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Sample records for seizures lennox-gastaut syndrome

  1. Lennox-Gastaut Syndrome

    Science.gov (United States)

    ... and supports a broad program of basic and clinical research on epilepsy including Lennox-Gastaut syndrome. These studies are aimed at finding the causes of these disorders, improving ... Publications Definition Lennox- ...

  2. Neurocisticercose e síndrome de lennox-gastaut: relato de caso Neurocysticercosis and Lennox-Gastaut syndrome: case report

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    SVETLANA AGAPEJEV

    2000-06-01

    Full Text Available Relata-se o caso de uma menina que, aos 2 anos de idade, apresentou a forma epiléptica, hidrocefálica e encefalítica da neurocisticercose, diagnosticada por exame do líquido cefalorraqueano e tomografia computadorizada de crânio, evolução com crises polimórficas, episódios de descompensação da hipertensão intracraniana por obstrução do sistema de derivação ventriculoperitoneal, retardo no desenvolvimento neuropsicomotor e cegueira até que, aos 10 anos de idade, foi diagnosticada síndrome de Lennox-Gastaut. Atualmente, a paciente tem 16 anos, apresenta sequelas neurológicas e crises parciais complexas com automatismos, parcialmente controladas com o uso de clobazan e oxcarbazepina. A primeira associação de neurocisticercose e síndrome de Lennox-Gastaut foi descrita em 1973, por Frochtengarten & Scarante, em uma menina com quadro clínico semelhante ao do caso relatado.Report of a girl with the epileptic, hydrocephalic and encephalitic form of neurocysticercosis, diagnosed by cerebrospinal flui and computed tomography exams, during her second year of life and an evolution with multiple types of seizures, prolonged periods of intracranial hypertension due to obstruction in the ventriculoperitoneal shunt, psicomotor regression and blindness until she was 10 years old, when the Lennox-Gastaut syndrome was diagnosed. Nowadays the patient is 16 years old and presents complex partial seizures with automatism not completely controlled with clobazan and oxcarbazepine, associated to left spastic hemiparesis, universal hyperreflexia, psychomotor agitation, self-mutilation, amaurosis and severe mental retardation. The association between neurocysticercosis and Lennox-Gastaut syndrome was first described in 1973 by Frochtengarten & Scarante in a Brazilian girl with a similar clinical picture.

  3. Clobazam-Treated Patients with Lennox Gastaut Syndrome Experienced Fewer Seizure-Related Injuries than Placebo Patients During Trail OV-1012

    Science.gov (United States)

    2016-08-19

    severe seizure- related AEs occurred in the placebo group, with three patients experiencing one severe AE each (fall, contusion, or jaw fracture ). In all...only one non–seizure- related injury, and therefore no evidence to suggest that the drug was associated with injuries. The analysis also helps to...Clobazam-treated patients with Lennox-Gastaut syndrome experienced fewer seizure- related injuries than placebo patients during trial OV-1012 *Jouko

  4. Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions

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    Ostendorf AP

    2017-04-01

    Full Text Available Adam P Ostendorf,1 Yu-Tze Ng2 1Department of Pediatrics, Neurology Section, Nationwide Children’s Hospital, The Ohio State University, Columbus, OH, 2Department of Pediatrics, Baylor College of Medicine, The Children’s Hospital of San Antonio, San Antonio, TX, USA Abstract: Lennox-Gastaut syndrome is a severe, childhood-onset electroclinical syndrome comprised of multiple seizure types, intellectual and behavioral disturbances and characteristic findings on electroencephalogram of slow spike and wave complexes and paroxysmal fast frequency activity. Profound morbidity often accompanies a common and severe seizure type, the drop attack. Seizures often remain refractory, or initial treatment efficacy fades. Few individuals are seizure free despite the development of multiple generations of antiseizure medications over decades and high-level evidence on several choices. Approved medications such as lamotrigine, topiramate, rufinamide, felbamate and clobazam have demonstrated efficacy in reducing seizure burden. Cannabidiol has emerged as a promising investigational therapy with vast social interest yet lacks a standard, approved formulation. Palliative surgical procedures, such as vagal nerve stimulation and corpus callosotomy may provide reduction in total seizures and drop attacks. Emerging evidence suggests that complete callosotomy provides greater improvement in seizures without additional side effects. Etiologies such as dysplasia or hypothalamic hamartoma may be amenable for focal resection and thus offer potential to reverse this devastating epileptic encephalopathy. Keywords: Lennox-Gastaut syndrome, epilepsy, epilepsy surgery, cannabidiol, epileptic encephalopathy

  5. Cannabidiol in patients with seizures associated with Lennox-Gastaut syndrome (GWPCARE4): a randomised, double-blind, placebo-controlled phase 3 trial.

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    Thiele, Elizabeth A; Marsh, Eric D; French, Jacqueline A; Mazurkiewicz-Beldzinska, Maria; Benbadis, Selim R; Joshi, Charuta; Lyons, Paul D; Taylor, Adam; Roberts, Claire; Sommerville, Kenneth

    2018-03-17

    Patients with Lennox-Gastaut syndrome, a rare, severe form of epileptic encephalopathy, are frequently treatment resistant to available medications. No controlled studies have investigated the use of cannabidiol for patients with seizures associated with Lennox-Gastaut syndrome. We therefore assessed the efficacy and safety of cannabidiol as an add-on anticonvulsant therapy in this population of patients. In this randomised, double-blind, placebo-controlled trial done at 24 clinical sites in the USA, the Netherlands, and Poland, we investigated the efficacy of cannabidiol as add-on therapy for drop seizures in patients with treatment-resistant Lennox-Gastaut syndrome. Eligible patients (aged 2-55 years) had Lennox-Gastaut syndrome, including a history of slow (caregivers, investigators, and individuals assessing data were masked to group assignment. The primary endpoint was percentage change from baseline in monthly frequency of drop seizures during the treatment period, analysed in all patients who received at least one dose of study drug and had post-baseline efficacy data. All randomly assigned patients were included in the safety analyses. This study is registered with ClinicalTrials.gov, number NCT02224690. Between April 28, 2015, and Oct 15, 2015, we randomly assigned 171 patients to receive cannabidiol (n=86) or placebo (n=85). 14 patients in the cannabidiol group and one in the placebo group discontinued study treatment; all randomly assigned patients received at least one dose of study treatment and had post-baseline efficacy data. The median percentage reduction in monthly drop seizure frequency from baseline was 43·9% (IQR -69·6 to -1·9) in the cannibidiol group and 21·8% (IQR -45·7 to 1·7) in the placebo group. The estimated median difference between the treatment groups was -17·21 (95% CI -30·32 to -4·09; p=0·0135) during the 14-week treatment period. Adverse events occurred in 74 (86%) of 86 patients in the cannabidiol group and 59 (69%) of

  6. Secondary late-onset Lennox-Gastaut syndrome: a critical view

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    Amilton Antunes Barreira

    1984-06-01

    Full Text Available From a group of 66 patients with the Lennox-Gastaut syndrome, 12 whose manifestations had started after the 6th year of life were selected for study. These patients were observed clinically and electroencephalographically for an average period of 2.5 years. We concluded that the late-onset syndrome can: occur after a long interval between diffuse encephalopathy and the first clinical manifestations, with or without previous alterations in psychomotor development; be associated from the onset with serious mental retardation; exhibit simple, complex and mixed seizures similar to those observed in the early form. These patients can also: suffer complex and mixed epileptic seizures previously unreported; paroxismal interictal EEG abnormalities that overlap those of the early form; and spike-slow wave complexes in the EEG that can be actived by hyperpnea. Our results demonstrate that the incidence of LGS after 6 years of age does not necessarily imply a lower frequency of organic antecedents, or beter neu-ropsychomotor development up to the onset of the syndrome or the presence of a higher rate of nonspecific seizures (generalized or partial seizures, and mainly those with elaborate symptomatolgy. The critical and encephalographic expression of the syndrome, which is secondary and starts after the 6th year of age, may depend at least in part on the age when diffuse encephalopathy started.

