Most patients had drug-resistant epilepsy. We detail EEG, neuroimaging, developmental, and cognitive features, treatment responsiveness, and physical examination. In silico evaluation revealed 7 different highly conserved motifs, with the most common pathogenic mutation located in the first. Neuronal outgrowth assays showed that some TBC1D24 mutations, associated with the most severe TBC1D24-associated disorders, are not necessarily the most disruptive to this gene function. TBC1D24-related epilepsy syndromes show marked phenotypic pleiotropy, with multisystem involvement and severity spectrum ranging from isolated deafness (not studied here), benign myoclonic epilepsy restricted to childhood with complete seizure control and normal intellect, to early-onset epileptic encephalopathy with severe developmental delay and early death. There is no distinct correlation with mutation type or location yet, but patterns are emerging. Given the phenotypic breadth observed, TBC1D24 mutation screening is indicated in a wide variety of epilepsies. A TBC1D24 consortium was formed to develop further research on this gene and its associated phenotypes. © 2016 American Academy of Neurology.

Neurofibrillary tangle pathology and Braak staging in chronic epilepsy in relation to traumatic brain injury and hippocampal sclerosis: a post-mortem study.

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Thom, Maria; Liu, Joan Y W; Thompson, Pam; Phadke, Rahul; Narkiewicz, Marta; Martinian, Lillian; Marsdon, Derek; Koepp, Matthias; Caboclo, Luis; Catarino, Claudia B; Sisodiya, Sanjay M

2011-10-01

The long-term pathological effects of chronic epilepsy on normal brain ageing are unknown. Previous clinical and epidemiological studies show progressive cognitive decline in subsets of patients and an increased prevalence of Alzheimer's disease in epilepsy. In a post-mortem series of 138 patients with long-term, mainly drug-resistant epilepsy, we carried out Braak staging for Alzheimer's disease neurofibrillary pathology using tau protein immunohistochemistry. The stages were compared with clinicopathological factors, including seizure history and presence of old traumatic brain injury. Overall, 31% of cases were Braak Stage 0, 36% Stage I/II, 31% Stage III/IV and 2% Stage V/VI. The mean age at death was 56.5 years and correlated with Braak stage (P < 0.001). Analysis of Braak stages within age groups showed a significant increase in mid-Braak stages (III/IV), in middle age (40-65 years) compared with data from an ageing non-epilepsy series (P < 0.01). There was no clear relationship between seizure type (generalized or complex partial), seizure frequency, age of onset and duration of epilepsy with Braak stage although higher Braak stages were noted with focal more than with generalized epilepsy syndromes (P < 0.01). In 30% of patients, there was pathological evidence of traumatic brain injury that was significantly associated with higher Braak stages (P < 0.001). Cerebrovascular disease present in 40.3% and cortical malformations in 11.3% were not significantly associated with Braak stage. Astrocytic-tau protein correlated with the presence of both traumatic brain injury (P < 0.01) and high Braak stage (P < 0.001). Hippocampal sclerosis, identified in 40% (bilateral in 48%), was not associated with higher Braak stages, but asymmetrical patterns of tau protein accumulation within the sclerotic hippocampus were noted. In over half of patients with cognitive decline, the Braak stage was low indicating causes other than Alzheimer's disease pathology. In summary

Personalized translational epilepsy research - Novel approaches and future perspectives: Part II: Experimental and translational approaches.

Science.gov (United States)

Bauer, Sebastian; van Alphen, Natascha; Becker, Albert; Chiocchetti, Andreas; Deichmann, Ralf; Deller, Thomas; Freiman, Thomas; Freitag, Christine M; Gehrig, Johannes; Hermsen, Anke M; Jedlicka, Peter; Kell, Christian; Klein, Karl Martin; Knake, Susanne; Kullmann, Dimitri M; Liebner, Stefan; Norwood, Braxton A; Omigie, Diana; Plate, Karlheinz; Reif, Andreas; Reif, Philipp S; Reiss, Yvonne; Roeper, Jochen; Ronellenfitsch, Michael W; Schorge, Stephanie; Schratt, Gerhard; Schwarzacher, Stephan W; Steinbach, Joachim P; Strzelczyk, Adam; Triesch, Jochen; Wagner, Marlies; Walker, Matthew C; von Wegner, Frederic; Rosenow, Felix

2017-11-01

Despite the availability of more than 15 new "antiepileptic drugs", the proportion of patients with pharmacoresistant epilepsy has remained constant at about 20-30%. Furthermore, no disease-modifying treatments shown to prevent the development of epilepsy following an initial precipitating brain injury or to reverse established epilepsy have been identified to date. This is likely in part due to the polyetiologic nature of epilepsy, which in turn requires personalized medicine approaches. Recent advances in imaging, pathology, genetics, and epigenetics have led to new pathophysiological concepts and the identification of monogenic causes of epilepsy. In the context of these advances, the First International Symposium on Personalized Translational Epilepsy Research (1st ISymPTER) was held in Frankfurt on September 8, 2016, to discuss novel approaches and future perspectives for personalized translational research. These included new developments and ideas in a range of experimental and clinical areas such as deep phenotyping, quantitative brain imaging, EEG/MEG-based analysis of network dysfunction, tissue-based translational studies, innate immunity mechanisms, microRNA as treatment targets, functional characterization of genetic variants in human cell models and rodent organotypic slice cultures, personalized treatment approaches for monogenic epilepsies, blood-brain barrier dysfunction, therapeutic focal tissue modification, computational modeling for target and biomarker identification, and cost analysis in (monogenic) disease and its treatment. This report on the meeting proceedings is aimed at stimulating much needed investments of time and resources in personalized translational epilepsy research. This Part II includes the experimental and translational approaches and a discussion of the future perspectives, while the diagnostic methods, EEG network analysis, biomarkers, and personalized treatment approaches were addressed in Part I [1]. Copyright © 2017

Mortality in epilepsy.

Science.gov (United States)

Hitiris, Nikolas; Mohanraj, Rajiv; Norrie, John; Brodie, Martin J

2007-05-01

All studies report an increased mortality risk for people with epilepsy compared with the general population. Population-based studies have demonstrated that the increased mortality is often related to the cause of the epilepsy. Common etiologies include neoplasia, cerebrovascular disease, and pneumonia. Deaths in selected cohorts, such as sudden unexpected death in epilepsy (SUDEP), status epilepticus (SE), suicides, and accidents are more frequently epilepsy-related. SUDEP is a particular cause for concern in younger people, and whether and when SUDEP should be discussed with patients with epilepsy remain problematic issues. Risk factors for SUDEP include generalized tonic-clonic seizures, increased seizure frequency, concomitant learning disability, and antiepileptic drug polypharmacy. The overall incidence of SE may be increasing, although case fatality rates remain constant. Mortality is frequently secondary to acute symptomatic disorders. Poor compliance with treatment in patients with epilepsy accounts for a small proportion of deaths from SE. The incidence of suicide is increased, particularly for individuals with epilepsy and comorbid psychiatric conditions. Late mortality figures in patients undergoing epilepsy surgery vary and are likely to reflect differences in case selection. Future studies of mortality should be prospective and follow agreed guidelines to better quantify risk and causation in individual populations.

Clinical and MRI features of supratentorial gliomas with adult-onset epilepsy

Energy Technology Data Exchange (ETDEWEB)

Hashimoto, Takahiro; Yamaura, Akira (Chiba Univ. (Japan). School of Medicine); Watanabe, Osamu

1992-02-01

Although some patients with supratentorial gliomas develop epilepsy in their clinical course, the details of adult-onset epilepsy with gliomas have not been fully evaluated. This paper reports on 15 cases of supratentorial glioma with the sole symptom of adult-onset epilepsy and characterizes their clinical and MRI features. The patients, 5 males and 10 females, developed the first epilepsy at the mean age of 37 years. Generalized seizure was encountered in all cases and focal seizure alone was never seen. Seizure was satisfactorily controlled with anticonvulsants in all except 2 cases. The tumor was located in the frontal lobe (9 cases) or temporal lobe (6 cases). Histologically, there were 12 astrocytomas, 2 glioblastomas, and 1 oligoastrocytoma. Of these, 12 were benign gliomas. Surprisingly, CT scan and MRI revealed tumors larger than predicted. The abnormal intensity region was delineated most prominently on T[sub 2]-weighted SE image and was broader on T[sub 2]-weighted spin echo image than on T[sub 1]-weighted spin echo and inversion recovery image. The authors conclude that gliomas presenting with epilepsy tend to be histologically benign, are predominantly seen in middle-aged women, and are located in the frontal and temporal lobes. Although a tumor may be large enough to be detected on CT scan or MRI, as in the present study, histological examination is needed to establish the diagnosis. Additionally, gliomas with equivocal abnormalities on CT and MRI do evolve despite further neurological deficits, so meticulous evaluation including stereotactic biopsy is the method of choice. Finally, T[sub 2]-weighted SE image in the coronal plane is advocated for patients with adult-onset epilepsy to achieve accurate diagnosis and to initiate early treatment. (author).

Positive psychological interventions for people with epilepsy: An assessment on factors related to intervention participation.

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Lai, Siew-Tim; Lim, Kheng-Seang; Tang, Venus; Low, Wah-Yun

2018-03-01

Positive psychological interventions (PPI) are increasingly employed as a coping strategy with physical and mental conditions, including neurological diseases. Its effectiveness on improving wellbeing in people with epilepsy (PWE) has been shown in a few studies. This study aimed to explore factors related to participants' willingness to engage in psychological interventions from the perspective of patients with epilepsy. Participants answered a needs assessment questionnaire eliciting information about their illness perception (Brief Illness Perception Questionnaire (Brief-IPQ)), emotions (Hospital Anxiety and Depression Scale (HADS)), willingness to participate in psychological interventions, preferences in types of PPI and intervention designs, as well as barriers in seeking mental health services. A total of 154 patients with epilepsy participated, with a mean age of 37.3years (range 16-86years). Most patients had focal epilepsy (68.2%), and drug-resistant (59.1%). Majority (71.4%) of them indicated a strong willingness to participate in PPI. Out of nine types of PPI, character strengths, mindfulness-based and expressive-based interventions were highly preferred. Those with negative illness perception (p=0.001), anxiety (p=0.004), and being unemployed (p=0.048) were more willing to participate in PPI. Most participants preferred group rather than individual session, and a shorter duration (30min) was favored by most. This study captured the self-report willingness to participate in psychological interventions. Findings suggested that psychological interventions delivered in short-group session were highly preferred. Future study is required to determine the feasibility of such design for patients with epilepsy. Copyright © 2017 Elsevier Inc. All rights reserved.

The study of the focal trough in panoramic radiograph

International Nuclear Information System (INIS)

Park, C. S.; Kim, H. P.

1982-01-01

In the study of the focal trough of panoramic radiograph, using the Moritta company Panex EC a series of 48 exposures were taken with the 6-18 brass pins placed in the holes of the plastic model plate, then evaluated by 4 observers. The author analyzed the focal trough defined by the sharpness criteria and calculated the vertical and horizontal magnification range in the corrected focal trough. The results were as follows; 1. Continuous focal trough was not defined in the anterior region using a very high degree of sharpness. 2. As degree of sharpness used in the analysis became less, focal trough was continuous in the anterior and posterior regions, symmetrized bilaterally, and the widths of the focal trough increased more in the posterior region. 3. As sharpness criteria were reduced, the percentage range of image magnification increased in both vertical and horizontal magnification, and especially the percentage range of horizontal magnification was greater than that of vertical magnification.

Sequential motor task (Luria's Fist-Edge-Palm Test in children with benign focal epilepsy of childhood with centrotemporal spikes Tarefa motora sequencial (Teste de Lúria punho-lado-palma em crianças com epilepsia focal benigna da infância com descarga centrotemporal

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Carmen Silvia Molleis Galego Miziara

2013-06-01

Full Text Available This study evaluated the sequential motor manual actions in children with benign focal epilepsy of childhood with centrotemporal spikes (BECTS and compares the results with matched control group, through the application of Luria's fist-edge-palm test. The children with BECTS underwent interictal single photon emission computed tomography (SPECT and School Performance Test (SPT. Significant difference occurred between the study and control groups for manual motor action through three equal and three different movements. Children with lower school performance had higher error rate in the imitation of hand gestures. Another factor significantly associated with the failure was the abnormality in SPECT. Children with BECTS showed abnormalities in the test that evaluated manual motor programming/planning. This study may suggest that the functional changes related to epileptiform activity in rolandic region interfere with the executive function in children with BECTS.Esse estudo avaliou ações motoras manuais sequenciais em crianças com epilepsia focal benigna da infância com descarga centrotemporal (EBICT e comparou os resultados com o grupo controle pareado, através do teste de Lúria (punho-lado-palma. As crianças com EBICT realizaram single photon emission computed tomography (SPECT interictal e Teste de Desempenho Escolar (TDE. Foram encontradas diferenças significativas entre os dois grupos nas atividades motoras de três movimentos iguais e três movimentos diferentes. As crianças com piores resultados no TDE e com SPECT alterado apresentaram mais erros no teste de imitação manual. Crianças com epilepsia fracassaram nos testes de avaliação motora que envolvem programação/planejamento. Esse estudo sugere que mudanças funcionais relacionadas à atividade epileptiforme na região rolândica interfere com as funções executivas de crianças com EBICT.

Characterization of focal cortical dysplasia with balloon cells by layer-specific markers: Evidence for differential vulnerability of interneurons.