  7. An unfortunate challenge: Ketogenic diet for the treatment of Lennox-Gastaut syndrome in tyrosinemia type 1.

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    De Lucia, Silvana; Pichard, Samia; Ilea, Adina; Greneche, Marie-Odile; François, Laurent; Delanoë, Catherine; Schiff, Manuel; Auvin, Stéphane

    2016-07-01

    The ketogenic diet is an evidence-based treatment for resistant epilepsy including Lennox-Gastaut syndrome. This diet is based on low carbohydrate-high fat intakes. Dietary treatment is also therapeutic for inborn errors of metabolism such as aminoacdiopathies. We report a child with both Lennox-Gastaut syndrome and tyrosinemia type 1. This epilepsy syndrome resulted form a porencephalic cyst secondary to brain abscesses that occurred during the management of malnutrition due to untreated tyrosinemia type 1. We used a ketogenic diet as treatment for Lennox-Gastaut syndrome taking into account dietary requirements for tyrosinemia type 1. The patient was transiently responder during a 6-month period. This report illustrates that ketogenic diet remains a therapeutic option even when additional dietary requirements are needed. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  8. Conceptualising Lennox-Gastaut Syndrome as a secondary network epilepsy

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    John S ARCHER

    2014-10-01

    Full Text Available Lennox-Gastaut Syndrome (LGS is a category of severe, disabling epilepsy, characterised by frequent, treatment-resistant seizures and cognitive impairment. EEG (electroencephalography shows characteristic generalised epileptic activity that is similar in those with lesional, genetic or unknown causes, suggesting a common underlying mechanism. The condition typically begins in young children, leaving many severely disabled with recurring seizures throughout their adult life.Scalp EEG of the tonic seizures of LGS is characterised by a diffuse high voltage slow transient evolving into generalised low voltage fast activity, likely reflecting sustained fast neuronal firing over a wide cortical area. The typical interictal discharges (runs of slow spike-and-wave (SSW and bursts of generalised paroxysmal fast activity (GPFA also have a ‘generalised’ electrical field, suggesting widespread cortical involvement. Recent brain mapping studies have begun to reveal which cortical and subcortical regions are active during these ‘generalised’ discharges.In this critical review we examine findings from neuroimaging studies of LGS and place these in the context of the electrical and clinical features of the syndrome. We suggest that LGS can be conceptualised as a ‘secondary network epilepsy’, where the epileptic activity is expressed through large-scale brain networks, particularly the attention and default-mode networks. Cortical lesions, when present, appear to chronically interact with these networks to produce network instability rather than triggering each individual epileptic discharge. LGS can be considered a ‘secondary’ network epilepsy because the epileptic manifestations of the disorder reflect the networks being driven, rather than the specific initiating process.

  9. Ketogenic Diet and Hormonal Therapy in Prevention of Evolution of West Syndrome to Lennox-Gastaut

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    J Gordon Millichap

    2009-08-01

    Full Text Available Medical records of 98 patients diagnosed with West syndrome and monitored at Sanggye Paik Hospital, Seoul, Korea, for at least 3 years were retrospectively reviewed to assess etiology, age at onset, value of various therapies, and the rate of evolution from West syndrome to Lennox-Gastaut syndrome.

  10. Lennox gastaut syndrome, review of the literature and a case report

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    Abu Saleh Tareq

    2008-06-01

    Full Text Available Abstract Background Lennox-Gastaut syndrome (LGS is a severe form of childhood epilepsy that is defined by generalized multiple type seizures, slowness of intellectual growth, and a specific EEG disturbance. Children affected might previously have infantile spasms or underlying brain disorder but etiology can be idiopathic. In South Africa, the incidence of secondary epilepsy is higher than what is found in developed countries resulting in higher incidence of the disease. LGS seizures are often treatment resistant and the long term prognosis is poor. Report A twenty six year old female, presented with anterior open bite, macroglossia, supragingival as well as subgingival calculus. The gingiva was red, swollen and friable and there was generalized bleeding and localized suppuration. The patient had gingival recession. After periodontal therapy a remarkable improvement in oral health status was noted. Conclusion The clinical findings in LGS included facial deformities, periodontitis and gingival swellings. Interdisciplinary treatment of these patients is fundamental and oral attention is of outstanding importance. Non-surgical periodontal therapy was effective in controlling periodontal disease in the reported case, but prevention of periodontal and dental diseases is preferable for this high-risk group of patients.

  11. A pilot study of topiramate in children with Lennox-Gastaut syndrome Estudo piloto com topiramato em crianças com síndrome de Lennox-Gastaut

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    MARILISA M. GUERREIRO

    1999-06-01

    Full Text Available We conducted an open, add-on study with topiramate (TPM as adjunctive therapy in Lennox-Gastaut syndrome (LGS, to assess the long-term efficacy and safety and to evaluate quality of life (QL measurements in the chronic use of TPM. We studied 19 patients (11 male; age ranging from 4 to 14 years with uncontrolled seizures receiving 2-3 anti-epileptic drugs. Patients were followed up to 36 months of treatment. A questionnaire was used to query parents about QL. Seven patients completed the study at 36 months and seizure frequency was reduced > 75% in 4, and Realizamos estudo aberto, de adição do topiramato (TPM como medicação adjuvante no tratamento da síndrome de Lennox-Gastaut (SLG, com a finalidade de verificar a eficácia e a segurança de avaliar a qualidade de vida (QV no uso crônico do TPM. Estudamos 19 pacientes (11 do sexo masculino; idade entre 4 e 14 anos com epilepsia de difícil controle e em uso de 2 a 3 drogas anti-epilépticas. Os pacientes foram seguidos por 36 meses. Um questionário foi aplicado aos pais para se avaliar QV. Sete crianças completaram o estudo. A frequência de crises foi reduzida em mais de 75% em 4 e em menos de 50% em 3 delas. Dois pacientes ficaram sem crises. A maioria dos efeitos colaterais relacionou-se ao sistema nervoso central, sendo sonolência e anorexia os mais frequentes e transitórios. Um paciente foi excluído do estudo por apresentar pó na urina. Nenhuma criança necessitou de hospitalização. Aos 36 meses, os pacientes encontravam-se mais alertas (2/7, e houve melhora na interação com o meio ambiente (5/7, na habilidade em realizar tarefas diárias (5/7 e na performance verbal (6/7. Concluímos que o TPM pode ser útil como terapia adjuvante na SLG. A eficácia manteve-se no tratamento a longo prazo em mais de 40% dos pacientes, a segurança foi confirmada e a QV melhorou com o uso do TPM.

  12. Rufinamide in patients with Lennox-Gastaut syndrome

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    Clark PO

    2016-11-01

    Full Text Available Peggy O Clark,1 Patricia A Gibson2 1Cincinnati Children’s Hospital Medical Center, The University of Cincinnati College of Medicine, Cincinnati, OH, 2Epilepsy Information Service, Wake Forest University School of Medicine, Winston-Salem, NC, USA Objectives: Lennox–Gastaut syndrome (LGS is an epileptic encephalopathy with an onset at the age of ~4 years. LGS is notoriously difficult to manage, as most patients experience multiple seizures per day, despite their concomitant use of several antiepileptic drugs (AEDs. Rufinamide (BANZEL® is an AED approved by the US Food and Drug Administration for the adjunctive treatment of seizures associated with LGS in pediatric patients aged ≥1 year and in adults. The expert care of nurses knowledgeable in the treatment options for LGS is valuable to patients and caregivers. This review summarizes the existing knowledge on LGS and data from clinical and real-world studies on the use of rufinamide in patients with LGS.Methods: Recent review articles and information from the Epilepsy Foundation Website were reviewed for data on LGS treatment. Primary articles on rufinamide were also selected for review.Results and conclusion: The efficacy and safety of rufinamide have been evaluated in children and adults by using double-blind, open-label, and observational studies. In general, these studies indicate that rufinamide effectively reduces the frequency and severity of multiple seizure types associated with LGS and has tolerable side effects, the most common being vomiting and somnolence. Dosing modifications based on age, weight, and concomitant AED usage are recommended for patients using rufinamide. Keywords: Lennox–Gastaut syndrome, anticonvulsants, rufinamide

  13. Onset of action and seizure control in Lennox-Gaustaut syndrome: focus on rufinamide

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    Russell P Saneto

    2009-03-01

    Full Text Available Russell P Saneto1, Gail D Anderson21Division of Pediatric Neurology, Seattle Children’s Hospital/University of Washington, Seattle, Washington, USA; 2Department of Pharmacy, University of Washington, Seattle, Washington, USAAbstract: Lennox-Gaustaut syndrome is an electroclinical epilepsy syndrome characterized by the triad of electroencephalogram showing diffuse slow spike-and-wave discharges and paroxysmal fast activity, multiple intractable seizure types, and cognitive impairment. The intractability to seizure medications and cognitive impairment gives rise to eventual institutionalized patient care. Only a small subset of seizure medications has been shown to be helpful in seizure control. Most patients take up to 3 medications at high therapeutic dosing and are susceptible to medication-induced side effects. The lack of medication efficacy in seizure control has led one meta-analysis to conclude that there is no single medication that is highly efficacious in controlling seizures in this syndrome. On this background, a new and structurally novel seizure medication, rufinamide, has been found to be beneficial in the treatment of seizures in this syndrome. In a multicenter, double-blinded, randomized, placebo-controlled study, rufinamide was found to reduce seizures by over 30%. More importantly, it reduced the frequency of the seizure type that induces most of the morbidity of this syndrome, the drop seizure, by over 40%. There were few side effects, the medication was well tolerated, and in the open labeled extension study, tolerance was not found. In this review, we describe the main electroclinical features of Lennox-Gaustaut syndrome and summarize the few controlled studies that have contributed to its rational treatment. Currently, there is no single agent or combination of agents that effectively treat the multiple seizure types and co-morbidities in this syndrome. Our focus will be on the role of the new medication rufinamide in

  14. Treating Lennox–Gastaut syndrome in epileptic pediatric patients with third-generation rufinamide

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    Jessica Gresham

    2010-09-01

    Full Text Available Jessica Gresham1, Lea S Eiland2,3, Allison M Chung2,41Auburn University, Harrison School of Pharmacy (AUHSOP, 2Department of Pharmacy Practice, AUHSOP, 3University of Alabama, School of Medicine, Huntsville Regional Medical Campus, 4University of South Alabama School of Medicine, Department of Pediatrics, Mobile, Alabama, USAAbstract: Lennox–Gastaut syndrome (LGS is a rare but debilitating pediatric epileptic encephalopathy characterized by multiple intractable seizure types. Treatment of LGS is challenging because of the small number of antiepileptic drugs (AEDs which are effective for this syndrome, as well as the need for polytherapy in the majority of patients. This review focuses on the treatment of LGS with rufinamide, a recently approved third-generation AED with reported efficacy as adjunctive therapy for LGS. All relevant papers identified through a PubMed search on the treatment of LGS with rufinamide were reviewed. To date, the literature suggests improvements in seizure frequency for pediatric patients with LGS on rufinamide. Rufinamide appears to be especially effective for atonic or drop attack seizures. Rufinamide also displays a favorable adverse event profile compared with the older anticonvulsants, as well as a minimal number of drug interactions, making it a promising option for the adjunctive treatment of seizures associated with LGS.Keywords: epilepsy, Lennox-Gastaut syndrome, pediatrics, seizure, rufinamide

  15. Caracterización etiológica del síndrome de Lennox-Gastaut sintomático

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    Ileana Valdivia Álvarez

    2012-03-01

    Full Text Available Introducción: el síndrome de Lennox-Gastaut es una encefalopatía epiléptica dependiente de la edad, de gran severidad, por su farmacorresistencia y las discapacidades asociadas. Objetivos: caracterizar el síndrome de Lennox-Gastaut sintomático según sexo, edad de inicio y diagnóstico, tipo de crisis predominantes, hallazgos en neuroimagen y etiología. Métodos: estudio descriptivo, retrospectivo con una muestra de 36 pacientes egresados del servicio de neuropediatría del Hospital Pediátrico "Juan M. Márquez", con diagnóstico de síndrome de Lennox-Gastaut sintomático y que tuvieran estudios de neuroimagen (tomografía axial computarizada y resonancia magnética nuclear. Las variables cualitativas se describieron estadísticamente mediante frecuencias absolutas y cifras porcentuales. Para los porcentajes de interés, se calculó su intervalo de confianza con el 95 % de confiabilidad (IC 95 %. Resultados y conclusiones: el síndrome de Lennox-Gastaut sintomático fue más frecuente en varones, que comenzaron con epilepsia antes del año de edad, el 33 % presentó síndrome de West. Más del 60 % se diagnosticó antes de los 4 años de edad. Las crisis más frecuentes fueron las tónicas y atónicas de cuello. La tomografía axial computarizada permite localizar zonas de atrofia y pocas lesiones estructurales, que se precisan por resonancia magnética nuclear como alteraciones de la migración neuronal. Después de las malformaciones del sistema nervioso central, la causa más frecuente fue la hipoxia perinatal.

  16. Onset of action and seizure control in Lennox-Gaustaut syndrome: focus on rufinamide

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    Saneto, Russell P; Anderson, Gail D

    2009-01-01

    Russell P Saneto1, Gail D Anderson21Division of Pediatric Neurology, Seattle Children’s Hospital/University of Washington, Seattle, Washington, USA; 2Department of Pharmacy, University of Washington, Seattle, Washington, USAAbstract: Lennox-Gaustaut syndrome is an electroclinical epilepsy syndrome characterized by the triad of electroencephalogram showing diffuse slow spike-and-wave discharges and paroxysmal fast activity, multiple intractable seizure types, and cognitive impairment...

  17. Lennox-Gastaut syndrome: impact on the caregivers and families of patients.

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    Gibson, Patricia A

    2014-01-01

    Lennox-Gastaut syndrome (LGS) has a major impact on the health-related quality of life (HRQL) of the affected children as well as their caregivers. The primary caregiver in the family is generally the mother, with support from the father and siblings. The burden of care and the effects of the disease on the child necessitate adjustments in virtually all aspects of the lives of their family. These adjustments inevitably affect the physical, emotional, social, and financial health of the whole family. Numerous sources of support for families can help to ease the burden of care. Improvements in the treatment of LGS, in addition to helping the child with LGS, would likely help improve the HRQL of the family members. This pilot parent survey was designed to explore the impact of epilepsy on caregiver HRQL. Parents of children with epilepsy who had contacted the Epilepsy Information Service at the Wake Forest University School of Medicine, Winston-Salem, NC, USA, were sent questionnaires comprising open- and closed-ended questions. A total of 200 surveys were distributed, with a return rate of 48%. The results revealed that 74% of the parents believed that having a child with epilepsy brought them and their partner closer together. However, when the parents were asked to explain the manner in which epilepsy affected their families, answers included continuous stress, major financial distress, and lack of time to spend with other children. Information and resources for the families of children with LGS could help improve the HRQL of both the patients and their relatives.

  18. Sindrome de Lennox-Gastaut com inicio tardio: I - frequencia de casos com inicio anterior e ulterior aos 6 anos de idade, fatores etiologicos e nivel intelectual

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    Amilton Antunes Barreira

    1980-12-01

    Full Text Available De um grupo de 66 pacientes com sindrome de Lennox Gastaut, 15 apresentaram as primeiras manifestações clinicas após os 6 anos de idade. Desses, 12 foram acompanhados durante o período médio de 2 anos e 6 meses, com avaliações neurológicas a intervalos de cerca de 2 meses. A revisão das publicações pertinentes permitiu estabelecer que em 20% das casuisticas apreciadas a sindrome de Lennox-Gastaut se inicia após o 6° ano de vida. As avaliações clinicas permitiram verificar que nosos pacientes apresentaram incidência de lesões neurológicas graves precedendo a instalação da síndrome por vários anos. As avaliações psicométricas evidenciaram intenso comprometimento do nível intelectual em contraste com os dados da literatura sobre a síndrome de Lennox-Gastaut de início tardio.