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Nakagawa, Julia M; Donkels, Catharina; Fauser, Susanne; Schulze-Bonhage, Andreas; Prinz, Marco; Zentner, Josef; Haas, Carola A

2017-04-01

Focal cortical dysplasia (FCD) is a major cause of pharmacoresistant focal epilepsy. Little is known about the pathomechanisms underlying the characteristic cytoarchitectural abnormalities associated with FCD. In the present study, a broad panel of markers identifying layer-specific neuron subpopulations was applied to characterize dyslamination and structural alterations in FCD with balloon cells (FCD 2b). Pan-neuronal neuronal nuclei (NeuN) and layer-specific protein expression (Reelin, Calbindin, Calretinin, SMI32 (nonphosphorylated neurofilament H), Parvalbumin, transducin-like enhancer protein 4 (TLE4), and Vimentin) was studied by immunohistochemistry on paraffin sections of FCD2b cases (n = 22) and was compared to two control groups with (n = 7) or without epilepsy (n = 4 postmortem cases). Total and layer-specific neuron densities were systematically quantified by cell counting considering age at surgery and brain region. We show that in FCD2b total neuron densities across all six cortical layers were not significantly different from controls. In addition, we present evidence that a basic laminar arrangement of layer-specific neuron subtypes was preserved despite the severe disturbance of cortical structure. SMI32-positive pyramidal neurons showed no significant difference in total numbers, but a reduction in layers III and V. The densities of supragranular Calbindin- and Calretinin-positive interneurons in layers II and III were not different from controls, whereas Parvalbumin-expressing interneurons, primarily located in layer IV, were significantly reduced in numbers when compared to control cases without epilepsy. In layer VI, the density of TLE4-positive projection neurons was significantly increased. Altogether, these data show that changes in cellular composition mainly affect deep cortical layers in FCD2b. The application of a broad panel of markers defining layer-specific neuronal subpopulations revealed that in FCD2b neuronal diversity and a basic

Structural brain abnormalities in the common epilepsies assessed in a worldwide ENIGMA study

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Altmann, Andre; Botía, Juan A; Jahanshad, Neda; Hibar, Derrek P; Absil, Julie; Alhusaini, Saud; Alvim, Marina K M; Auvinen, Pia; Bartolini, Emanuele; Bergo, Felipe P G; Bernardes, Tauana; Blackmon, Karen; Braga, Barbara; Caligiuri, Maria Eugenia; Calvo, Anna; Carr, Sarah J; Chen, Jian; Chen, Shuai; Cherubini, Andrea; David, Philippe; Domin, Martin; Foley, Sonya; França, Wendy; Haaker, Gerrit; Isaev, Dmitry; Keller, Simon S; Kotikalapudi, Raviteja; Kowalczyk, Magdalena A; Kuzniecky, Ruben; Langner, Soenke; Lenge, Matteo; Leyden, Kelly M; Liu, Min; Loi, Richard Q; Martin, Pascal; Mascalchi, Mario; Morita, Marcia E; Pariente, Jose C; Rodríguez-Cruces, Raul; Rummel, Christian; Saavalainen, Taavi; Semmelroch, Mira K; Severino, Mariasavina; Thomas, Rhys H; Tondelli, Manuela; Tortora, Domenico; Vaudano, Anna Elisabetta; Vivash, Lucy; von Podewils, Felix; Wagner, Jan; Weber, Bernd; Yao, Yi; Yasuda, Clarissa L; Zhang, Guohao; Bargalló, Nuria; Bender, Benjamin; Bernasconi, Neda; Bernasconi, Andrea; Bernhardt, Boris C; Blümcke, Ingmar; Carlson, Chad; Cavalleri, Gianpiero L; Cendes, Fernando; Concha, Luis; Delanty, Norman; Depondt, Chantal; Devinsky, Orrin; Doherty, Colin P; Focke, Niels K; Gambardella, Antonio; Guerrini, Renzo; Hamandi, Khalid; Jackson, Graeme D; Kälviäinen, Reetta; Kochunov, Peter; Kwan, Patrick; Labate, Angelo; McDonald, Carrie R; Meletti, Stefano; O'Brien, Terence J; Ourselin, Sebastien; Richardson, Mark P; Striano, Pasquale; Thesen, Thomas; Wiest, Roland; Zhang, Junsong; Vezzani, Annamaria; Ryten, Mina; Thompson, Paul M

2018-01-01

Abstract Progressive functional decline in the epilepsies is largely unexplained. We formed the ENIGMA-Epilepsy consortium to understand factors that influence brain measures in epilepsy, pooling data from 24 research centres in 14 countries across Europe, North and South America, Asia, and Australia. Structural brain measures were extracted from MRI brain scans across 2149 individuals with epilepsy, divided into four epilepsy subgroups including idiopathic generalized epilepsies (n =367), mesial temporal lobe epilepsies with hippocampal sclerosis (MTLE; left, n = 415; right, n = 339), and all other epilepsies in aggregate (n = 1026), and compared to 1727 matched healthy controls. We ranked brain structures in order of greatest differences between patients and controls, by meta-analysing effect sizes across 16 subcortical and 68 cortical brain regions. We also tested effects of duration of disease, age at onset, and age-by-diagnosis interactions on structural measures. We observed widespread patterns of altered subcortical volume and reduced cortical grey matter thickness. Compared to controls, all epilepsy groups showed lower volume in the right thalamus (Cohen’s d = −0.24 to −0.73; P left, but not right, MTLE (d = −0.29 to −0.54; P right, but not left, MTLE (d = −0.27 to −0.51; P right MTLE groups (beta, b brain measures that can be further targeted for study in genetic and neuropathological studies. This worldwide initiative identifies patterns of shared grey matter reduction across epilepsy syndromes, and distinctive abnormalities between epilepsy syndromes, which inform our understanding of epilepsy as a network disorder, and indicate that certain epilepsy syndromes involve more widespread structural compromise than previously assumed. PMID:29365066

Brain SPECT with 123I-isopropyl amphetamine in epilepsy

International Nuclear Information System (INIS)

Biersack, H.J.; Reske, S.N.; Rasche, A.; Reichmann, K.; Winkler, C.

1983-01-01

Ten patients were studied with N-isopropyl I-123 p-iodoamphetamine. Single photon emission computed tomography (SPECT) was carried out by hand of a rotating gamma camera system (Gammatome T9000/CGR, high resolution collimator). During 1 rotation (360 0 ) 64 frames (4k matrix) were acquired within 20 min 1 hour after injection of 6.5 mCi I-123 labeled amphetamine. The content of I-124 was less than 2%. After reconstruction of transverse slices coronar and sagittal reconstructions were rapidly performed using an array processor. Nine patients suffered from epilepsy and one from severe migraine. Excellent differentiation between gray and white matter of the cerebral cortex and the basal ganglia was evident in all of the cases. In 2 out of 3 patients with epilepsy and negative CT results SPECT revealed circumscribed areas with increased amphetamine uptake in accordance with the EEG findings. In 4 out of 6 cases with positive CT findings SPECT lesions with diminished amphetamine uptake could be established. One patient with severe migraine showed focal increased amphetamine uptake in accordance with the respective clinical results. (orig.)

The diagnostic utility of 3D-ESI rotating and moving dipole methodology in the pre-surgical evaluation of MRI-negative childhood epilepsy due to focal cortical dysplasia.

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Russo, Angelo; Lallas, Matt; Jayakar, Prasanna; Miller, Ian; Hyslop, Ann; Dunoyer, Catalina; Resnick, Trevor; Duchowny, Michael

2016-09-01

This study investigates whether a combined rotating dipole (RD) and moving dipole (MD) solution enhances three-dimensional electroencephalography (EEG) source imaging (3D-ESI) localization in magnetic resonance imaging (MRI)-negative pediatric patients with focal cortical dysplasia (FCD). We retrospectively selected 14 MRI-negative patients with FCD from a cohort of 60 pediatric patients previously used to evaluate the diagnostic utility of 3D-ESI in epilepsy surgery. Patients were younger than 18 years at time of surgery and had at least 1 year of outcome data. RD and MD models were constructed for each interictal spike or sharp wave, and it was determined whether each inverse algorithm localized within the surgical resection cavity (SRC). We also compared the 3D-ESI findings and surgical outcome with positron emission tomography (PET) and ictal single photon emission computed tomography (iSPECT). RD analyses revealed a high concordance with the SRC (78.6%), particularly for temporal lobe resection (100.0%), and showed superior localization compared to PET and iSPECT, with the highest correlation in FCD type I and temporal lobe resection. Furthermore, the RD method was superior to iSPECT in FCD type II cases and to PET in extratemporal resections. RD and MD results were comparable, but in 18.2% of patients with FCD type I with localizing RDs, the MD solution was only partially within the SRC; in all of these patients 3D-ESI also correlated with superior surgical outcome compared to PET and iSPECT, especially when RD and MD solutions were analyzed together. 3D-ESI in MRI-negative cases showed superior localization compared to iSPECT or PET, especially in FCD type I and temporal lobe epilepsy, and correlated with superior surgical outcome compared to iSPECT and PET at 1 year and 2 years postoperatively, especially when RD and MD solutions were analyzed together. These findings suggest that 3D-ESI based on a combined RD-MD solution improves surgical accuracy in

Positron emission tomography in pre-surgical evaluation of partial epilepsy in adults

International Nuclear Information System (INIS)

Semah, F.; Dupont, S.

1999-01-01

Positron emission tomography (PET) may be used to map regional cerebral glucose metabolism using 18 F-deoxyglucose in patients with partial epilepsy. An area of reduced glucose metabolism, that is commonly more extensive than the underlying anatomical abnormality, is found in most of the patients with medically refractory partial epilepsy. These functional lesions are very useful in the delineation of the epileptogenic focus prior to surgery. PET may also be used to demonstrate abnormalities in the binding of specific ligands, such as 11 C-flumazenil, to the central benzodiazepine-GABA A receptor complex. Focal decreases in benzodiazepine receptor binding is commonly seen at an epileptic focus, in a more restricted distribution than the area of hypo-metabolism. (author)

Correlation between perceived stigma and EEG paroxysmal abnormality in childhood epilepsy.

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Kanemura, Hideaki; Sano, Fumikazu; Ohyama, Tetsuo; Sugita, Kanji; Aihara, Masao

2015-11-01

We investigated the relationship between abnormal electroencephalogram (EEG) findings such as localized EEG paroxysmal abnormality (PA) and the perception of stigma to determine EEG factors associated with perceived stigma in childhood epilepsy. Participants comprised 40 patients (21 boys, 19 girls; mean age, 14.6 years) with epilepsy at enrollment. The criteria for inclusion were as follows: 1) age of 12-18 years, inclusive; 2) ≥6 months after epilepsy onset; 3) the ability to read and speak Japanese; and 4) the presence of EEG PA. Fifteen healthy seizure-free children were included as a control group. Participants were asked to rate how often they felt or acted in the ways described in the items of the Child Stigma Scale using a 5-point scale. Electroencephalogram paroxysms were classified based on the presence of spikes, sharp waves, or spike-wave complexes, whether focal or generalized. Participants showed significantly higher stigma scores than healthy subjects (pstigma. The average total scores of patients presenting with EEG PA at generalized, frontal, RD, midtemporal, and occipital regions were 2.3, 4.0, 2.4, 3.2, and 2.2, respectively. The scores of all questions were higher in the frontal group than those in other regions (pstigma than children presenting with nonfrontal EEG PA (pstigma. Further studies are needed to confirm whether frontal EEG PA may function as a mediator of emotional responses such as perceived stigma in childhood epilepsy. Copyright © 2015 Elsevier Inc. All rights reserved.

Childhood onset temporal lobe epilepsy: Beyond hippocampal sclerosis.

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Mühlebner, Angelika; Breu, Markus; Kasprian, Gregor; Schmook, Maria T; Stefanits, Harald; Scholl, Theresa; Samueli, Sharon; Gröppel, Gudrun; Dressler, Anastasia; Prayer, Daniela; Czech, Thomas; Hainfellner, Johannes A; Feucht, Martha

2016-03-01

Hippocampal Sclerosis (HS) is widely recognized as a significant underlying cause of drug-resistant temporal lobe epilepsy (TLE) in adults. In contrast, HS is a rare finding in pediatric surgical series, and a higher incidence of HS associated with cortical dysplasia (i.e. FCD type IIIa according to the new ILAE classification) than in adult series has been reported. Data about the electro-clinical characteristics of this subgroup are scarce. We studied 15 children and adolescents with drug-resistant TLE and HS who had anterior temporal lobe resection at our center with regard to electroclinical characteristics, MRI features and histopathology. Children in whom histopathology was consistent with Focal Cortical Dysplasia (FCD) type IIIa (n = 7) were compared with those who had HS only (n = 8). Clinical characteristics associated with this highly selective subset of patients with FCD type IIIa were: the presence of febrile seizures during infancy, a shorter duration of active epilepsy and a lower age at epilepsy surgery. In addition, there were non-significant trends towards more extended abnormalities on both EEG and neuroimaging. We were, however, not able to find group differences with respect to neuropathologic subtyping of the HS. We present the first detailed description and comprehensive data analysis of children with FCD type IIIa. According to our results, this patient group seems to show a distinct clinical phenotype. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Information about epilepsy on the internet: An exploratory study of Arabic websites.

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Alkhateeb, Jamal M; Alhadidi, Muna S

2018-01-01

The aim of this study was to explore information about epilepsy found on Arabic websites. The researchers collected information from the internet between November 2016 and January 2017. Information was obtained using Google and Yahoo search engines. Keywords used were the Arabic equivalent of the following two keywords: epilepsy (Al-saraa) and convulsion (Tashanoj). A total of 144 web pages addressing epilepsy in Arabic were reviewed. The majority of web pages were websites of medical institutions and general health websites, followed by informational and educational websites, others, blogs and websites of individuals, and news and media sites. Topics most commonly addressed were medical treatments for epilepsy (50% of all pages) followed by epilepsy definition (41%) and epilepsy etiology (34.7%). The results also revealed that the vast majority of web pages did not mention the source of information. Many web pages also did not provide author information. Only a small proportion of the web pages provided adequate information. Relatively few web pages provided inaccurate information or made sweeping generalizations. As a result, it is concluded that the findings of the present study suggest that development of more credible Arabic websites on epilepsy is needed. These websites need to go beyond basic information, offering more evidence-based and updated information about epilepsy. Copyright © 2017 Elsevier Inc. All rights reserved.

Pulmonary and cardiac pathology in sudden unexpected death in epilepsy (SUDEP).

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Nascimento, Fábio A; Tseng, Zian H; Palmiere, Cristian; Maleszewski, Joseph J; Shiomi, Takayuki; McCrillis, Aileen; Devinsky, Orrin

2017-08-01

To review studies on structural pulmonary and cardiac changes in SUDEP cases as well as studies showing pulmonary or cardiac structural changes in living epilepsy patients. We conducted electronic literature searches using the PubMed database for articles published in English, regardless of publication year, that included data on cardiac and/or pulmonary structural abnormalities in SUDEP cases or in living epilepsy patients during the postictal period. Fourteen postmortem studies reported pulmonary findings in SUDEP cases. Two focused mainly on assessing lung weights in SUDEP cases versus controls; no group difference was found. The other 12 reported descriptive autopsy findings. Among all SUDEP cases with available descriptive postmortem pulmonary examination, 72% had pulmonary changes, most often pulmonary edema/congestion, and, less frequently, intraalveolar hemorrhage. Eleven studies reported on cardiac pathology in SUDEP. Cardiac abnormalities were found in approximately one-fourth of cases. The most common findings were myocyte hypertrophy and myocardial fibrosis of various degrees. Among living epilepsy patients, postictal pulmonary pathology was the most commonly reported pulmonary abnormality and the most common postictal cardiac abnormality was transient left ventricular dysfunction - Takotsubo or neurogenic stunned myocardium. Cardiac and pulmonary pathological abnormalities are frequent among SUDEP cases, most commonly pulmonary edema/congestion and focal interstitial myocardial fibrosis. Most findings are not quantified, with subjective elements and undefined interobserver reliability, and lack of controls such as matched epilepsy patients who died from other causes. Further, studies have not systematically evaluated potential confounding factors, including postmortem interval to autopsy, paramedic resuscitation and IV fluids administration, underlying heart/lung disease, and risk factors for cardiac or pulmonary disease. Prospective studies with

Personalized translational epilepsy research - Novel approaches and future perspectives: Part I: Clinical and network analysis approaches.