  19. Gastaut type idiopathic childhood occipital epilepsy.

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    Ferrari-Marinho, Taissa; Macedo, Eugenia Fialho; Costa Neves, Rafael Scarpa; Costa, Lívia Vianez; Tudesco, Ivanda S S; Carvalho, Kelly C; Carrete, Henrique; Caboclo, Luis Otavio; Yacubian, Elza Marcia; Hamad, Ana Paula

    2013-03-01

    Gastaut type idiopathic childhood occipital epilepsy is an uncommon epileptic syndrome characterised by frequent seizures, most commonly presenting as elementary visual hallucinations or blindness. Other occipital (non-visual) symptoms may also occur. Interictal EEG typically shows occipital paroxysms, often with fixation-off sensitivity. Ictal EEG is usually characterised by interruption by paroxysms and sudden appearance of low-voltage, occipital, fast rhythm and/or spikes. Despite well described clinical and EEG patterns, to our knowledge, there are very few reports in the literature with video-EEG recording of either seizure semiology or fixation-off phenomena. We present a video-EEG recording of a 12-year-old girl with Gastaut type epilepsy, illustrating the interictal and ictal aspects of this syndrome. Our aim was to demonstrate the clinical and neurophysiological pattern of a typical seizure of Gastaut type epilepsy, as well as the fixation-off phenomena, in order to further clarify the typical presentation of this syndrome. [Published with video sequences].

  20. Felbamate

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    ... treat certain seizures in adults and children with epilepsy whose seizures have not improved with other treatments. ... seizures in children with Lennox-Gastaut syndrome (a disorder that causes seizures and developmental delays). Felbamate is in a class ...

  1. Brainstem dysfunction in patients with late-onset Lennox-Gastaut syndrome: Voxel-based morphometry and tract-based spatial statistics study

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    Kang Min Park

    2016-01-01

    Full Text Available Background: There have been a few reports of patients who developed Lennox-Gastaut syndrome (LGS in the second decades of their life. Objectives: The aim of this study was to investigate electroclinical presentation in patients with late-onset LGS. In addition, we evaluated structural abnormalities of the brain, which may give some clue about the common pathogenic pathway in LGS. Materials and Methods: We enrolled the patients with late-onset LGS. We collected electroclinical characteristics of the patients and evaluated structural abnormalities using voxel-based morphometry (VBM and tract-based spatial statistics (TBSS analysis. Results: The three subjects were diagnosed with late-onset LGS. The patients have no mental retardation and normal background activities on electroencephalography (EEG, and they had generalized paroxysmal fast activities on EEG, especially during sleep. The TBSS analysis revealed that fractional anisotropy values in the patients were significantly reduced in the white matter of brainstem compared with normal controls. However, VBM analysis did not show any significant difference between the patients and normal controls. Conclusions: Patients with late-onset LGS have different clinical and EEG characteristics from those with early-onset LGS. In addition, we demonstrated that brainstem dysfunction might contribute to the pathogenesis of late-onset LGS.

  2. Clobazam as an adjunctive therapy in treating seizures associated with Lennox–Gastaut syndrome

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    Leahy JT

    2011-11-01

    Full Text Available Jennifer T Leahy1, Catherine J Chu-Shore1,2, Janet L Fisher3 1Massachusetts General Hospital, Department of Neurology, Programs in Neurophysiology and Epilepsy, Boston, MA, 2Harvard Medical School, Boston, MA, 3University of South Carolina School of Medicine, Department of Pharmacology, Physiology and Neuroscience, Columbia, SC, USA Abstract: Lennox–Gastaut syndrome (LGS is a devastating childhood epilepsy syndrome characterized by the occurrence of multiple types of seizures and cognitive decline. Most children suffer from frequent seizures that are refractory to current medical management. Recent clinical trials have suggested that addition of clobazam may improve the clinical outcome for some LGS patients. Although clobazam has been available for over five decades, it has only recently been approved by the US Food and Drug Administration for this indication. As a 1,5-benzodiazepine, clobazam is structurally related to the widely used 1,4-benzodiazepines, which include diazepam. Clobazam has been shown to modulate GABAergic neurotransmission by positive allosteric modulation of GABAA receptors, and to increase expression of transporters for both GABA and glutamate. The active metabolite n-desmethylclobazam (norclobazam also modulates GABAA receptors, and the relative importance of these two compounds in the clinical effectiveness of clobazam remains an open question. Clinical trials involving clobazam as an addon therapy in a variety of pediatric epilepsy populations have found a significant improvement in seizure control. In patients with LGS, clobazam may have greatest efficacy for drop seizures. Longstanding clinical experience suggests that clobazam is a safe and well tolerated antiepileptic drug with infrequent and mild adverse effects. These results suggest that adjunctive treatment with clobazam may be a reasonable option for LGS patients, particularly those who are treatment-resistant. Keywords: benzodiazepine, epilepsy, gamma

  3. Thalamocortical functional connectivity in Lennox-Gastaut syndrome is abnormally enhanced in executive-control and default-mode networks.

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    Warren, Aaron E L; Abbott, David F; Jackson, Graeme D; Archer, John S

    2017-12-01

    To identify abnormal thalamocortical circuits in the severe epilepsy of Lennox-Gastaut syndrome (LGS) that may explain the shared electroclinical phenotype and provide potential treatment targets. Twenty patients with a diagnosis of LGS (mean age = 28.5 years) and 26 healthy controls (mean age = 27.6 years) were compared using task-free functional magnetic resonance imaging (MRI). The thalamus was parcellated according to functional connectivity with 10 cortical networks derived using group-level independent component analysis. For each cortical network, we assessed between-group differences in thalamic functional connectivity strength using nonparametric permutation-based tests. Anatomical locations were identified by quantifying spatial overlap with a histologically informed thalamic MRI atlas. In both groups, posterior thalamic regions showed functional connectivity with visual, auditory, and sensorimotor networks, whereas anterior, medial, and dorsal thalamic regions were connected with networks of distributed association cortex (including the default-mode, anterior-salience, and executive-control networks). Four cortical networks (left and right executive-control network; ventral and dorsal default-mode network) showed significantly enhanced thalamic functional connectivity strength in patients relative to controls. Abnormal connectivity was maximal in mediodorsal and ventrolateral thalamic nuclei. Specific thalamocortical circuits are affected in LGS. Functional connectivity is abnormally enhanced between the mediodorsal and ventrolateral thalamus and the default-mode and executive-control networks, thalamocortical circuits that normally support diverse cognitive processes. In contrast, thalamic regions connecting with primary and sensory cortical networks appear to be less affected. Our previous neuroimaging studies show that epileptic activity in LGS is expressed via the default-mode and executive-control networks. Results of the present study suggest that

  4. CLINICAL AND ENCEPHALOGRAPHIC CHANGES AT LENNOX–GASTAUT SYNDROME

    Directory of Open Access Journals (Sweden)

    K. Yu. Mukhin

    2015-01-01

    Full Text Available The Lennox–Gastaut syndrome (LGS is an epileptic encephalopathy, starting in childhood and showing in often polymorphic seizures (including tonic axial ones, severe cognitive deficite, slow activity of the acute–slow wave in the interictal period at the electroencephalography (EEG, runs of fast activity of 10–20 Hz, often associated with tonic seizures, as well as with the resistance to therapy. According ILAE Classification of epilepsy syndromes and epilepsies 1989 LGS was referred to generalized cryptogenic or symptomatic forms of the epilepsy. According to Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy (2001 LGS is a classic representative of the group of childhood epileptic encephalopathies. LGS is a rather rare form of the epilepsy. The syndrome frequency makes from 1–4 to 6.6 % among all forms of the childhood epilepsy. LGS is subdivided into the cryptogenic and the symptomatic variants. From our point of view the latter it will be more correct to refer to the symptomatic focal epilepsy with the secondary bilateral synchrony phenomena at EEG. The LGS can be caused by cortical development defects, by perinatal encephalopathies, by brain tumors, by inherited metabolism diseases, by chromosomal anomalies, as well as by other factors. In case of the classic cryptogenic variant the ethiology of the LGS remains unknown. The disease onset is at the age of 2–8 y. o. In 20–40 % of cases LGS is transformed from the West syndrome. The LGS attribute is the polymorphism of seizures. The syndrome structure can combine tonic seizures, epileptic drop seizures, atypical absences, generalized tonic-clonic seizures. Focal seizures at LGS are a matter of argument. The article gives details on the clinical EEG criteria of LGS, the semiology of epileptic seizures in the syndrome structure, diagnostic and treatment approaches. The main accent is made on EEG peculiarities of the disease. The author presents the

  5. PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS

    Directory of Open Access Journals (Sweden)

    K. Yu. Mukhin

    2016-01-01

    Full Text Available Pseudo-Lennox syndrome (PLS, or atypical benign partial epilepsy of childhood, is a disease from a group of age-related epileptic encephalopathies with a phenomenon of continuous spike-wave activity during slow sleep, which manifests itself as frequent polymorphic focal motor and pseudogeneralized seizures, cognitive impairments, as well as regional and diffuse epileptiform activity on electroencephalogram (EEG by the morphology identical to that of benign epileptiform patterns of childhood. The disease was first described by J. Aicardi and J. J. Chevrie in 1982, based on a study of 7 cases. Its diagnostic complexity is the polymorphism of both epileptic seizures and EEG data, as well as low awareness of the syndrome among physicians and its absence in the international classification of epilepsies. The typical triad of seizures, which occurs in nearly 100 % of patients, encompasses focal motor paroxysms (identical to those as observed in Rolandic epilepsy, atypical absences, and atonic seizures. Seizures in PLS in its active period (generally up to 7–8 years are highly resistant to antiepileptic drugs. Only a few agents have been proven to be effective in PLS; these include valproates, succinimides, benzodiazepines, topiramate, and sulthiame. The frequency of seizures are noted to increase in patients with PLS treated with drugs, such as vigabatrin, gabapentin, lamotrigine, phenobarbital, or phenytoin. The author considers in detail the history of studies of the disease, clinical manifestations, diagnostic criteria, therapeutic approaches, and prognosis.

  6. Ketogenic Diet in Epileptic Encephalopathies

    OpenAIRE

    Sharma, Suvasini; Tripathi, Manjari

    2013-01-01

    The ketogenic diet is a medically supervised high-fat, low-carbohydrate diet that has been found useful in patients with refractory epilepsy. It has been shown to be effective in treating multiple seizure types and epilepsy syndromes. In this paper, we review the use of the ketogenic diet in epileptic encephalopathies such as Ohtahara syndrome, West syndrome, Dravet syndrome, epilepsy with myoclonic atonic seizures, and Lennox-Gastaut syndrome.

  7. Application of rare variant transmission disequilibrium tests to epileptic encephalopathy trio sequence data

    NARCIS (Netherlands)

    Allen, Andrew S.; Berkovic, Samuel F.; Bridgers, Joshua; Cossette, Patrick; Dlugos, Dennis; Epstein, Michael P.; Glauser, Tracy; Goldstein, David B.; Heinzen, Erin L.; Jiang, Yu; Johnson, Michael R.; Kuzniecky, Ruben; Lowenstein, Daniel H.; Marson, Anthony G.; Mefford, Heather C.; O'Brien, Terence J.; Ottman, Ruth; Petrou, Steven; Petrovski, Slavé; Poduri, Annapurna; Ren, Zhong; Scheffer, Ingrid E.; Sherr, Elliott; Wang, Quanli; Balling, Rudi; Barisic, Nina; Baulac, Stéphanie; Caglayan, Hande; Craiu, Dana; De Jonghe, Peter; Depienne, Christel; Guerrini, Renzo; Helbig, Ingo; Hjalgrim, Helle; Hoffman-Zacharska, Dorota; Jähn, Johanna A.; Klein, Karl Martin; Koeleman, Bobby; Komarek, Vladimir; Krause, Roland; Leguern, Eric; Lehesjoki, Anna-Elina; Lemke, Johannes R.; Lerche, Holger; Linnankivi, Tarja; Marini, Carla; May, Patrick; Møller, Rikke S.; Muhle, Hiltrud; Pal, Deb; Palotie, Aarno; Rosenow, Felix; Selmer, Kaja; Serratosa, Jose M.; Sisodiya, Sanjay M.; Stephani, Ulrich; Sterbova, Katalin; Striano, Pasquale; Suls, Arvid; Talvik, Tiina; von Spiczak, Sarah; Weber, Yvonne G.; Weckhuysen, Sarah; Zara, Federico; Abou-Khalil, Bassel; Alldredge, Brian K.; Amrom, Dina; Andermann, Eva; Andermann, Frederick; Bautista, Jocelyn F.; Bluvstein, Judith; Cascino, Gregory D.; Consalvo, Damian; Crumrine, Patricia; Devinsky, Orrin; Fiol, Miguel E.; Fountain, Nathan B.; French, Jacqueline; Friedman, Daniel; Haas, Kevin; Haut, Sheryl R.; Hayward, Jean; Joshi, Sucheta; Kanner, Andres; Kirsch, Heidi E.; Kossoff, Eric H.; Kuperman, Rachel; McGuire, Shannon M.; Motika, Paul V.; Novotny, Edward J.; Paolicchi, Juliann M.; Parent, Jack; Park, Kristen; Shellhaas, Renée A; Sirven, Joseph; Smith, Michael C.; Sullivan, Joseph; Thio, Liu Lin; Venkat, Anu; Vining, Eileen P. G.; Von Allmen, Gretchen K.; Weisenberg, Judith L.; Widdess-Walsh, Peter; Winawer, Melodie R.

    2017-01-01

    The classic epileptic encephalopathies, including infantile spasms (IS) and Lennox-Gastaut syndrome (LGS), are severe seizure disorders that usually arise sporadically. De novo variants in genes mainly encoding ion channel and synaptic proteins have been found to account for over 15% of patients

  8. Efeito de medicamentos sobre tipos eletroclínicos de crises epilépticas na síndrome de Lennox-Gastaut

    Directory of Open Access Journals (Sweden)

    José Geraldo Speciali

    1977-09-01

    Full Text Available Analisamos os resultados terapêuticos obtidos com o uso de diferentes drogas (diazepam, nitrazepam, clonazepam, difenilhidantoina, barbitúricos - fenobarbital e primidona -, dipropilacetato de sódio e ACTH em 29 pacientes com síndrome de Lennox-Gastaut. Discutimos a ação de cada droga sobre as crises epilépticas (tônicas, tônico-clônicas, clônicas, mioclônicas ou mioclonoatônicas, atônicas e ausências atípicas durante o primeiro mês de cada esquema terapêutico. Delimitamos os campos de ação específica de cada medicação e verificamos a possibilidade de reduzir a incidência de crises epilépticas retirando ou diminuindo as dosagens das drogas administradas.