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Rosenow, Felix; van Alphen, Natascha; Becker, Albert; Chiocchetti, Andreas; Deichmann, Ralf; Deller, Thomas; Freiman, Thomas; Freitag, Christine M; Gehrig, Johannes; Hermsen, Anke M; Jedlicka, Peter; Kell, Christian; Klein, Karl Martin; Knake, Susanne; Kullmann, Dimitri M; Liebner, Stefan; Norwood, Braxton A; Omigie, Diana; Plate, Karlheinz; Reif, Andreas; Reif, Philipp S; Reiss, Yvonne; Roeper, Jochen; Ronellenfitsch, Michael W; Schorge, Stephanie; Schratt, Gerhard; Schwarzacher, Stephan W; Steinbach, Joachim P; Strzelczyk, Adam; Triesch, Jochen; Wagner, Marlies; Walker, Matthew C; von Wegner, Frederic; Bauer, Sebastian

2017-11-01

Despite the availability of more than 15 new "antiepileptic drugs", the proportion of patients with pharmacoresistant epilepsy has remained constant at about 20-30%. Furthermore, no disease-modifying treatments shown to prevent the development of epilepsy following an initial precipitating brain injury or to reverse established epilepsy have been identified to date. This is likely in part due to the polyetiologic nature of epilepsy, which in turn requires personalized medicine approaches. Recent advances in imaging, pathology, genetics and epigenetics have led to new pathophysiological concepts and the identification of monogenic causes of epilepsy. In the context of these advances, the First International Symposium on Personalized Translational Epilepsy Research (1st ISymPTER) was held in Frankfurt on September 8, 2016, to discuss novel approaches and future perspectives for personalized translational research. These included new developments and ideas in a range of experimental and clinical areas such as deep phenotyping, quantitative brain imaging, EEG/MEG-based analysis of network dysfunction, tissue-based translational studies, innate immunity mechanisms, microRNA as treatment targets, functional characterization of genetic variants in human cell models and rodent organotypic slice cultures, personalized treatment approaches for monogenic epilepsies, blood-brain barrier dysfunction, therapeutic focal tissue modification, computational modeling for target and biomarker identification, and cost analysis in (monogenic) disease and its treatment. This report on the meeting proceedings is aimed at stimulating much needed investments of time and resources in personalized translational epilepsy research. Part I includes the clinical phenotyping and diagnostic methods, EEG network-analysis, biomarkers, and personalized treatment approaches. In Part II, experimental and translational approaches will be discussed (Bauer et al., 2017) [1]. Copyright © 2017 Elsevier Inc

Epilepsy Characteristics and Clinical Outcome in Patients With Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes (MELAS).

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Lee, Ha Neul; Eom, Soyong; Kim, Se Hoon; Kang, Hoon-Chul; Lee, Joon Soo; Kim, Heung Dong; Lee, Young-Mock

2016-11-01

Epileptic seizures in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) are heterogeneous with no pathognomonic features. We reviewed epilepsy characteristics and clinical outcome exclusively in a pediatric population. Twenty-two children and adolescents (13 males) with confirmed mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes due to mitochondrial DNA A3243G mutation and epilepsy were recruited. Clinical data including seizure semiology, treatment response, neuroimaging findings, and electroencephalography were analyzed. We also examined the effect of the age at seizure onset and initial symptoms on the clinical variables. Seizure semiology and electroencephalography abnormalities showed no syndrome-specific findings. Focal seizures occurred in 21 of 22 subjects (95.5%), whereas generalized seizures developed in seven of 22 subjects (31.8%). Twenty of 22 subjects (90.9%) achieved partial to complete reduction of clinical seizures for more than one year with a combination of more than two antiepileptic drugs. The subgroup with earlier seizure onset presented significantly earlier and showed significantly higher rates of drug-resistant epilepsy compared with the late onset group, although there were no significant differences in the initial symptoms. The subjects with severe epileptic conditions tended to have more severe clinical dysfunction and more severe organ involvement. Both focal and generalized seizures occurred in patients with MELAS. Epilepsy in this population is drug resistant, but a certain degree of clinical seizure reduction was achievable with antiepileptic drugs, with more favorable outcomes than historically expected. Close observation and active epilepsy treatment of individuals with MELAS episodes and earlier seizure onset might improve the prognosis. Copyright © 2016 Elsevier Inc. All rights reserved.

Homozygous TBC1D24 mutation in two siblings with familial infantile myoclonic epilepsy (FIME) and moderate intellectual disability.

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Poulat, Anne-Lise; Ville, Dorothée; de Bellescize, Julitta; André-Obadia, Nathalie; Cacciagli, Pierre; Milh, Mathieu; Villard, Laurent; Lesca, Gaetan

2015-03-01

Mutations in the TBC1D24 gene were first reported in an Italian family with a unique epileptic phenotype consisting of drug-responsive, early-onset idiopathic myoclonic seizures. Patients presented with isolated bilateral or focal myoclonia, which could evolve to long-lasting attacks without loss of consciousness, with a peculiar reflex component, and were associated with generalized tonic-clonic seizures. This entity was named "familial infantile myoclonic epilepsy" (FIME). More recently, TBC1D24 mutations have been shown to cause a variable range of disorders, including epilepsy of various seizure types and severity, non-syndromic deafness, and DOORS syndrome. We report on the electro-clinical features of two brothers, born to first-cousin parents, affected with infantile-onset myoclonic epilepsy. The peculiar epileptic presentation prompted us to perform direct sequencing of the TBC1D24 gene. The patients had very early onset of focal myoclonic fits with variable topography, lasting a few minutes to several hours, without loss of consciousness, which frequently evolved to generalized myoclonus or myoclonic status. Reflex myoclonia were noticed in one patient. Neurological outcome was marked by moderate intellectual disability. Despite the high frequency of seizures, repeated EEG recordings showed normal background rhythm and rare interictal spikes and waves. We found a homozygous missense mutation, c.457G>A/p.Glu153Lys, in the two affected brothers. This observation combined with recent data from the literature, suggest that mutations in TBCD24 cause a pathological continuum, with FIME at the "benign" end and severe drug-refractory epileptic encephalopathy on the severe end. Early-onset myoclonic epilepsy with focal and generalized myoclonic seizures is a common characteristic of this continuum. Copyright © 2015 Elsevier B.V. All rights reserved.

Localization Accuracy of Distributed Inverse Solutions for Electric and Magnetic Source Imaging of Interictal Epileptic Discharges in Patients with Focal Epilepsy.

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Heers, Marcel; Chowdhury, Rasheda A; Hedrich, Tanguy; Dubeau, François; Hall, Jeffery A; Lina, Jean-Marc; Grova, Christophe; Kobayashi, Eliane

2016-01-01

Distributed inverse solutions aim to realistically reconstruct the origin of interictal epileptic discharges (IEDs) from noninvasively recorded electroencephalography (EEG) and magnetoencephalography (MEG) signals. Our aim was to compare the performance of different distributed inverse solutions in localizing IEDs: coherent maximum entropy on the mean (cMEM), hierarchical Bayesian implementations of independent identically distributed sources (IID, minimum norm prior) and spatially coherent sources (COH, spatial smoothness prior). Source maxima (i.e., the vertex with the maximum source amplitude) of IEDs in 14 EEG and 19 MEG studies from 15 patients with focal epilepsy were analyzed. We visually compared their concordance with intracranial EEG (iEEG) based on 17 cortical regions of interest and their spatial dispersion around source maxima. Magnetic source imaging (MSI) maxima from cMEM were most often confirmed by iEEG (cMEM: 14/19, COH: 9/19, IID: 8/19 studies). COH electric source imaging (ESI) maxima co-localized best with iEEG (cMEM: 8/14, COH: 11/14, IID: 10/14 studies). In addition, cMEM was less spatially spread than COH and IID for ESI and MSI (p < 0.001 Bonferroni-corrected post hoc t test). Highest positive predictive values for cortical regions with IEDs in iEEG could be obtained with cMEM for MSI and with COH for ESI. Additional realistic EEG/MEG simulations confirmed our findings. Accurate spatially extended sources, as found in cMEM (ESI and MSI) and COH (ESI) are desirable for source imaging of IEDs because this might influence surgical decision. Our simulations suggest that COH and IID overestimate the spatial extent of the generators compared to cMEM.

Epilepsy is a possible feature in Williams-Beuren syndrome patients harboring typical deletions of the 7q11.23 critical region.

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Nicita, Francesco; Garone, Giacomo; Spalice, Alberto; Savasta, Salvatore; Striano, Pasquale; Pantaleoni, Chiara; Spartà, Maria Valentina; Kluger, Gerhard; Capovilla, Giuseppe; Pruna, Dario; Freri, Elena; D'Arrigo, Stefano; Verrotti, Alberto

2016-01-01

Seizures are rarely reported in Williams-Beuren syndrome (WBS)--a contiguous-gene-deletion disorder caused by a 7q11.23 heterozygous deletion of 1.5-1.8 Mb--and no previous study evaluated electro-clinical features of epilepsy in this syndrome. Furthermore, it has been hypothesized that atypical deletion (e.g., larger than 1.8 Mb) may be responsible for a more pronounced neurological phenotypes, especially including seizures. Our objectives are to describe the electro-clinical features in WBS and to correlate the epileptic phenotype with deletion of the 7q11.23 critical region. We evaluate the electro-clinical features in one case of distal 7q11.23 deletion syndrome and in eight epileptic WBS (eWBS) patients. Additionally, we compare the deletion size-and deleted genes-of four epileptic WBS (eWBS) with that of four non-epileptic WBS (neWBS) patients. Infantile spasms, focal (e.g., motor and dyscognitive with autonomic features) and generalized (e.g., tonic-clonic, tonic, clonic, myoclonic) seizures were encountered. Drug-resistance was observed in one patient. Neuroimaging discovered one case of focal cortical dysplasia, one case of fronto-temporal cortical atrophy and one case of periventricular nodular heterotopia. Comparison of deletion size between eWBS and neWBS patients did not reveal candidate genes potentially underlying epilepsy. This is the largest series describing electro-clinical features of epilepsy in WBS. In WBS, epilepsy should be considered both in case of typical and atypical deletions, which do not involve HIP1, YWHAG or MAGI2. © 2015 Wiley Periodicals, Inc.

Investigating Inhibitory Control in Children with Epilepsy: An fMRI Study

Science.gov (United States)

Triplett, Regina L.; Velanova, Katerina; Luna, Beatriz; Padmanabhan, Aarthi; Gaillard, William D.; Asato, Miya R.

2014-01-01

SUMMARY Objective Deficits in executive function are increasingly noted in children with epilepsy and have been associated with poor academic and psychosocial outcomes. Impaired inhibitory control contributes to executive dysfunction in children with epilepsy; however, its neuroanatomic basis has not yet been investigated. We used functional Magnetic Resonance Imaging (fMRI) to probe the integrity of activation in brain regions underlying inhibitory control in children with epilepsy. Methods This cross-sectional study consisted of 34 children aged 8 to 17 years: 17 with well-controlled epilepsy and 17 age-and sex-matched controls. Participants performed the antisaccade (AS) task, representative of inhibitory control, during fMRI scanning. We compared AS performance during neutral and reward task conditions and evaluated task-related blood-oxygen level dependent (BOLD) activation. Results Children with epilepsy demonstrated impaired AS performance compared to controls during both neutral (non-reward) and reward trials, but exhibited significant task improvement during reward trials. Post-hoc analysis revealed that younger patients made more errors than older patients and all controls. fMRI results showed preserved activation in task-relevant regions in patients and controls, with the exception of increased activation in the left posterior cingulate gyrus in patients specifically with generalized epilepsy across neutral and reward trials. Significance Despite impaired inhibitory control, children with epilepsy accessed typical neural pathways as did their peers without epilepsy. Children with epilepsy showed improved behavioral performance in response to the reward condition, suggesting potential benefits of the use of incentives in cognitive remediation. PMID:25223606

Common genetic variation and susceptibility to partial epilepsies: a genome-wide association study.

Science.gov (United States)

Kasperaviciūte, Dalia; Catarino, Claudia B; Heinzen, Erin L; Depondt, Chantal; Cavalleri, Gianpiero L; Caboclo, Luis O; Tate, Sarah K; Jamnadas-Khoda, Jenny; Chinthapalli, Krishna; Clayton, Lisa M S; Shianna, Kevin V; Radtke, Rodney A; Mikati, Mohamad A; Gallentine, William B; Husain, Aatif M; Alhusaini, Saud; Leppert, David; Middleton, Lefkos T; Gibson, Rachel A; Johnson, Michael R; Matthews, Paul M; Hosford, David; Heuser, Kjell; Amos, Leslie; Ortega, Marcos; Zumsteg, Dominik; Wieser, Heinz-Gregor; Steinhoff, Bernhard J; Krämer, Günter; Hansen, Jörg; Dorn, Thomas; Kantanen, Anne-Mari; Gjerstad, Leif; Peuralinna, Terhi; Hernandez, Dena G; Eriksson, Kai J; Kälviäinen, Reetta K; Doherty, Colin P; Wood, Nicholas W; Pandolfo, Massimo; Duncan, John S; Sander, Josemir W; Delanty, Norman; Goldstein, David B; Sisodiya, Sanjay M

2010-07-01

Partial epilepsies have a substantial heritability. However, the actual genetic causes are largely unknown. In contrast to many other common diseases for which genetic association-studies have successfully revealed common variants associated with disease risk, the role of common variation in partial epilepsies has not yet been explored in a well-powered study. We undertook a genome-wide association-study to identify common variants which influence risk for epilepsy shared amongst partial epilepsy syndromes, in 3445 patients and 6935 controls of European ancestry. We did not identify any genome-wide significant association. A few single nucleotide polymorphisms may warrant further investigation. We exclude common genetic variants with effect sizes above a modest 1.3 odds ratio for a single variant as contributors to genetic susceptibility shared across the partial epilepsies. We show that, at best, common genetic variation can only have a modest role in predisposition to the partial epilepsies when considered across syndromes in Europeans. The genetic architecture of the partial epilepsies is likely to be very complex, reflecting genotypic and phenotypic heterogeneity. Larger meta-analyses are required to identify variants of smaller effect sizes (odds ratio<1.3) or syndrome-specific variants. Further, our results suggest research efforts should also be directed towards identifying the multiple rare variants likely to account for at least part of the heritability of the partial epilepsies. Data emerging from genome-wide association-studies will be valuable during the next serious challenge of interpreting all the genetic variation emerging from whole-genome sequencing studies.

Long-term outcomes of epilepsy surgery in school-aged children with partial epilepsy.

Science.gov (United States)

Liang, Shuli; Wang, Shuai; Zhang, Junchen; Ding, Chengyun; Zhang, Zhiwen; Fu, Xiangping; Hu, Xiaohong; Meng, Xiaoluo; Jiang, Hong; Zhang, Shaohui

2012-10-01

The pediatric epileptic spectrum and seizure control in surgical patients have been defined in developed countries. However, corresponding data on school-aged children from developing countries are insufficient. We summarized epileptic surgical data from four centers in China, to compare surgical outcomes of school-aged children with intractable partial epilepsy from China and those from developed countries, and introduce surgical candidate criteria. Data from 206 children (aged 6-14 years) undergoing surgical resection for epilepsy between September 2001 and January 2007 were selected. Postoperative freedom from seizures was achieved in 173 cases (84.0%) at 1 year, 149 (72.3%) at 3 years, and 139 (67.5%) at 5 years. Patients with focal magnetic resonance imaging abnormalities and a short history of seizure were most likely to become seizure-free postoperatively. Those with preoperative low intelligence quotients who became seizure-free postoperatively achieved improvements in full memory quotients, intelligence quotients, and overall quality of life at 2 years. Significant differences were evident in mean changes of full intelligence quotient, full memory quotient, and overall quality of life between patients with preoperative low intelligence quotients who received corpus callosotomies and those with a normal preoperative intelligence quotient, and between seizure-free children and those with continual seizures. Copyright © 2012 Elsevier Inc. All rights reserved.

A mouse model of DEPDC5-related epilepsy: Neuronal loss of Depdc5 causes dysplastic and ectopic neurons, increased mTOR signaling, and seizure susceptibility.

Science.gov (United States)

Yuskaitis, Christopher J; Jones, Brandon M; Wolfson, Rachel L; Super, Chloe E; Dhamne, Sameer C; Rotenberg, Alexander; Sabatini, David M; Sahin, Mustafa; Poduri, Annapurna

2018-03-01

DEPDC5 is a newly identified epilepsy-related gene implicated in focal epilepsy, brain malformations, and Sudden Unexplained Death in Epilepsy (SUDEP). In vitro, DEPDC5 negatively regulates amino acid sensing by the mTOR complex 1 (mTORC1) pathway, but the role of DEPDC5 in neurodevelopment and epilepsy has not been described. No animal model of DEPDC5-related epilepsy has recapitulated the neurological phenotypes seen in patients, and germline knockout rodent models are embryonic lethal. Here, we establish a neuron-specific Depdc5 conditional knockout mouse by cre-recombination under the Synapsin1 promotor. Depdc5 flox/flox -Syn1 Cre (Depdc5cc+) mice survive to adulthood with a progressive neurologic phenotype that includes motor abnormalities (i.e., hind limb clasping) and reduced survival compared to littermate control mice. Depdc5cc+ mice have larger brains with increased cortical neuron size and dysplastic neurons throughout the cortex, comparable to the abnormal neurons seen in human focal cortical dysplasia specimens. Depdc5 results in constitutive mTORC1 hyperactivation exclusively in neurons as measured by the increased phosphorylation of the downstream ribosomal protein S6. Despite a lack of increased mTORC1 signaling within astrocytes, Depdc5cc+ brains show reactive astrogliosis. We observed two Depdc5cc+ mice to have spontaneous seizures, including a terminal seizure. We demonstrate that as a group Depdc5cc+ mice have lowered seizure thresholds, as evidenced by decreased latency to seizures after chemoconvulsant injection and increased mortality from pentylenetetrazole-induced seizures. In summary, our neuron-specific Depdc5 knockout mouse model recapitulates clinical, pathological, and biochemical features of human DEPDC5-related epilepsy and brain malformations. We thereby present an important model in which to study targeted therapeutic strategies for DEPDC5-related conditions. Copyright © 2017 Elsevier Inc. All rights reserved.

A study of idiopathic generalised epilepsy in an Irish population.

LENUS (Irish Health Repository)

Mullins, G M

2012-02-03

Idiopathic generalised epilepsy (IGE) is subdivided into syndromes based on clinical and EEG features. PURPOSE: The aim of this study was to characterise all cases of IGE with supportive EEG abnormalities in terms of gender differences, seizure types reported, IGE syndromes, family history of epilepsy and EEG findings. We also calculated the limited duration prevalence of IGE in our cohort. METHODS: Data on abnormal EEGs were collected retrospectively from two EEG databases at two tertiary referral centres for neurology. Clinical information was obtained from EEG request forms, standardised EEG questionnaires and medical notes of patients. RESULTS: two hundred twenty-three patients met our inclusion criteria, 89 (39.9%) male and 134 (60.1%) females. Tonic clonic seizures were the most common seizure type reported, 162 (72.65%) having a generalised tonic clonic seizure (GTCS) at some time. IGE with GTCS only (EGTCSA) was the most common syndrome in our cohort being present in 94 patients (34 male, 60 female), with 42 (15 male, 27 female) patients diagnosed with Juvenile myoclonic epilepsy (JME), 23 (9 male, 14 female) with Juvenile absence epilepsy (JAE) and 20 (9 male, 11 female) with childhood absence epilepsy (CAE). EEG studies in all patients showed generalised epileptiform activity. CONCLUSIONS: More women than men were diagnosed with generalised epilepsy. Tonic clonic seizures were the most common seizure type reported. EGTCSA was the most frequent syndrome seen. Gender differences were evident for JAE and JME as previously reported and for EGTCSA, which was not reported to date, and reached statistical significance for EGTCA and JME.

Brain SPECT with /sup 123/I-isopropyl amphetamine in epilepsy

Energy Technology Data Exchange (ETDEWEB)

Biersack, H.J.; Reske, S.N.; Rasche, A.; Reichmann, K.; Winkler, C.; Froescher, W.; Kluenenberg, H.

1983-04-01

Ten patients were studied with N-isopropyl I-123 p-iodoamphetamine. Single photon emission computed tomography (SPECT) was carried out by hand of a rotating gamma camera system (Gammatome T9000/CGR, high resolution collimator). During 1 rotation (360/sup 0/) 64 frames (4k matrix) were acquired within 20 min 1 hour after injection of 6.5 mCi I-123 labeled amphetamine. The content of I-124 was less than 2%. After reconstruction of transverse slices coronar and sagittal reconstructions were rapidly performed using an array processor. Nine patients suffered from epilepsy and one from severe migraine. Excellent differentiation between gray and white matter of the cerebral cortex and the basal ganglia was evident in all of the cases. In 2 out of 3 patients with epilepsy and negative CT results SPECT revealed circumscribed areas with increased amphetamine uptake in accordance with the EEG findings. In 4 out of 6 cases with positive CT findings SPECT lesions with diminished amphetamine uptake could be established. One patient with severe migraine showed focal increased amphetamine uptake in accordance with the respective clinical results.

Epilepsy with dual pathology: surgical treatment of cortical dysplasia accompanied by hippocampal sclerosis.

Science.gov (United States)

Kim, Dong W; Lee, Sang K; Nam, Hyunwoo; Chu, Kon; Chung, Chun K; Lee, Seo-Young; Choe, Geeyoung; Kim, Hyun K

2010-08-01

The presence of two or more epileptogenic pathologies in patients with epilepsy is often observed, and the coexistence of focal cortical dysplasia (FCD) with hippocampal sclerosis (HS) is one of the most frequent clinical presentations. Although surgical resection has been an important treatment for patients with refractory epilepsy associated with FCD, there are few studies on the surgical treatment of FCD accompanied by HS, and treatment by resection of both neocortical dysplastic tissue and hippocampus is still controversial. We retrospectively recruited epilepsy patients who had undergone surgical treatment for refractory epilepsy with the pathologic diagnosis of FCD and the radiologic evidence of HS. We evaluated the prognostic roles of clinical factors, various diagnostic modalities, surgical procedures, and the severity of pathology. A total of 40 patients were included, and only 35.0% of patients became seizure free. Complete resection of the epileptogenic area (p = 0.02), and the presence of dysmorphic neurons or balloon cells on histopathology (p = 0.01) were associated with favorable surgical outcomes. Patients who underwent hippocampal resection were more likely to have a favorable surgical outcome (p = 0.02). We show that patients with complete resection of epileptogenic area, the presence of dysmorphic neurons or balloon cells on histopathology, or resection of hippocampus have a higher chance of a favorable surgical outcome. We believe that this observation is useful in planning of surgical procedures and predicting the prognoses of individual patients with FCD patients accompanied by HS. Wiley Periodicals, Inc. © 2009 International League Against Epilepsy.

Prevalence of epilepsy in 74,949 school children in Khartoum State, Sudan.

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Mohamed, Inaam N; Elseed, Maha A; Hamed, Ahlam A; Abdel-Rahman, Manar E; El-Sadig, Sarah M; Omer, Ilham M; Osman, Abdelgadir H; Ahmed, Ammar E; Karrar, Zein A; Salih, Mustafa A

2017-08-01

Data on childhood epilepsy in Sudan are scarce and the only published study on its prevalence was published in 1983. This study aimed to determine the current prevalence of epilepsy in school children in Khartoum State. This is an analytical population-based, cross-sectional study conducted in Khartoum State, Sudan. The study included students in the basic (primary) schools aged 6-14 years. Simple random sampling was used to draw a cluster of four of the seven localities comprising Khartoum State. The sample frame consisted of 1609 public schools (808,624 pupils) and 787 private schools (194,613 pupils), a total of 2396 schools (1,003,237 pupils). A sample size of 75 940 pupils was estimated and 250 schools were drawn from a sample frame of 2396 schools using a stratified random sampling technique. Consent was obtained from the headmaster/head teacher of the selected schools who arranged a meeting with the tutor/teacher responsible for each class. The study team asked whether any of the pupils was ever noticed or known to have had any kind of seizures, and a confidential letter was sent to the parents of each identified pupil. The letter included an explanation of the aims of the study, information on the research group and the kind of help the research group could offer; contact numbers and email addresses were made available if they wished to participate in this study. Those who consented to participate were then given an appointment at the Epilepsy Outpatient Department, Gaafar Ibnauf Children's Hospital, Khartoum where they were evaluated by the paediatric neurologist. Altogether, 74,949 pupils were enrolled for the study, 398 of whom were identified initially as having seizures and 332 of whom (83.4%) were identified by a paediatric neurologist. Of the 332, 303 (91.3%) proved to have epileptic seizures, 250 (82.5%) were known to have epilepsy, and 53 (17.5%) were newly diagnosed during the survey. The male to female ratio was 1.5:1. The total prevalence of

Changes in glutamate concentration, glucose metabolism, and cerebral blood flow during focal brain cooling of the epileptogenic cortex in humans.

Science.gov (United States)

Nomura, Sadahiro; Fujii, Masami; Inoue, Takao; He, Yeting; Maruta, Yuichi; Koizumi, Hiroyasu; Suehiro, Eiichi; Imoto, Hirochika; Ishihara, Hideyuki; Oka, Fumiaki; Matsumoto, Mishiya; Owada, Yuji; Yamakawa, Takeshi; Suzuki, Michiyasu

2014-05-01

Recently, focal brain cooling (FBC) was proposed as a method for treating refractory epilepsy. However, the precise influence of cooling on the molecular basis of epilepsy has not been elucidated. Thus the aim of this study was to assess the effect of FBC on glutamate (Glu) concentration, cerebral blood flow (CBF), and glucose metabolism in patients with intractable epilepsy. Nine patients underwent FBC at 15°C for 30 min prior to cortical resection (n = 6) or hippocampectomy (n = 3). Measurement of metabolites and CBF, as well as electrocorticography (ECoG), was performed. Epileptic discharge (ED), as observed by ECoG, disappeared in the cooling period and reappeared in the rewarming period. Glu concentrations were high during the precooling period and were reduced to 51.2% during the cooling period (p = 0.025). Glycerol levels showed a similar decrease (p = 0.028). Lactate concentration was high during the precooling period and was reduced during the cooling period (21.3% decrease; p = 0.005). Glucose and pyruvate levels were maintained throughout the procedure. Changes in CBF were parallel to those observed by ECoG. FBC reduced EDs and concentrations of Glu and glycerol. This demonstrates the neuroprotective effect of FBC. Our findings confirm that FBC is a reasonable and optimal treatment option for patients with intractable epilepsy. Wiley Periodicals, Inc. © 2014 International League Against Epilepsy.

Regression of nodules on cranial computerized tomography (CCT) scans in 4 focal epileptic patients

International Nuclear Information System (INIS)

Bahk, Yong Whee; Yoon, Sei Chul; Park, Seog Hee; Huh, Choon Woong

1983-01-01

Epilepsy can be defined as a paroxysmal, excessive, neuronal discharge within the brain originating from either cortical or sugcortical regions. The incidence of epilepsy is increasing possibly due to the survival of persons who should have died of brain injuries or other cerebral abnormalities acquired in early life. The use of antibiotics and improvement in the medical care have saved many children who might have died of meningitis, brain abscess, encephalitis, severe head injuries, etc. CCT scan is new radiologic procedure for defining cranial and intracranial structures and also an useful procedure for evaluation and follow-up (FU) of patient with focal seizure disorder. Recently we experienced nodules which were isodense or hypodense on initial non contrasted CT (NECT) and scans became hyperdense of the enhancement in 4 cases of focal epileptic seizure. Nearly complete disappearance or regression of the epileptic foci occurred on the FU CT scans in 2 cases. Operation was performed in 2 cases. The tissue specimen obtained from the CT nodule revealed cerebral edema in one case and localized gliosis and congestion in the other. All the patients showed marked clinical improvement when the CT nodule improved. Review of literature failed to disclose any previous report on such observation

Epilepsi

DEFF Research Database (Denmark)

Sabers, Anne; Kjær, Troels W

2014-01-01

Epilepsy affects around 33,000 people in Denmark. The classification of the epilepsies is currently under revision and the clinical course of the disease depends on the underlying aetiology. Diagnostic evaluation includes EEG and often long-term video-EEG monitoring to ensure the diagnosis and clas......-sification. More than two thirds of patients with epilepsy can obtain complete seizure control. The remainders, counting around 12.000 patients in Denmark, having medical refractory epilepsy should be considered for other treatment options; epilepsy surgery or other non-pharmacological treatment....