  9. Disease: H00577 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available syndrome (H01460), Lennox-Gastaut syndrome (H01813), Dravet syndrome (H01818), Epilepsy with myoclonic-astat...ileptic encephalopathy (H00606), and other symptomatic generalized epilepsies. Congenital disorder; Epilepsy... ... Other symptomatic generalized epilepsies include Epilepsy, hearing loss, an...and Hypomagnesemia, seizures, and mental retardation (HOMGSMR). The International League Against Epilepsy (I...tional League Against ... Epilepsy. ... JOURNAL ... Epilepsia 30:389-99 (1989) DOI:10.1111/j.1528-1157.1989.tb05316

  10. Síndrome de lennox-gastaut com início na vida adulta? a respeito de um caso

    Directory of Open Access Journals (Sweden)

    P. C. Trevisol-Bittencourt

    1990-12-01

    Full Text Available Apesar de ter sido relatada anteriormente, a sindrome de Lennox-Gastaut (SLG só foi definitivamente aceita como entidade distinta em 1966. Desde então, o epônimo tem sido utilizado para designar encefalopatia severa da infância, caracterizada fundamentalmente por crises epilépticas refratárias de diversos tipos associadas com alterações eletrográficas típicas. Entretanto, vários autores consideram que o rótulo de SLG está abrigando quadros nosológicos distintos, que deveriam ser separados e individualizados. Ilustrando esta situação, o caso de um paciente que iniciou quadro de «SLG-like» aos 20 anos de idade é relatado. Adicionando mais confusão, alguns casos semelhantes têm sido descritos nos últimos anos sob variada nomenclatura. Desde que um dos principais critérios para o diagnóstico da SLG é a idade dependência, como deverão então ser chamados tais quadros? Revisão dos critérios empregados para o diagnóstico da SLG se faz necessária.

  11. Diffuse alveolar hemorrhage due to valproic acid: Case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Francesco Inzirillo

    2015-01-01

    Full Text Available Valproic acid (VPA is one of the most frequently used antiepileptic drugs for the treatment of focal and generalized epilepsies, absence seizures, and Lennox-Gastaut syndrome (LGS. VPA has been demonstrated to have a negative effect on both the intrinsic and extrinsic coagulation systems and controversy exists about the clinical relevance of such hematological abnormalities. We describe a case of reversible lung hemorrage due to VPA. In English-language literature only two other similar cases (one of which fatal have been described so far.

  12. The molecular and phenotypic spectrum of IQSEC2-related epilepsy.

    Science.gov (United States)

    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

  13. Deletions in 16p13 including GRIN2A in patients with intellectual disability, various dysmorphic features, and seizure disorders of the rolandic region.

    NARCIS (Netherlands)

    Reutlinger, C.; Helbig, I.; Gawelczyk, B.; Subero, J.I.; Tonnies, H.; Muhle, H.; Finsterwalder, K.; Vermeer, S.; Pfundt, R.; Sperner, J.; Stefanova, I.; Gillessen-Kaesbach, G.; Spiczak, S. von; Baalen, A. van; Boor, R.; Siebert, R.; Stephani, U.; Caliebe, A.

    2010-01-01

    Seizure disorders of the rolandic region comprise a spectrum of different epilepsy syndromes ranging from benign rolandic epilepsy to more severe seizure disorders including atypical benign partial epilepsy/pseudo-Lennox syndrome,electrical status epilepticus during sleep, and Landau-Kleffner

  14. Brain perfusion SPECT in children with frequent fits

    International Nuclear Information System (INIS)

    Heiskala, H.; Launes, J.; Pihko, H.; Nikkinen, P.; Santavuori, P.

    1993-01-01

    We studied 14 children with frequent fits using 99m Tc-HM-PAO single photon emission computed tomography (SPECT). There were 11 patients with partial secondary generalized epilepsy (PSGE) and 3 with Lennox-Gastaut syndrome (LGS). The typical regional cerebral blood flow (rCBF) finding in PSGE was a single area of abnormally low perfused cortex, and that in LGS, multiple hypoperfused areas. Clinically, the LGS patients were more severely affected. SPECT was more sensitive in detecting abnormalities than EEG, CT or MRI. Extensive impairment of rCBF may thus indicate unfavourable development of intellectual performance and poor seizure control. (author)

  15. Thirty Years of Orphan Drug Legislation and the Development of Drugs to Treat Rare Seizure Conditions: A Cross Sectional Analysis.

    Science.gov (United States)

    Döring, Jan Henje; Lampert, Anette; Hoffmann, Georg F; Ries, Markus

    2016-01-01

    Epilepsy is a serious chronic health condition with a high morbidity impairing the life of patients and afflicted families. Many epileptic conditions, especially those affecting children, are rare disorders generating an urgent medical need for more efficacious therapy options. Therefore, we assessed the output of the US and European orphan drug legislations. Quantitative analysis of the FDA and EMA databases for orphan drug designations according to STrengthening the Reporting of OBservational studies in Epidemiology (STROBE) criteria. Within the US Orphan Drug Act 40 designations were granted delivering nine approvals, i.e. clobazam, diazepam viscous solution for rectal administration, felbamate, fosphenytoin, lamotrigine, repository corticotropin, rufinamide, topiramate, and vigabatrin. Since 2000 the EMA granted six orphan drug designations whereof two compounds were approved, i.e. rufinamide and stiripentol. In the US, two orphan drug designations were withdrawn. Orphan drugs were approved for conditions including Lennox-Gastaut syndrome, infantile spasms, Dravet syndrome, and status epilepticus. Comparing time to approval for rufinamide, which was approved in the US and the EU to treat rare seizure conditions, the process seems faster in the EU (2.2 years) than in the US (4.3 years). Orphan drug development in the US and in the EU delivered only few molecular entities to treat rare seizure disorders. The development programs focused on already approved antiepileptic drugs or alternative pharmaceutical formulations. Most orphan drugs approved in the US are not approved in the EU to treat rare seizures although some were introduced after 2000 when the EU adopted the Orphan Drug Regulation.

  16. Epilepsy with myoclonic absences - favourable response to add-on rufinamide treatment in 3 cases

    DEFF Research Database (Denmark)

    Häusler, M; Kluger, G; Nikanorova, M

    2011-01-01

    Epilepsy with myoclonic absences (EMA) is a rare epileptic syndrome with frequently poor response to antiepileptic treatment. Rufinamide (RUF) is a relatively new EMEA- and FDA-approved anticonvulsant licensed as an orphan drug for the adjunctive treatment of patients with Lennox-Gastaut syndrome....

  17. Estados de mal epiléptico na sindrome Delennox-Gastaut: aspectos terapeuticos

    Directory of Open Access Journals (Sweden)

    José Geraldo Speciali

    1977-09-01

    Full Text Available Foram descritas as condutas terapêuticas utilizadas nos estados de mal epiléptico, convulsivos e não convulsivos, observados em 14 pacientes com síndrome de Lennox-Gastaut. Verificou-se relação temporal entre aparecimento de estado de mal epiléptico e início de tratamento com clonazepam e difenilhidantoina; a retirada dessas medicações foi seguida pelo desaparecimento dos estados de mal. Indicou-se a possibilidade de tratamento dos estados de mal epiléptico modificando-se o esquema terapêutico administrado por via oral, reservando-se o tratamento parenteral para os estados com comprometimento progressivo da consciência e/ou dos distúrbios neurovegetativos.

  18. Stimulation of the nervous system for the management of seizures: current and future developments.

    Science.gov (United States)

    Murphy, Jerome V; Patil, Arunangelo

    2003-01-01

    Vagal nerve stimulation (VNS) for the treatment of refractory epilepsy appears to have started from the theory that since VNS can alter the EEG, it may influence epilepsy. It proved effective in several models of epilepsy and was then tried in short-term, open-label and double-blind trials, leading to approval in Canada, Europe and the US. Follow-up observations in these patients demonstrated continued improvement in seizure control for up to 2 years. Close to 50% of treated patients have achieved at least a 50% reduction in seizure frequency. This therapy was also useful as rescue therapy for ongoing seizures in some patients; many patients are more alert. The initial trials were completed in patients >/=12 years of age with refractory partial seizures. Subsequently, similar benefits were shown in patients with tuberous sclerosis complex, Lennox-Gastaut syndrome, hypothalamic hamartomas and primary generalised seizures. Implanting the generator and leads is technically easy, and complications are few. The method of action is largely unknown, although VNS appears to alter metabolic activity in specific brain nuclei. Considering that improvement in mood is frequently found in patients using VNS, it has undergone trials in patients with depression. Other illnesses deserving exploration with this unusual therapy are Alzheimer's disease and autism. Some aspects of VNS have proven disappointing. Although patients have fewer seizures, the number of antiepileptic drugs they take is not significantly reduced. In addition, there is no way to accurately predict the end of life of the generator. Optimal stimulation parameters, if they exist, are unknown. Deep brain stimulation is a new method for controlling medically refractory seizures. It is based on the observation that thalamic stimulation can influence the EEG over a wide area. Several thalamic nuclei have been the object of stimulation in different groups of patients. Intraoperative brain imaging is essential for