Depression in Patients with Epilepsy: A Study from Enugu, South ...

African Journals Online (AJOL)

hanumantp

Depression is one of the most prevalent psychiatric disorders occurring in patients with epilepsy.[1] Most of the time it is underrecognized and has a huge impact on their quality of life.[1-3] Patients with epilepsy have a higher prevalence of depression than the general population and studies estimate the incidence to range ...

The value of magnetoencephalography for seizure-onset zone localization in magnetic resonance imaging-negative partial epilepsy.

Science.gov (United States)

Jung, Julien; Bouet, Romain; Delpuech, Claude; Ryvlin, Philippe; Isnard, Jean; Guenot, Marc; Bertrand, Olivier; Hammers, Alexander; Mauguière, François

2013-10-01

Surgical treatment of epilepsy is a challenge for patients with non-contributive brain magnetic resonance imaging. However, surgery is feasible if the seizure-onset zone is precisely delineated through intracranial electroencephalography recording. We recently described a method, volumetric imaging of epileptic spikes, to delineate the spiking volume of patients with focal epilepsy using magnetoencephalography. We postulated that the extent of the spiking volume delineated with volumetric imaging of epileptic spikes could predict the localizability of the seizure-onset zone by intracranial electroencephalography investigation and outcome of surgical treatment. Twenty-one patients with non-contributive magnetic resonance imaging findings were included. All patients underwent intracerebral electroencephalography investigation through stereotactically implanted depth electrodes (stereo-electroencephalography) and magnetoencephalography with delineation of the spiking volume using volumetric imaging of epileptic spikes. We evaluated the spatial congruence between the spiking volume determined by magnetoencephalography and the localization of the seizure-onset zone determined by stereo-electroencephalography. We also evaluated the outcome of stereo-electroencephalography and surgical treatment according to the extent of the spiking volume (focal, lateralized but non-focal or non-lateralized). For all patients, we found a spatial overlap between the seizure-onset zone and the spiking volume. For patients with a focal spiking volume, the seizure-onset zone defined by stereo-electroencephalography was clearly localized in all cases and most patients (6/7, 86%) had a good surgical outcome. Conversely, stereo-electroencephalography failed to delineate a seizure-onset zone in 57% of patients with a lateralized spiking volume, and in the two patients with bilateral spiking volume. Four of the 12 patients with non-focal spiking volumes were operated upon, none became seizure

MRI and HMPAO-SPECT in the diagnosis of epilepsy

International Nuclear Information System (INIS)

Bokisch, A.; Gruenwald, F.; Elger, C.E.; Kaiser, W.A.; Biersack, H.J.

1992-01-01

Eighty-three patients suffering from intractable epilepsy were investigated by magnetic resonance imaging (MRI) and by HMPAO-SPECT during presurgical evaluation. MRI was proven to be most sensitive concerning the detection of focal lesions. Acquisition of thin slices and high signal/noise ratios are prerequisites for optimal sensitivity, the use of Gd-DTPA, in addition, increases the reliability slightly. Histological findings were available in 21 patients and in all cases of focal lesions without prior surgery, malignancy was detected or excluded correctly. The sensitivity was reduced, however, in the 4 patients with recurrency of astrocytoma. 2 cases were missed. Quantitative evaluation of signal intensities in the amygdala/hippocampal region or of the signal dynamics after contrast media application were not helpful in patients without focal lesion. In these patients no reliable criterium to determine the side of the focus with MRI investigations could be established. HMPAO-SPECT investigations were found to be complementary to MRI. In 19 of 36 patients without focal lesions the lateralisation was feasible by scintigraphy. In addition a correlation between the volume asymmetry of the temporal horns detected by MRI and the scintigraphic finding was established. The statistical proven correlation between the unilateral dilatation of the temporal horn and relative hypoperfusion is too weak, however, to imply individual consequences. (orig.) [de

Epileptogenic zone localization using magnetoencephalography predicts seizure freedom in epilepsy surgery

Science.gov (United States)

Englot, Dario J.; Nagarajan, Srikantan S.; Imber, Brandon S.; Raygor, Kunal P.; Honma, Susanne M.; Mizuiri, Danielle; Mantle, Mary; Knowlton, Robert C.; Kirsch, Heidi E.; Chang, Edward F.

2015-01-01

Objective The efficacy of epilepsy surgery depends critically upon successful localization of the epileptogenic zone. Magnetoencephalography (MEG) enables non-invasive detection of interictal spike activity in epilepsy, which can then be localized in three dimensions using magnetic source imaging (MSI) techniques. However, the clinical value of MEG in the pre-surgical epilepsy evaluation is not fully understood, as studies to date are limited by either a lack of long-term seizure outcomes or small sample size. Methods We performed a retrospective cohort study of focal epilepsy patients who received MEG for interictal spike mapping followed by surgical resection at our institution. Results We studied 132 surgical patients, with mean post-operative follow-up of 3.6 years (minimum 1 year). Dipole source modelling was successful in 103 (78%) patients, while no interictal spikes were seen in others. Among patients with successful dipole modelling, MEG findings were concordant with and specific to: i) the region of resection in 66% of patients, ii) invasive electrocorticography (ECoG) findings in 67% of individuals, and iii) the MRI abnormality in 74% of cases. MEG showed discordant lateralization in ~5% of cases. After surgery, 70% of all patients achieved seizure-freedom (Engel class I outcome). Whereas 85% of patients with concordant and specific MEG findings became seizure-free, this outcome was achieved by only 37% of individuals with MEG findings that were non-specific or discordant with the region of resection (χ2 = 26.4, p < 0.001). MEG reliability was comparable in patients with or without localized scalp EEG, and overall, localizing MEG findings predicted seizure freedom with an odds ratio of 5.11 (2.23–11.8, 95% CI). Significance MEG is a valuable tool for non-invasive interictal spike mapping in epilepsy surgery, including patients with non-localized findings on long-term EEG monitoring, and localization of the epileptogenic zone using MEG is associated

Prevalence and incidence of epilepsy: A systematic review and meta-analysis of international studies.

Science.gov (United States)

Fiest, Kirsten M; Sauro, Khara M; Wiebe, Samuel; Patten, Scott B; Kwon, Churl-Su; Dykeman, Jonathan; Pringsheim, Tamara; Lorenzetti, Diane L; Jetté, Nathalie

2017-01-17

To review population-based studies of the prevalence and incidence of epilepsy worldwide and use meta-analytic techniques to explore factors that may explain heterogeneity between estimates. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards were followed. We searched MEDLINE and EMBASE for articles published on the prevalence or incidence of epilepsy since 1985. Abstract, full-text review, and data abstraction were conducted in duplicate. Meta-analyses and meta-regressions were used to explore the association between prevalence or incidence, age group, sex, country level income, and study quality. A total of 222 studies were included (197 on prevalence, 48 on incidence). The point prevalence of active epilepsy was 6.38 per 1,000 persons (95% confidence interval [95% CI] 5.57-7.30), while the lifetime prevalence was 7.60 per 1,000 persons (95% CI 6.17-9.38). The annual cumulative incidence of epilepsy was 67.77 per 100,000 persons (95% CI 56.69-81.03) while the incidence rate was 61.44 per 100,000 person-years (95% CI 50.75-74.38). The prevalence of epilepsy did not differ by age group, sex, or study quality. The active annual period prevalence, lifetime prevalence, and incidence rate of epilepsy were higher in low to middle income countries. Epilepsies of unknown etiology and those with generalized seizures had the highest prevalence. This study provides a comprehensive synthesis of the prevalence and incidence of epilepsy from published international studies and offers insight into factors that contribute to heterogeneity between estimates. Significant gaps (e.g., lack of incidence studies, stratification by age groups) were identified. Standardized reporting of future epidemiologic studies of epilepsy is needed. © 2016 American Academy of Neurology.

Apolipoprotein E in Temporal Lobe Epilepsy: A Case-Control Study

Science.gov (United States)

Kumar, Amit; Tripathi, Manjari; Pandey, Ravindra M.; Ramakrishnan, Lakshmy; Srinivas, M.; Luthra, Kalpana

2006-01-01

Purpose: To investigate the relationship of apolipoprotein E (apoE) genotype, plasma levels of apoE and lipids in temporal lobe epilepsy (TLE) patients in Asian Indians. Status of plasma levels of Apo E in epilepsy patients has not been reported till date. Methods: ApoE gene polymorphism was analyzed in 58 patients with temporal lobe epilepsy (TLE) and 57 age and sex approximated controls using Polymerase chain reaction- restriction fragment length polymorphism (PCR-RFLP). Levels of plasma apoE and lipids were measured using ELISA and enzymatic kits respectively. Results: The distribution of ApoE genotype in epilepsy patients and controls was comparable. Higher levels of plasma ApoE were observed in TLE patients as compared to controls (p = 0.0001). Individuals with plasma levels of apoE > 190 mg/L were at 20 times higher odds (95%CI = 2.46–163.34, p = 0.005), while those with levels of apoE between 150–190 mg/L were at 4.9 times higher odds (95% CI = 1.85–13.9, p = 0.001), to develop TLE. Conclusions: We have observed for the first time, high levels of plasma apoE in epilepsy patients. The findings of this case-control study suggest that apolipoprotein E may play an important role in epilepsy. PMID:17264404

Surgical outcome of MRI-negative refractory extratemporal lobe epilepsy.

Science.gov (United States)

Shi, Jianguo; Lacuey, Nuria; Lhatoo, Samden

2017-07-01

The aim of this study is to determine outcome of resective epilepsy surgery in MRI-negative extratemporal lobe epilepsy (MNETLE) patients who underwent invasive evaluations and to determine factors governing outcome. We studied 28 patients who underwent resective epilepsy surgery for MNETLE from August 2006 to November 2015, in whom complete follow-up information was available. Electro-clinical, pathological and surgical data were evaluated. 24 patients (82.8%) were explored with intracranial EEG (9 stereoelectroencephalography (SEEG), 7 subdural grids and 8 both). All patients were followed for at least 6 months. During a mean follow up period of 32 [6-113] months, 13 (46.4%) patients became seizure-free (ILAE 1) and 18 (64.3%) had a good (ILAE 1, 2, 3) outcome. 21 (75.0%) patients had focal cortical dysplasia (FCD). Univariate analysis showed that more restricted (regional) interictal and ictal epileptiform discharges in surface EEG were significantly associated with seizure freedom (P=0.016 and P=0.024). Multivariate analysis confirmed that having ≥120 electrode contacts in the evaluation is an independent variable predicting seizure freedom (HR=4.283, 95% CI=1.342-13.676, P=0.014). Invasive EEG is a powerful tool in the pre-surgical evaluation of patients with MNETLE. Invasive EEG implantation that include the irritative zone and EEG onset zone as indicated by surface EEG, as well as wider brain coverage predict seizure freedom, contingent upon a sound anatomo-electro-clinical hypothesis for implantation. Copyright © 2017 Elsevier B.V. All rights reserved.

The challenges that parents of children with epilepsy face: A qualitative study.

Science.gov (United States)

Kampra, Matina; Tzerakis, Nikolaos; Lund Holm Thomsen, Louise; Katsarou, Efstathia; Voudris, Konstantinos; D Mastroyianni, Sotiria; Mouskou, Stella; Drossou, Kyriaki S; Siatouni, Anna; Gatzonis, Stylianos

2017-06-01

This qualitative study explored the challenges that Greek parents/caregivers of children with controlled epilepsy (CwE) face regarding the disorder. Interviews were conducted based on open-ended questions guided by a review of the literature. A total of 91 parents/caregivers were recruited by neurologists at the neurology clinics of two Athens public hospitals. A hermeneutic phenomenological approach was used to explore parent/caregiver experiences. The data were grouped and analyzed through a textual interpretation. Two key challenges were identified for parents of CwE: the disclosure of epilepsy and the absence of adequate information about coping with epilepsy. Parents in Greece were hesitant to reveal their child's epilepsy to school staff and their wider social milieu. Also, although satisfied with the patient-centered approach they experienced with their hospital doctor, parents/caregivers found that they needed more education about the existing sources of psychosocial and emotional support to cope with their child's epilepsy personally and as a family. Finally, the parents/caregivers who let their child know about the epilepsy and discussed the implications with the child found that parent-child communication improved. This study provides valuable insight into the impact of epilepsy on parents of CwE, which might help hospital and school staff support families with greater understanding, sensitivity, and skill. The findings suggest that Greek authorities should staff hospitals and schools with experts and more systematically advertise sources of information about epilepsy and ways to cope with it. Copyright © 2017 Elsevier Inc. All rights reserved.

Effects of dual pathology on cognitive outcome following left anterior temporal lobectomy for treatment of epilepsy.

Science.gov (United States)

Prayson, B E; Prayson, R A; Kubu, C S; Bingaman, W; Najm, I M; Busch, R M

2013-09-01

The objective of this retrospective study was to determine if dual pathology [DUAL - focal cortical dysplasia (FCD) and mesial temporal sclerosis (MTS)] in patients with left temporal lobe epilepsy is associated with greater risk for cognitive decline following temporal lobectomy than single pathology (MTS only). Sixty-three adults (Mage=36.5years, female: 52.4%) who underwent left anterior temporal lobectomy for treatment of epilepsy (MTS=28; DUAL=35) completed preoperative and postoperative neuropsychological evaluations. The base rate of dual pathology was 55.5%. Repeated measures ANOVAs yielded significant 2-way interactions (group×time) on most measures of language and memory with generally moderate effect sizes. Specifically, patients with MTS only demonstrated postoperative declines, while those with dual pathology remained unchanged or improved. Results suggest that dual pathology may be associated with better cognitive outcome following epilepsy surgery than MTS alone, possibly reflecting limited functionality of the resected tissue or intrahemispheric reorganization of function in the context of a developmental lesion. Copyright © 2013 Elsevier Inc. All rights reserved.

The Managing Epilepsy Well Network:: Advancing Epilepsy Self-Management.