  19. Respiratory arrest at the onset of idiopathic childhood occipital epilepsy of Gastaut.

    Science.gov (United States)

    Funata, Keiko; Shike, Tatsuhiko; Takenouchi, Toshiki; Yamashita, Yukio; Takahashi, Takao

    2018-01-01

    Occipital lobe epilepsy of childhood includes two entities: Panayiotopoulos syndrome in pre-school children, and idiopathic childhood occipital epilepsy of Gastaut (ICOEG) in school-age children. The typical initial manifestation of the former is vomiting, and that of the latter is visual hallucinations. Ictal cardiopulmonary arrest at initial presentation has been reported for Panayiotopoulos syndrome, but not for ICOEG. We document a 7-year-old previously healthy girl who experienced an acute elemental visual hallucination of seeing insects, followed by a new-onset generalized seizure. Upon arrival at the local hospital, she was unconscious and soon thereafter, developed respiratory arrest. She was resuscitated and initiated on mechanical ventilation. An electroencephalogram taken three days after seizure cessation showed frequent occipital spikes, consistent with the diagnosis of ICOEG. The sequence of acute elementary visual hallucination followed by a motor seizure, and then witnessed respiratory arrest illustrated occurrence of life-threatening autonomic involvement at initial onset in ICOEG. We speculate that the epileptic propagation from the occipital lobes eventually compromised the respiratory center in the brainstem. The possibility of occipital lobe epilepsy should be considered in school-age children presenting with acute visual hallucination followed by respiratory arrest. Such a presentation should prompt an urgent electroencephalogram and initiation of antiepileptic treatment if indicated. Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  20. [Electroencephalography and epileptology in the 20th century].

    Science.gov (United States)

    Karbowski, K

    1995-12-05

    In 1875, Caton was already able to detect cerebral electric currents during experimental studies in animals. In 1914, Cybulski and Jeleńska-Macieszyna reported on the increase of current-intensity during a focal motor epileptic seizure. In 1929 in Jena, Berger revolutionized the study of epilepsy with his paper on the human electroencephalogram 'Uber das Elektrenkephalogramm des Menschen'. His discovery and further publications as well as later works of numerous researchers, especially F. and E. Gibbs, Lennox, Penfield and Jasper, made it possible to distinguish different forms of 'little' epileptic seizures and to separate them from nonepileptic paroxysmal disorders. New epileptic syndromes could be singled out, as e.g. the symptomatic epilepsy of childhood with variable clinical manifestations of seizures and slow spike-wave complexes in the EEG (Lennox-Gastaut syndrome) or the benign partial epilepsy of childhood with centrotemporal EEG spikes. In these fields, as well as for the epileptic seizures in newborns and babies and for the differentiation between epileptic and nonepileptic twilight states in later stages of life, the EEG remains an indispensable tool in the CT and MRI era. It also contributes largely to the diagnosis of nonepileptic cerebral illness such as herpes simplex encephalitis, subacute sclerosing panencephalitis van Bogaert and Creutzfeldt-Jakob disease. Since the introduction of phenobarbital by Hauptmann in 1912, the palet of effective drugs against epilepsy, such as phenytoin, carbamazepine, valproate and benzodiazepines used for status-epilepticus treatment, became essentially larger. The value of newer substances (vigabatrin, progabide, gabapentin, lamotrigin) can't be estimated actually.(ABSTRACT TRUNCATED AT 250 WORDS)

  1. Benign occipital lobe seizures: Natural progression and atypical evolution

    Directory of Open Access Journals (Sweden)

    Prithika Chary

    2013-01-01

    Full Text Available Benign occipital seizure syndromes are benign childhood epilepsy syndromes and are mainly of two types, Panayiotopoulos syndrome, an autonomic epilepsy and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G including the idiopathic photosensitive occipital lobe epilepsy. Although both these types are categorized as occipital seizures, they are distinct in presentation and management. They can also be tricky to diagnose as visual symptoms may not always be the presenting feature and it is also not very easy to elicit visual hallucinations during history taking. These seizures have a good response to treatment; however, there could be atypical evolution and refractoriness to treatment especially with ICOE-G. We describe three children who presented with visual and non-visual symptoms and the electroencephalography (EEG in all the three cases showed occipital paroxysms. We have emphasized the clues in the clinical history and EEG leading to the diagnosis of these distinct epilepsy syndromes. We have also discussed the natural course of these epilepsy syndromes with some atypical evolution, which clinicians need to be aware of during treatment of these children.

  2. Benign occipital lobe seizures: Natural progression and atypical evolution.

    Science.gov (United States)

    Chary, Prithika; Rajendran, Bhuvaneshwari

    2013-10-01

    Benign occipital seizure syndromes are benign childhood epilepsy syndromes and are mainly of two types, Panayiotopoulos syndrome, an autonomic epilepsy and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) including the idiopathic photosensitive occipital lobe epilepsy. Although both these types are categorized as occipital seizures, they are distinct in presentation and management. They can also be tricky to diagnose as visual symptoms may not always be the presenting feature and it is also not very easy to elicit visual hallucinations during history taking. These seizures have a good response to treatment; however, there could be atypical evolution and refractoriness to treatment especially with ICOE-G. We describe three children who presented with visual and non-visual symptoms and the electroencephalography (EEG) in all the three cases showed occipital paroxysms. We have emphasized the clues in the clinical history and EEG leading to the diagnosis of these distinct epilepsy syndromes. We have also discussed the natural course of these epilepsy syndromes with some atypical evolution, which clinicians need to be aware of during treatment of these children.

  3. Expert Opinion on the Management of Lennox–Gastaut Syndrome: Treatment Algorithms and Practical Considerations

    Directory of Open Access Journals (Sweden)

    J. Helen Cross

    2017-09-01

    Full Text Available Lennox–Gastaut syndrome (LGS is a severe epileptic and developmental encephalopathy that is associated with a high rate of morbidity and mortality. It is characterized by multiple seizure types, abnormal electroencephalographic features, and intellectual disability. Although intellectual disability and associated behavioral problems are characteristic of LGS, they are not necessarily present at its outset and are therefore not part of its diagnostic criteria. LGS is typically treated with a variety of pharmacological and non-pharmacological therapies, often in combination. Management and treatment decisions can be challenging, due to the multiple seizure types and comorbidities associated with the condition. A panel of five epileptologists met to discuss consensus recommendations for LGS management, based on the latest available evidence from literature review and clinical experience. Treatment algorithms were formulated. Current evidence favors the continued use of sodium valproate (VPA as the first-line treatment for patients with newly diagnosed de novo LGS. If VPA is ineffective alone, evidence supports lamotrigine, or subsequently rufinamide, as adjunctive therapy. If seizure control remains inadequate, the choice of next adjunctive antiepileptic drug (AED should be discussed with the patient/parent/caregiver/clinical team, as current evidence is limited. Non-pharmacological therapies, including resective surgery, the ketogenic diet, vagus nerve stimulation, and callosotomy, should be considered for use alongside AED therapy from the outset of treatment. For patients with LGS that has evolved from another type of epilepsy who are already being treated with an AED other than VPA, VPA therapy should be considered if not trialed previously. Thereafter, the approach for a de novo patient should be followed. Where possible, no more than two AEDs should be used concomitantly. Patients with established LGS should undergo review by a neurologist

  4. Application of rare variant transmission disequilibrium tests to epileptic encephalopathy trio sequence data

    DEFF Research Database (Denmark)

    2017-01-01

    The classic epileptic encephalopathies, including infantile spasms (IS) and Lennox-Gastaut syndrome (LGS), are severe seizure disorders that usually arise sporadically. De novo variants in genes mainly encoding ion channel and synaptic proteins have been found to account for over 15% of patients...... with IS or LGS. The contribution of autosomal recessive genetic variation, however, is less well understood. We implemented a rare variant transmission disequilibrium test (TDT) to search for autosomal recessive epileptic encephalopathy genes in a cohort of 320 outbred patient-parent trios that were generally...... not find evidence of a role for individual autosomal recessive genes, our current sample is insufficiently powered to assess the overall role of autosomal recessive genotypes in an outbred epileptic encephalopathy population....