Science.gov (United States)

Sajatovic, Martha; Jobst, Barbara C; Shegog, Ross; Bamps, Yvan A; Begley, Charles E; Fraser, Robert T; Johnson, Erica K; Pandey, Dilip K; Quarells, Rakale C; Scal, Peter; Spruill, Tanya M; Thompson, Nancy J; Kobau, Rosemarie

2017-03-01

Epilepsy, a complex spectrum of disorders, affects about 2.9 million people in the U.S. Similar to other chronic disorders, people with epilepsy face challenges related to management of the disorder, its treatment, co-occurring depression, disability, social disadvantages, and stigma. Two national conferences on public health and epilepsy (1997, 2003) and a 2012 IOM report on the public health dimensions of epilepsy highlighted important knowledge gaps and emphasized the need for evidence-based, scalable epilepsy self-management programs. The Centers for Disease Control and Prevention translated recommendations on self-management research and dissemination into an applied research program through the Prevention Research Centers Managing Epilepsy Well (MEW) Network. MEW Network objectives are to advance epilepsy self-management research by developing effective interventions that can be broadly disseminated for use in people's homes, healthcare providers' offices, or in community settings. The aim of this report is to provide an update on the MEW Network research pipeline, which spans efficacy, effectiveness, and dissemination. Many of the interventions use e-health strategies to eliminate barriers to care (e.g., lack of transportation, functional limitations, and stigma). Strengths of this mature research network are the culture of collaboration, community-based partnerships, e-health methods, and its portfolio of prevention activities, which range from efficacy studies engaging hard-to-reach groups, to initiatives focused on provider training and knowledge translation. The MEW Network works with organizations across the country to expand its capacity, help leverage funding and other resources, and enhance the development, dissemination, and sustainability of MEW Network programs and tools. Guided by national initiatives targeting chronic disease or epilepsy burden since 2007, the MEW Network has been responsible for more than 43 scientific journal articles, two

Transneuronal degeneration in patients with temporal lobe epilepsy: evaluation by MR imaging

Energy Technology Data Exchange (ETDEWEB)

Kodama, Fumiko; Ogawa, Toshihide; Sugihara, Shuji; Kamba, Masayuki; Kinoshita, Toshibumi [Department of Radiology, Faculty of Medicine' ' Tottori University, 36-1 Nishi-cho, 683-8504, Yonago, Tottori (Japan); Kohaya, Norimasa; Kondo, Shinji [Department of Neurosurgery, Faculty of Medicine' ' Tottori University, 36-1 Nishi-cho, 683-8504, Yonago, Tottori (Japan)

2003-09-01

The aim of this study was to assess the MR imaging findings of transneuronal degeneration of limbic system in the patients with temporal lobe epilepsy, and to detect the influence of surgery on the anatomy of the limbic system. Axial and coronal T1- and T2-weighted MR images were retrospectively analyzed in 34 patients with temporal lobe epilepsy, focusing on transneuronal degeneration. In 17 of the 34 patients, MR images were also analyzed after selective amygdalo-hippocampectomy. Atrophy of the fornix, mamillary body, mamillothalamic tract (MTT), and thalamus ipsilateral to the epileptic focus was demonstrated on MR images in 14.7, 17.6, 8.8, and 11.8% of the 34 patients, respectively. Focal hyperintensity of the thalamus was found on T2-weighted images in 8.8% of the 34 patients. In 17 patients who were evaluated before and after surgery, transneuronal degeneration was seen more frequently after surgery: fornix (11.8 vs 29.4%), mamillary body (11.8 vs 52.9%), MTT (5.9 vs 11.8%), and thalamus (11.8 vs 11.8%). Transneuronal degeneration of the limbic system is clearly demonstrated by MR imaging in patients with temporal lobe epilepsy, and surgical intervention induces transneuronal degeneration more frequently. (orig.)

Transneuronal degeneration in patients with temporal lobe epilepsy: evaluation by MR imaging

International Nuclear Information System (INIS)

Kodama, Fumiko; Ogawa, Toshihide; Sugihara, Shuji; Kamba, Masayuki; Kinoshita, Toshibumi; Kohaya, Norimasa; Kondo, Shinji

2003-01-01

The aim of this study was to assess the MR imaging findings of transneuronal degeneration of limbic system in the patients with temporal lobe epilepsy, and to detect the influence of surgery on the anatomy of the limbic system. Axial and coronal T1- and T2-weighted MR images were retrospectively analyzed in 34 patients with temporal lobe epilepsy, focusing on transneuronal degeneration. In 17 of the 34 patients, MR images were also analyzed after selective amygdalo-hippocampectomy. Atrophy of the fornix, mamillary body, mamillothalamic tract (MTT), and thalamus ipsilateral to the epileptic focus was demonstrated on MR images in 14.7, 17.6, 8.8, and 11.8% of the 34 patients, respectively. Focal hyperintensity of the thalamus was found on T2-weighted images in 8.8% of the 34 patients. In 17 patients who were evaluated before and after surgery, transneuronal degeneration was seen more frequently after surgery: fornix (11.8 vs 29.4%), mamillary body (11.8 vs 52.9%), MTT (5.9 vs 11.8%), and thalamus (11.8 vs 11.8%). Transneuronal degeneration of the limbic system is clearly demonstrated by MR imaging in patients with temporal lobe epilepsy, and surgical intervention induces transneuronal degeneration more frequently. (orig.)

Epilepsy-related clinical factors and psychosocial functions in pediatric epilepsy.

Science.gov (United States)

Eom, Soyong; Eun, So-Hee; Kang, Hoon-Chul; Eun, Baik-Lin; Nam, Sang Ook; Kim, Sun Jun; Chung, Hee Jung; Kwon, Soon Hak; Lee, Young-Mock; Lee, Joon Soo; Kim, Dong Wook; Oh, Kyung Ja; Kim, Heung Dong

2014-08-01

The aim of this study was to identify the different influencing patterns of demographic and epilepsy-related variables on various aspects of psychosocial function in pediatric epilepsy. Five hundred ninety-eight patients with pediatric epilepsy between the ages of 4 and 18 years (boys=360, 60% and girls=238, 40%) and their parents participated in the study. Parents completed the Social Maturity Scale (SMS), the Korean version of the Child Behavior Checklist (K-CBCL), and the Korean version of the Quality of Life in Childhood Epilepsy Questionnaire (K-QOLCE) to assess daily living function, behavior, and quality of life. The Children's Global Assessment Scale (CGAS) was completed by clinicians to assess general adaptive function. Demographic variables, such as age and sex of child, and epilepsy-related clinical variables, including seizure type, seizure frequency, duration of epilepsy, and number of medications, were obtained from medical records. Demographic and epilepsy-related clinical variables had a strong influence (22-32%) on the cognition-related domain such as general adaptive function, school/total competence, and quality of life for cognitive function while a comparatively smaller effect (2-16%) on the more psychological domain including behavioral, emotional, and social variables. Younger age, shorter duration of illness, and smaller number of medications showed a strong positive impact on psychosocial function in pediatric epilepsy, particularly for adaptive function, competence, and quality-of-life aspects. Given the wide range of impact of demographic and clinical variables on various facets of psychosocial functions, more specific understanding of the various aspects of factors and their particular pattern of influence may enable more effective therapeutic approaches that address both the medical and psychological needs in pediatric epilepsy. Copyright © 2014 Elsevier Inc. All rights reserved.

Subtle pathological changes in neocortical temporal lobe epilepsy.

Science.gov (United States)

Ochoa, Juan G; Hentgarden, Diana; Paulzak, Audrey; Ogden, Melissa; Pryson, Richard; Lamle, Markus; Rusyniak, Walter G

2017-06-01

This was a prospective observational study to correlate the clinical symptoms, electrophysiology, imaging, and surgical pathology of patients with temporal lobe epilepsy (TLE) without hippocampal sclerosis. We selected consecutive patients with TLE and normal MRI undergoing temporal lobe resection between April and September 2015. Clinical features, imaging, and functional data were reviewed. Intracranial monitoring and language mapping were performed when it was required according to our team recommendation. Prior to hippocampal resection, intraoperative electrocorticography was performed using depth electrodes in the amygdala and the hippocampus. The resected hippocampus was sent for pathological analysis. Five patients with diagnosis with non-lesional TLE were included. We did not find distinctive clinical features that could be a characteristic of non-lesional TLE. The mean follow-up was 13.2months (11-15months); 80% of patients achieved Engel Class I outcome. There was no distinctive electrographic findings in these patients. Histopathologic analysis was negative for mesial temporal sclerosis. A second blinded independent neuropathologist with expertise in epilepsy found ILAE type I focal cortical dysplasia in the parahippocampal gyrus in all patients. A third independent neuropathologist reported changes in layer 2 with larger pyramidal neurons in 4 cases but concluded that none of these cases met the diagnostic criteria of FCD. Subtle pathological changes could be associated with a parahippocampal epileptic zone and should be investigated in patients with MRI-negative TLE. This study also highlights the lack of interobserver reliability for the diagnosis of mild cortical dysplasia. Finally, selective amygdalo-hippocampectomy or laser ablation of the hippocampus may not control intractable epilepsy in this specific population. Copyright © 2017 Elsevier Inc. All rights reserved.

Connectivity and tissue microstructural alterations in right and left temporal lobe epilepsy revealed by diffusion spectrum imaging.

Science.gov (United States)

Lemkaddem, Alia; Daducci, Alessandro; Kunz, Nicolas; Lazeyras, François; Seeck, Margitta; Thiran, Jean-Philippe; Vulliémoz, Serge

2014-01-01

Focal epilepsy is increasingly recognized as the result of an altered brain network, both on the structural and functional levels and the characterization of these widespread brain alterations is crucial for our understanding of the clinical manifestation of seizure and cognitive deficits as well as for the management of candidates to epilepsy surgery. Tractography based on Diffusion Tensor Imaging allows non-invasive mapping of white matter tracts in vivo. Recently, diffusion spectrum imaging (DSI), based on an increased number of diffusion directions and intensities, has improved the sensitivity of tractography, notably with respect to the problem of fiber crossing and recent developments allow acquisition times compatible with clinical application. We used DSI and parcellation of the gray matter in regions of interest to build whole-brain connectivity matrices describing the mutual connections between cortical and subcortical regions in patients with focal epilepsy and healthy controls. In addition, the high angular and radial resolution of DSI allowed us to evaluate also some of the biophysical compartment models, to better understand the cause of the changes in diffusion anisotropy. Global connectivity, hub architecture and regional connectivity patterns were altered in TLE patients and showed different characteristics in RTLE vs LTLE with stronger abnormalities in RTLE. The microstructural analysis suggested that disturbed axonal density contributed more than fiber orientation to the connectivity changes affecting the temporal lobes whereas fiber orientation changes were more involved in extratemporal lobe changes. Our study provides further structural evidence that RTLE and LTLE are not symmetrical entities and DSI-based imaging could help investigate the microstructural correlate of these imaging abnormalities.

Phenotypic and imaging features of FLNA-negative patients with bilateral periventricular nodular heterotopia and epilepsy.

Science.gov (United States)

Fallil, Zianka; Pardoe, Heath; Bachman, Robert; Cunningham, Benjamin; Parulkar, Isha; Shain, Catherine; Poduri, Annapurna; Knowlton, Robert; Kuzniecky, Ruben

2015-10-01

Periventricular nodular heterotopia (PVNH) is a malformation of cortical development due to impaired neuronal migration resulting in the formation of nodular masses of neurons and glial cells in close proximity to the ventricular walls. We report the clinical characteristics of the largest case series of FLNA-negative patients with seizures and bilateral periventricular heterotopia. Participants were recruited through the Epilepsy Phenome/Genome Project (EPGP), a multicenter collaborative effort to collect detailed phenotypic data and DNA on a large number of individuals with epilepsy, including a cohort with symptomatic epilepsy related to PVNH. Included subjects had epilepsy, and MRI confirmed bilateral PVNH. Magnetic resonance imaging studies were visually and quantitatively reviewed to investigate the topographic extent of PVNH, symmetry, and laterality. We analyzed data on 71 patients with bilateral PVNH. The incidence of febrile seizures was 16.6%. There was at least one other family member with epilepsy in 36.9% of this population. Developmental delay was present in 21.8%. Focal onset seizures were the most common type of seizure presentation (79.3%). High heterotopia burden was strongly associated with female gender and trigonal nodular localization. There was no evidence for differences in brain volume between PVNH subjects and controls. No relationship was observed between heterotopic volume and gender, developmental delay, location of PVNH, ventricular or cerebellar abnormalities, laterality of seizure onset, age at seizure onset, and duration of epilepsy. A direct correlation was observed between high heterotopia burden, female gender, and trigonal location in this large cohort of FLNA-negative bilateral PVNH patients with epilepsy. Quantitative MRI measurements indicated that this correlation is based on the diffuse nature of the heterotopic nodules rather than on the total volume of abnormal heterotopic tissue. Copyright © 2015 Elsevier Inc. All

Epilepsy

Science.gov (United States)

... Epilepsia What Is Epilepsy? Epilepsy comes from a Greek word meaning "to hold or seize," and people ... for epilepsy than somebody whose family has no history of seizures. How Can Doctors Help? If a ...

Ego functions in epilepsy

DEFF Research Database (Denmark)

Sørensen, A S; Hansen, H; Høgenhaven, H

1988-01-01

Two groups of epilepsy patients (28 patients with temporal lobe epilepsy and 15 patients with primary generalized epilepsy) entered a study of personality traits related to epilepsy, based on a modification of Bellak's semistructured interview for assessment of ego strength. Two groups of subjects...... than 15 years when the disease began. The number of anticonvulsants administered did not influence the results. No difference on adaptive level of ego functioning was found between the group with primary generalized epilepsy and the group with temporal lobe epilepsy. Similarly, the temporal lobe...... served as controls: 15 patients with a non-neurological but relapsing disorder, psoriasis, and 15 healthy volunteers. Compared with the group of healthy volunteers, a decreased adaptive level of ego functioning was found in the epilepsy groups, regardless of seizure types and EEG findings, and...

Epilepsy Surgery Series: A Study of 502 Consecutive Patients from a Developing Country

Science.gov (United States)

Al-Otaibi, Faisal; Baz, Salah; Althubaiti, Ibrahim; Aldhalaan, Hisham; MacDonald, David; Abalkhail, Tareq; Fiol, Miguel E.; Alyamani, Suad; Chedrawi, Aziza; Leblanc, Frank; Parrent, Andrew; Maclean, Donald; Girvin, John

2014-01-01

Purpose. To review the postoperative seizure outcomes of patients that underwent surgery for epilepsy at King Faisal Specialist Hospital & Research Centre (KFSHRC). Methods. A descriptive retrospective study for 502 patients operated on for medically intractable epilepsy between 1998 and 2012. The surgical outcome was measured using the ILAE criteria. Results. The epilepsy surgery outcome for temporal lobe epilepsy surgery (ILAE classes 1, 2, and 3) at 12, 36, and 60 months is 79.6%, 74.2%, and 67%, respectively. The favorable 12- and 36-month outcomes for frontal lobe epilepsy surgery are 62% and 52%, respectively. For both parietal and occipital epilepsy lobe surgeries the 12- and 36-month outcomes are 67%. For multilobar epilepsy surgery, the 12- and 36-month outcomes are 65% and 50%, respectively. The 12- and 36-month outcomes for functional hemispherectomy epilepsy surgery are 64.2% and 63%, respectively. According to histopathology diagnosis, mesiotemporal sclerosis (MTS) and benign CNS tumors had the best favorable outcome after surgery at 1 year (77.27% and 84.3%, resp.,) and 3 years (76% and 75%, resp.,). The least favorable seizure-free outcome after 3 years occurred in cases with dual pathology (66.6%). Thirty-four epilepsy patients with normal magnetic resonance imaging (MRI) brain scans were surgically treated. The first- and third-year epilepsy surgery outcome of 17 temporal lobe surgeries were (53%) and (47%) seizure-free, respectively. The first- and third-year epilepsy surgery outcomes of 15 extratemporal epilepsy surgeries were (47%) and (33%) seizure-free. Conclusion. The best outcomes are achieved with temporal epilepsy surgery, mesial temporal sclerosis, and benign CNS tumor. The worst outcomes are from multilobar surgery, dual pathology, and normal MRI. PMID:24627805

Social outcomes of young adults with childhood-onset epilepsy: A case-sibling-control study.

Science.gov (United States)

Baca, Christine B; Barry, Frances; Vickrey, Barbara G; Caplan, Rochelle; Berg, Anne T

2017-05-01

We aimed to compare long-term social outcomes in young adults with childhood-onset epilepsy (cases) with neurologically normal sibling controls. Long-term social outcomes were assessed at the 15-year follow-up of the Connecticut Study of Epilepsy, a community-based prospective cohort study of children with newly diagnosed epilepsy. Young adults with childhood-onset epilepsy with complicated (abnormal neurologic exam findings, abnormal brain imaging with lesion referable to epilepsy, intellectual disability (ID; IQ < 60) or informative history of neurologic insults to which the occurrence of epilepsy might be attributed), and uncomplicated epilepsy presentations were compared to healthy sibling controls. Age, gender, and matched-pair adjusted generalized linear models stratified by complicated epilepsy and 5-year seizure-free status estimated adjusted odds ratios (aORs) and 95% confidence intervals [CIs] for each outcome. The 15-year follow-up included 361 individuals with epilepsy (59% of initial cases; N = 291 uncomplicated and N = 70 complicated epilepsy; mean age 22 years [standard deviation, SD 3.5]; mean epilepsy onset 6.2 years [SD 3.9]) and 173 controls. Social outcomes for cases with uncomplicated epilepsy with ≥5 years terminal remission were comparable to controls; cases with uncomplicated epilepsy <5 years seizure-free were more likely to be less productive (school/employment < 20 h/week) (aOR 3.63, 95% CI 1.83-7.20) and not to have a driver's license (aOR 6.25, 95% CI 2.85-13.72). Complicated cases with epilepsy <5 years seizure-free had worse outcomes across multiple domains; including not graduating high school (aOR 24.97, 95% CI 7.49-83.30), being un- or underemployed (<20 h/week) (aOR 11.06, 95% CI 4.44-27.57), being less productively engaged (aOR 15.71, 95% CI 6.88-35.88), and not living independently (aOR 10.24, 95% CI 3.98-26.36). Complicated cases without ID (N = 36) had worse outcomes with respect to productive engagement (aOR 6.02; 95% CI 2

Posterior cortex epilepsy surgery in childhood and adolescence: Predictors of long-term seizure outcome.

Science.gov (United States)

Ramantani, Georgia; Stathi, Angeliki; Brandt, Armin; Strobl, Karl; Schubert-Bast, Susanne; Wiegand, Gert; Korinthenberg, Rudolf; van Velthoven, Vera; Zentner, Josef; Schulze-Bonhage, Andreas; Bast, Thomas

2017-03-01

We aimed to investigate the long-term seizure outcome of children and adolescents who were undergoing epilepsy surgery in the parietooccipital cortex and determine their predictive factors. We retrospectively analyzed the data of 50 consecutive patients aged 11.1 (mean) ± 5.1 (standard deviation) years at surgery. All patients but one had a magnetic resonance imaging (MRI)-visible lesion. Resections were parietal in 40%, occipital in 32%, and parietooccipital in 28% cases; 24% patients additionally underwent a resection of the posterior border of the temporal lobe. Etiology included focal cortical dysplasia in 44%, benign tumors (dysembryoplastic neuroepithelial tumor, ganglioglioma, angiocentric glioma, and pilocystic astrocytoma) in 32%, peri- or postnatal ischemic lesions in 16%, and tuberous sclerosis in 8% cases. At last follow-up (mean 8 years, range 1.5-18 years), 60% patients remained seizure-free (Engel class I): 30% had discontinued and 20% had reduced antiepileptic drugs. Most seizure recurrences (71%) occurred within the first 6 months, and only three patients presented with seizures ≥2 years after surgery. Independent predictors of seizure recurrence included left-sided as well as parietal epileptogenic zones and resections. Longer epilepsy duration to surgery was identified as the only modifiable independent predictor of seizure recurrence. Our study demonstrates that posterior cortex epilepsy surgery is highly effective in terms of lasting seizure control and antiepileptic drug cessation in selected pediatric candidates. Most importantly, our data supports the early consideration of surgical intervention in children and adolescents with refractory posterior cortex epilepsy. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

Diagnosis and Prognosis of Seizures and Epilepsy in Childhood: Dutch study of epilepsy in childhood

NARCIS (Netherlands)

H. Stroink (Hans)

2008-01-01

textabstractMany people suffer from one or more epileptic seizures during life, but not all these people have epilepsy. Moreover, epilepsy is not one disease or syndrome, but a collection of different disorders, which have in common the repeated occurrence of unprovoked epileptic seizures during

De novo interstitial deletion of 9q32-34.1 with mental retardation, developmental delay, epilepsy, and cortical dysplasia

DEFF Research Database (Denmark)

Tos, T; Alp, M Y; Karacan, C D

2014-01-01

In this report we describe a 10 year-old female patient with interstitial deletion of 9q32-q34.1 associated with mental retardation, developmental delay, short stature, mild facial dysmorphism, epilepsy, abnormal EEG and brain MRI findings consistent with focal cortical dysplasia. Interstitial...

New Classification of Focal Cortical Dysplasia: Application to Practical Diagnosis

Science.gov (United States)

Bae, Yoon-Sung; Kang, Hoon-Chul; Kim, Heung Dong; Kim, Se Hoon

2012-01-01

Background and Purpose: Malformation of cortical development (MCD) is a well-known cause of drug-resistant epilepsy and focal cortical dysplasia (FCD) is the most common neuropathological finding in surgical specimens from drug-resistant epilepsy patients. Palmini’s classification proposed in 2004 is now widely used to categorize FCD. Recently, however, Blumcke et al. recommended a new system for classifying FCD in 2011. Methods: We applied the new classification system in practical diagnosis of a sample of 117 patients who underwent neurosurgical operations due to drug-resistant epilepsy at Severance Hospital in Seoul, Korea. Results: Among 117 cases, a total of 16 cases were shifted to other FCD subtypes under the new classification system. Five cases were reclassified to type IIIa and five cases were categorized as dual pathology. The other six cases were changed within the type I category. Conclusions: The most remarkable changes in the new classification system are the advent of dual pathology and FCD type III. Thus, it will be very important for pathologists and clinicians to discriminate between these new categories. More large-scale research needs to be conducted to elucidate the clinical influence of the alterations within the classification of type I disease. Although the new FCD classification system has several advantages compared to the former, the correlation with clinical characteristics is not yet clear. PMID:24649461

Epilepsy after stroke

DEFF Research Database (Denmark)

Olsen, T S; Høgenhaven, H; Thage, O

1987-01-01

Development of epilepsy was studied prospectively in a group of 77 consecutive stroke patients. Included were stroke patients less than 75 years old admitted within the first 3 days after the stroke. Excluded were patients with subarachnoid hemorrhage, vertebrobasilar stroke, and patients...... with other severe diseases. Cerebral angiography, CT, and EEG were performed in all patients. The patients were followed clinically for 2 to 4 years. Seven patients (9%) developed epilepsy. Of 23 patients with lesions involving the cortex, 6 (26%) developed epilepsy. Of 54 patients in whom the cortex...... was not involved, only 1 (2%) developed epilepsy. Patients with persisting paresis and cortical involvement seem to be at particularly high risk of developing epilepsy, as 50% of such patients (6 of 12) developed the disease....

Seizure characteristics of epilepsy in childhood after acute encephalopathy with biphasic seizures and late reduced diffusion.

Science.gov (United States)

Ito, Yuji; Natsume, Jun; Kidokoro, Hiroyuki; Ishihara, Naoko; Azuma, Yoshiteru; Tsuji, Takeshi; Okumura, Akihisa; Kubota, Tetsuo; Ando, Naoki; Saitoh, Shinji; Miura, Kiyokuni; Negoro, Tamiko; Watanabe, Kazuyoshi; Kojima, Seiji

2015-08-01

The aim of this study was to clarify characteristics of post-encephalopathic epilepsy (PEE) in children after acute encephalopathy with biphasic seizures and late reduced diffusion (AESD), paying particular attention to precise diagnosis of seizure types. Among 262 children with acute encephalopathy/encephalitis registered in a database of the Tokai Pediatric Neurology Society between 2005 and 2012, 44 were diagnosed with AESD according to the clinical course and magnetic resonance imaging (MRI) findings and were included in this study. Medical records were reviewed to investigate clinical data, MRI findings, neurologic outcomes, and presence or absence of PEE. Seizure types of PEE were determined by both clinical observation by pediatric neurologists and ictal video-electroencephalography (EEG) recordings. Of the 44 patients after AESD, 10 (23%) had PEE. The period between the onset of encephalopathy and PEE ranged from 2 to 39 months (median 8.5 months). Cognitive impairment was more severe in patients with PEE than in those without. Biphasic seizures and status epilepticus during the acute phase of encephalopathy did not influence the risk of PEE. The most common seizure type of PEE on clinical observation was focal seizures (n = 5), followed by epileptic spasms (n = 4), myoclonic seizures (n = 3), and tonic seizures (n = 2). In six patients with PEE, seizures were induced by sudden unexpected sounds. Seizure types confirmed by ictal video-EEG recordings were epileptic spasms and focal seizures with frontal onset, and all focal seizures were startle seizures induced by sudden acoustic stimulation. Intractable daily seizures remain in six patients with PEE. We demonstrate seizure characteristics of PEE in children after AESD. Epileptic spasms and startle focal seizures are common seizure types. The specific seizure types may be determined by the pattern of diffuse subcortical white matter injury in AESD and age-dependent reorganization of the brain

The evaluation of FDG-PET imaging for epileptogenic focus localization in patients with MRI positive and MRI negative temporal lobe epilepsy

International Nuclear Information System (INIS)

Gok, Beril; Jallo, George; Hayeri, Reza; Wahl, Richard; Aygun, Nafi

2013-01-01

We studied the contribution of interictal FDG-PET ([18 F] fluorodeoxyglucose-positron emission tomography) in epileptic focus identification in temporal lobe epilepsy patients with positive, equivocal and negative magnetic resonance imaging (MRI). Ninety-eight patients who underwent surgical treatment for drug resistant temporal lobe epilepsy after neuropsychological evaluation, scalp video EEG monitoring, FDG-PET, MRI and/or long-term intracranial EEG and with >12 months clinical follow-up were included in this study. FDG-PET findings were compared to MRI, histopathology, scalp video EEG and long-term intracranial EEG monitoring. FDG-PET lateralized the seizure focus in 95 % of MRI positive, 69 % of MRI equivocal and 84 % of MRI negative patients. There was no statistically significant difference between the surgical outcomes among the groups with Engel class I and II outcomes achieved in 86 %, 86 %, 84 % of MRI positive, equivocal and negative temporal lobe epilepsy patients, respectively. The patients with positive unilateral FDG-PET demonstrated excellent postsurgical outcomes, with 96 % Engel class I and II. Histopathology revealed focal lesions in 75 % of MRI equivocal, 84 % of MRI positive, and 23 % of MRI negative temporal lobe epilepsy cases. FDG-PET is an accurate noninvasive method in lateralizing the epileptogenic focus in temporal lobe epilepsy, especially in patients with normal or equivocal MRIs, or non-lateralized EEG monitoring. Very subtle findings in MRI are often associated with histopathological lesions and should be described in MRI reports. The patients with negative or equivocal MRI temporal lobe epilepsy are good surgical candidates with comparable postsurgical outcomes to patients with MRI positive temporal lobe epilepsy. (orig.)

The evaluation of FDG-PET imaging for epileptogenic focus localization in patients with MRI positive and MRI negative temporal lobe epilepsy

Energy Technology Data Exchange (ETDEWEB)

Gok, Beril [Drexel University, Department of Radiology, Mercy Catholic Medical Center, Philadelphia, PA (United States); Johns Hopkins University, Division of Nuclear Medicine, Department of Radiology and Radiological Sciences, Baltimore, MD (United States); Jallo, George [Johns Hopkins University, Department of Neurosurgery, Baltimore, MD (United States); Hayeri, Reza [Drexel University, Department of Radiology, Mercy Catholic Medical Center, Philadelphia, PA (United States); Wahl, Richard [Johns Hopkins University, Division of Nuclear Medicine, Department of Radiology and Radiological Sciences, Baltimore, MD (United States); Aygun, Nafi [Johns Hopkins University, Division of Neuroradiology, Department of Radiology and Radiological Sciences, Baltimore, MD (United States)

2013-05-15

We studied the contribution of interictal FDG-PET ([18 F] fluorodeoxyglucose-positron emission tomography) in epileptic focus identification in temporal lobe epilepsy patients with positive, equivocal and negative magnetic resonance imaging (MRI). Ninety-eight patients who underwent surgical treatment for drug resistant temporal lobe epilepsy after neuropsychological evaluation, scalp video EEG monitoring, FDG-PET, MRI and/or long-term intracranial EEG and with >12 months clinical follow-up were included in this study. FDG-PET findings were compared to MRI, histopathology, scalp video EEG and long-term intracranial EEG monitoring. FDG-PET lateralized the seizure focus in 95 % of MRI positive, 69 % of MRI equivocal and 84 % of MRI negative patients. There was no statistically significant difference between the surgical outcomes among the groups with Engel class I and II outcomes achieved in 86 %, 86 %, 84 % of MRI positive, equivocal and negative temporal lobe epilepsy patients, respectively. The patients with positive unilateral FDG-PET demonstrated excellent postsurgical outcomes, with 96 % Engel class I and II. Histopathology revealed focal lesions in 75 % of MRI equivocal, 84 % of MRI positive, and 23 % of MRI negative temporal lobe epilepsy cases. FDG-PET is an accurate noninvasive method in lateralizing the epileptogenic focus in temporal lobe epilepsy, especially in patients with normal or equivocal MRIs, or non-lateralized EEG monitoring. Very subtle findings in MRI are often associated with histopathological lesions and should be described in MRI reports. The patients with negative or equivocal MRI temporal lobe epilepsy are good surgical candidates with comparable postsurgical outcomes to patients with MRI positive temporal lobe epilepsy. (orig.)

Prevalence and treatment gap in childhood epilepsy in a north Indian city: a community-based study.

Science.gov (United States)

Pandey, Swati; Singhi, Pratibha; Bharti, Bhavneet

2014-04-01

Epilepsy is one of the most common neurological disorders prevalent in childhood period. There is scarcity of epidemiological data, required to plan services in resource constrained developing nations. To study the prevalence and treatment gap in childhood epilepsy in north Indian city, in the age group of 1-18 years. A two stage stratified cluster sampling; probability proportionate to size (PPS) was employed. A ten question screening questionnaire was employed to identify the presence of epilepsy. Definitions provided by International League against Epilepsy (ILAE) were used to classify screen positive subjects as epilepsy and to calculate the treatment gap. The prevalence rate for epilepsy was 6.24/1000 population. Febrile seizures and neurocysticercosis were most common causes of symptomatic seizures in childhood. This study of epidemiology of epilepsy provides valuable aid in optimizing effective community approach, thereby improving outcomes of childhood epilepsy.

Encephalopathy with status epilepticus during sleep (ESES) induced by oxcarbazepine in idiopathic focal epilepsy in childhood

DEFF Research Database (Denmark)

Pavlidis, Elena; Rubboli, Guido; Nikanorova, Marina

2015-01-01

Encephalopathy with status epilepticus during sleep (ESES) is an age-related disorder characterized by neuropsychological regression, epilepsy and a typical EEG pattern of continuous epileptiform activity (> 85%) during NREM sleep. Cases of worsening or induction of ESES with phenytoin...

Epilepsy

Science.gov (United States)

... eventually become less frequent or disappear altogether. What Causes Epilepsy? This's no clear-cut answer to why people ... epilepsy. Often doctors can't pinpoint the exact cause of a person's epilepsy. But scientists do know that some things can ...

Small temporal pole encephalocele: A hidden cause of "normal" MRI temporal lobe epilepsy.

Science.gov (United States)

Toledano, Rafael; Jiménez-Huete, Adolfo; Campo, Pablo; Poch, Claudia; García-Morales, Irene; Gómez Angulo, Juan Carlos; Coras, Roland; Blümcke, Ingmar; Álvarez-Linera, Juan; Gil-Nagel, Antonio

2016-05-01

Small temporal pole encephalocele (STPE) can be the pathologic substrate of epilepsy in a subgroup of patients with noninformative magnetic resonance imaging (MRI). Herein, we analyzed the clinical, neurophysiologic, and radiologic features of the epilepsy found in 22 patients with STPE, and the frequency of STPE in patients with refractory focal epilepsy (RFE). We performed an observational study of all patients with STPE identified at our epilepsy unit from January 2007 to December 2014. Cases were detected through a systematic search of our database of RFE patients evaluated for surgery, and a prospective collection of patients identified at the outpatient clinic. The RFE database was also employed to analyze the frequency of STPE among the different clinical subgroups. We identified 22 patients with STPE (11 women), including 12 (4.0%) of 303 patients from the RFE database, and 10 from the outpatient clinic. The median age was 51.5 years (range 29-75) and the median age at seizure onset was 38.5 years (range 15-73). Typically, 12 (80%) of 15 patients with left STPE reported seizures with impairment of language. Among the RFE cases, STPE were found in 9.6% of patients with temporal lobe epilepsy (TLE), and in 0.5% of those with extra-TLE (p = 0.0001). STPEs were more frequent in TLE patients with an initial MRI study reported as normal (23.3%) than in those with MRI-visible lesions (1.4%; p = 0.0002). Stereo-electroencephalography was performed in four patients, confirming the localization of the epileptogenic zone at the temporal pole with late participation of the hippocampus. Long-term seizure control was achieved in four of five operated patients. STPE can be a hidden cause of TLE in a subgroup of patients with an initial report of "normal" MRI. Early identification of this lesion may help to select patients for presurgical evaluation and tailored resection. Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.

Clinical and electrophysiological findings in patients with phenylketonuria and epilepsy: Reflex features.

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Yildiz Celik, Senay; Bebek, Nerses; Gurses, Candan; Baykan, Betul; Gokyigit, Aysen

2018-03-23

Phenylketonuria (PKU) is the most common form of amino acid metabolism disorders with autosomal recessive inheritance. The brain damage can be prevented by early diagnosis and a phenylalanine-restricted diet. Untreated or late-treated patients may show mental retardation and other cognitive dysfunctions, as well as motor disability and/or epilepsy. Three patients with PKU and epilepsy were recognized to have reflex epileptic features, and there were ten consecutive adult patients with PKU and epilepsy who were evaluated retrospectively. Medical history, ages at diagnosis and therapy onset, age at seizure onset, seizure types and reflex features, neurological findings, cranial imaging, electroencephalography (EEG) findings, and final clinical condition were evaluated. Reflex epilepsy features were examined in detail. The cases (6 females, 4 males) were diagnosed at ages between 3.5months and 12years. All patients had various degrees of mental-motor retardation and focal or generalized seizures with age at seizure onset varied between neonatal period and 15years. Three patients had febrile seizure, 3 patients had myoclonia, and 3 patients had status epilepticus. All patients had abnormal EEG findings except one. There was a slowing of background activity, and generalized discharges were observed in 7 patients; 3 of them had asymmetrical discharges. One patient had right hippocampal sclerosis (HS), and another patient had hypointensities in the basal ganglia and corpus callosum. Reflex features were clinically observed in 3 of the patients; however, EEG results did not show any related findings. One patient had reflex seizures triggered by photic stimuli, hot water, and startling; one by photic stimuli; and the other one by startling. Reports on the clinical and electrophysiological features of adult patients with PKU were scant. We emphasized that reflex clinical features may be observed in this metabolic disease, and focal epileptiform abnormalities and asymmetry

Depression and genetic causal attribution of epilepsy in multiplex epilepsy families.

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Sorge, Shawn T; Hesdorffer, Dale C; Phelan, Jo C; Winawer, Melodie R; Shostak, Sara; Goldsmith, Jeff; Chung, Wendy K; Ottman, Ruth

2016-10-01

Rapid advances in genetic research and increased use of genetic testing have increased the emphasis on genetic causes of epilepsy in patient encounters. Research in other disorders suggests that genetic causal attributions can influence patients' psychological responses and coping strategies, but little is known about how epilepsy patients and their relatives will respond to genetic attributions of epilepsy. We investigated the possibility that among members of families containing multiple individuals with epilepsy, depression, the most frequent psychiatric comorbidity in the epilepsies, might be related to the perception that epilepsy has a genetic cause. A self-administered survey was completed by 417 individuals in 104 families averaging 4 individuals with epilepsy per family. Current depression was measured with the Patient Health Questionnaire. Genetic causal attribution was assessed by three questions addressing the following: perceived likelihood of having an epilepsy-related mutation, perceived role of genetics in causing epilepsy in the family, and (in individuals with epilepsy) perceived influence of genetics in causing the individual's epilepsy. Relatives without epilepsy were asked about their perceived chance of developing epilepsy in the future, compared with the average person. Prevalence of current depression was 14.8% in 182 individuals with epilepsy, 6.5% in 184 biologic relatives without epilepsy, and 3.9% in 51 individuals married into the families. Among individuals with epilepsy, depression was unrelated to genetic attribution. Among biologic relatives without epilepsy, however, prevalence of depression increased with increasing perceived chance of having an epilepsy-related mutation (p = 0.02). This association was not mediated by perceived future epilepsy risk among relatives without epilepsy. Depression is associated with perceived likelihood of carrying an epilepsy-related mutation among individuals without epilepsy in families containing

Evaluation of knowledge about epilepsy and attitudes towards patients with epilepsy among university students in Upper Egypt.

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Thabit, Mohamed N; Sayed, Mohamed A; Ali, Magda M

2018-05-05

Epilepsy is a major public health problem worldwide. There are many misconceptions about people's knowledge and attitudes about epilepsy, which influence people's behavior towards patients with epilepsy. We conducted a cross-sectional study in Sohag University, a public Egyptian University, in Upper Egypt. We used an Arabic language designed questionnaire to assess people's knowledge about epilepsy and their attitudes towards patients with epilepsy. We included a total of 920 students in the study. 12.4% of study respondents had never heard of or read about epilepsy. Moreover, there was much misunderstanding about the etiology of epilepsy, as 68.2% of epileptic and 74.5% of nonepileptic respondents believe epilepsy is caused by evil spirits and evil eyes or due to psychiatric disorders. There were also many people who held negative attitudes towards patients with epilepsy in regards to major life milestones such as marriage and having children. Among nonepileptics, 54.5% believe epileptics should not marry and 49.9% believe they should not have children. Among patients with epilepsy, these percentages are 27.3% and 36.4% respectively. Knowledge about epilepsy is insufficient and should be increased. The attitudes towards patients with epilepsy are negative and should be changed in Upper Egypt. Copyright © 2018 Elsevier B.V. All rights reserved.

Clinical features and long term outcome of epilepsy in periventricular nodular heterotopia. Simple compared with plus forms

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d'Orsi, G; Tinuper, P; Bisulli, F; Zaniboni, A; Bernardi, B; Rubboli, G; Riva, R; Michelucci, R; Volpi, L; Tassinari, C; Baruzzi, A

2004-01-01

Objectives: Little is known about the long term outcome of patients with periventricular nodular heterotopia (PNH) and epilepsy, particularly the course of seizures. This study investigated the electroclinical and prognostic features of 16 patients with PNH. Methods: Of 120 patients with epilepsy and malformations of cortical development, 16 had PNH. Of these, eight patients had periventricular nodules only (simple PNH) and eight also presented with other cortical or cerebral malformations (subcortical heterotopia; polymicrogyria; focal dysplasia; schizencephaly; cortical infolding; agenesis of the corpus callosum; mega cisterna magna and cerebellar atrophy) (PNH plus). All patients underwent clinical, neurophysiological, and MRI investigation. The mean follow up was 17.3 years (2–40 years). Results: Two electroclinical patterns emerged: (1) The first pattern, associated with simple PNH, was characterised by normal intelligence and seizures, usually partial, which began during the second decade of life. The seizures never became frequent and tended to disappear or become very rare. The EEG showed focal abnormalities. (2) The second pattern, associated with PNH plus, was characterised by mental retardation and seizures that began during the first decade of life. The seizures were very frequent in most cases and sudden drops were observed in six patients. Seizures were medically refractory in four patients. The EEG showed focal and bisynchronous abnormalities. Conclusions: Two groups of PNH patients with different electroclinical and neuroradiological features can be identified after a long term follow up. The presence of other types of cortical or cerebral malformations, in addition to periventricular nodules, determines a poor prognosis. PMID:15146004

A brief history of epilepsy and its therapy in the Western Hemisphere.

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Gross, R A

1992-07-01

The history of epilepsy and its treatment in the western world dates back at least 4 millennia to the ancient civilization of the middle east. Past and present treatments have been empirical, usually reflecting the prevailing views of epilepsy, be they medical, theological or superstitious. Ancient physicians relied on clinical observation to distinguish between epileptic syndromes and infer their cause. Early pathophysiological theories of epilepsy correctly identified the brain as the site of the problem, but emphasized incorrect causes such as an excess of phlegm in the brain. Treatments consisted of prescribed diets or living conditions, occasional surgery such as bloodletting or skull trephination and medicinal herbs. These treatments, often ineffective, had the intellectual advantage of being based on pathophysiological principles, unlike current, more empirical, therapies. The unfortunate but widely held view of epilepsy as being due to occult or evil influences gained adherents even in the medical world during ancient times, and the later acceptance of Christianity allowed theological interpretations of seizures as well. Magical or religious treatments were more frequently prescribed as a result, practices which persist to this day. In the Renaissance an attempt was made to view epilepsy as a manifestation of physical illness rather than a moral or occult affliction, but it was during the Enlightenment that epilepsy was viewed along more modern lines, helped by advances in anatomy and pathology and the development of chemistry, pharmacy and physiology. The idea that focal irritation may cause seizures came about from clinical and experimental work, and was supported by the successful control of seizures by the (sedative) bromides and barbiturates in the late 19th century. The introduction of phenytoin showed that non-sedative drugs could be effective in controlling seizures as well, and the development of in vivo seizure models widened the scope of

Nonsymptomatic generalized epilepsy in children younger than six years : Excellent prognosis, but classification should be reconsidered after follow-up: The Dutch study of epilepsy in childhood

NARCIS (Netherlands)

Middeldorp, CM; Geerts, AT; Stroink, H; van Donselaar, CA; Arts, WFM

Purpose: To assess the prognosis and the accuracy of the epilepsy classification in young children with nonsymptomatic generalized epilepsy. Methods: Of the cohort of the Dutch Study of Epilepsy in Childhood (n = 466), all children younger than 6 years with a diagnosis of idiopathic (IGE) or

[Epilepsy: incidens, prevalens and causes].

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Forsgren, Lars; Sundelin, Heléne; Sveinsson, Olafur

2018-05-21

Epilepsy affects people in all ages with the highest incidence in small children, particularly before age one year, and in elderly aged 65 years and older. In Sweden, between 4500-5000 persons develop epilepsy annually. Based on studies from North America and Europe, including the Nordic countries, the number of people with active epilepsy in Sweden is between 60000-70000. The lifetime risk for epilepsy up to age 85 years is 4-5 %, i.e. approximately every 25th person. The new epilepsy classification divides etiology into the following groups: structural, genetic, infectious, metabolic, immune and unknown. The majority (70%) of people with epilepsy eventually become seizure free. Epilepsy increases the risk of psychosocial problems and accidents. People with epilepsy have up to a 3-fold increase in mortality, mainly due to the underlying causes and epilepsy related deaths, e.g. status epilepticus, SUDEP and accidents. Somatic, psychiatric and neuropsychiatric comorbidities are common in epilepsy.

A study of brain MRI findings in children with epilepsy

Energy Technology Data Exchange (ETDEWEB)

Kanematsu, Sachiko; Sumida, Sawako; Muto, Ayako; Osawa, Makiko; Ono, Yuko [Tokyo Women' s Medical Coll. (Japan); Uchida, Moriyasu; Maruyama, Hiroshi

2000-06-01