  5. Neuropharmacological imaging in epilepsy with PET and SPECT

    International Nuclear Information System (INIS)

    Henry, T.R.; Pennell, P.B.

    1998-01-01

    Functional neuroimaging with positron and single photon emitter-labeling has added considerably to the understanding of epileptic seizure activity and of the postictal and interictal cerebral dysfunctions that accompany many epilepsies. Some of these functional alterations cannot be studied in humans by any other technique, and in other instances the information is complementary to that provided by other techniques, some of which are invasive or even require tissue destruction. Available radiotracer imaging techniques have yet to be fully applied to several important epileptic syndromes (including the Lennox-Gastaut syndrome and other secondary generalized epilepsies), to physiological aspects of the natural history of temporal lobe epilepsy or any other commonly occurring epilepsy, and to the assessment of mechanism of action and adverse effects of antiepileptic drugs and other epilepsy therapies. New radiotracers should be developed to permit study of specific excitatory amino acid receptors and other receptor sites that are known to be relevant to the development of epilepsy, to the onset of individual seizures, and to interical dysfunctions

  6. A seizuring alagille syndrome

    Directory of Open Access Journals (Sweden)

    Jomon Mathew John

    2017-01-01

    Full Text Available Alagille syndrome is a rare autosomal dominant inherited disorder with incidence of one in 100,000 live births. This syndrome with seizure as a presentation has been rarely reported in Indian studies. We present a 3-month-old infant who presented to us with seizures was found to have a dysmorphic face, jaundice, hepatomegaly, and soft systolic murmur. Infant was stabilized and remained seizure free. A detailed clinical evaluation of a common presentation may reveal a rare syndrome.

  7. Clinical evaluation and MRI findings in early infantile epileptic encephalopathy with suppression-burst

    Energy Technology Data Exchange (ETDEWEB)

    Konishi, Hideki; Kodama, Soichi; Momota, Keiko and others

    1988-02-01

    The clinical courses and a follow-up study on the MRI findings in four cases with early infantile epileptic encephalopathy (EIEE) are reported. The patients consisted of one male and three females. The age at onset was before 15 days on life and the etiology was unknown in all cases. EEG improvement and a decrease in seizure frequency were seen after treatment with ACTH and anticonvulsants in three of the four patients, while no treatment was effective in the other patient who developed Lennox-Gastaut syndrome through West syndrome. Psychomotor development of all patients was severely retarded, and it was impossible for three cases to gain head control until 12 months old. MRI findings revealed dysmyelination of white matter in the cerebrum in three patients and asymmetrical myelination in the other patient. These results suggested that EIEE is based on brain immaturity combined with dysmyelination.

  8. Clinical evaluation and MRI findings in early infantile epileptic encephalopathy with suppression-burst

    International Nuclear Information System (INIS)

    Konishi, Hideki; Kodama, Soichi; Momota, Keiko

    1988-01-01

    The clinical courses and a follow-up study on the MRI findings in four cases with early infantile epileptic encephalopathy (EIEE) are reported. The patients consisted of one male and three females. The age at onset was before 15 days on life and the etiology was unknown in all cases. EEG improvement and a decrease in seizure frequency were seen after treatment with ACTH and anticonvulsants in three of the four patients, while no treatment was effective in the other patient who developed Lennox-Gastaut syndrome through West syndrome. Psychomotor development of all patients was severely retarded, and it was impossible for three cases to gain head control until 12 months old. MRI findings revealed dysmyelination of white matter in the cerebrum in three patients and asymmetrical myelination in the other patient. These results suggested that EIEE is based on brain immaturity combined with dysmyelination. (author)

  9. Report of a parent survey of cannabidiol-enriched cannabis use in pediatric treatment-resistant epilepsy.

    Science.gov (United States)

    Porter, Brenda E; Jacobson, Catherine

    2013-12-01

    Severe childhood epilepsies are characterized by frequent seizures, neurodevelopmental delays, and impaired quality of life. In these treatment-resistant epilepsies, families often seek alternative treatments. This survey explored the use of cannabidiol-enriched cannabis in children with treatment-resistant epilepsy. The survey was presented to parents belonging to a Facebook group dedicated to sharing information about the use of cannabidiol-enriched cannabis to treat their child's seizures. Nineteen responses met the following inclusion criteria for the study: a diagnosis of epilepsy and current use of cannabidiol-enriched cannabis. Thirteen children had Dravet syndrome, four had Doose syndrome, and one each had Lennox-Gastaut syndrome and idiopathic epilepsy. The average number of antiepileptic drugs (AEDs) tried before using cannabidiol-enriched cannabis was 12. Sixteen (84%) of the 19 parents reported a reduction in their child's seizure frequency while taking cannabidiol-enriched cannabis. Of these, two (11%) reported complete seizure freedom, eight (42%) reported a greater than 80% reduction in seizure frequency, and six (32%) reported a 25-60% seizure reduction. Other beneficial effects included increased alertness, better mood, and improved sleep. Side effects included drowsiness and fatigue. Our survey shows that parents are using cannabidiol-enriched cannabis as a treatment for their children with treatment-resistant epilepsy. Because of the increasing number of states that allow access to medical cannabis, its use will likely be a growing concern for the epilepsy community. Safety and tolerability data for cannabidiol-enriched cannabis use among children are not available. Objective measurements of a standardized preparation of pure cannabidiol are needed to determine whether it is safe, well tolerated, and efficacious at controlling seizures in this pediatric population with difficult-to-treat seizures. © 2013.

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

    Science.gov (United States)

    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.

  11. Report from a Survey of Parents Regarding the Use of Cannabidiol (Medicinal cannabis in Mexican Children with Refractory Epilepsy

    Directory of Open Access Journals (Sweden)

    Carlos G. Aguirre-Velázquez

    2017-01-01

    Full Text Available Structured online surveys were used to explore the experiences of the parents of children with refractory epilepsy using medicinal cannabis in Mexico during September 2016. The surveys, which were completed in full, were reviewed, and 53 cases of children aged between 9 months and 18 years were identified. Of these, 43 cases (82% were from Mexico and 10 (18% were from Latin American countries. Of the 43 Mexican cases, the diagnoses were as follows: 20 cases (47% had Lennox-Gastaut syndrome (LGS; 13 cases (30% had unspecified refractory epilepsy (URE; 8 cases (19% had West syndrome (WS; 1 case (2% had Doose syndrome (DS; and 1 case (2% had Ohtahara syndrome (OS. In total, 47.1% of cases had previously been treated with 9 or more anticonvulsant therapies. The parents reported a decrease in convulsions when cannabidiol was used in 81.3% of the cases; a moderate to significant decrease occurred in 51% of cases, and 16% of cases were free from seizure. The number of antiepileptic drugs being used was reduced in 9/43 (20.9% cases. No serious adverse effects were reported, with only some mild adverse effects, such as increased appetite or changes in sleep patterns, reported in 42% of cases.

  12. Localization of epileptogenic zones in Lennox–Gastaut syndrome using frequency domain source imaging of intracranial electroencephalography: a preliminary investigation

    International Nuclear Information System (INIS)

    Cho, Jae-Hyun; Jung, Young-Jin; Kim, Jeong-Youn; Im, Chang-Hwan; Kang, Hoon-Chul; Kim, Heung Dong; Yoon, Dae Sung; Lee, Yong-Ho

    2013-01-01

    Although intracranial electroencephalography (iEEG) has been widely used to localize epileptogenic zones in epilepsy, visual inspection of iEEG recordings does not always result in a favorable surgical outcome, especially in secondary generalized epilepsy such as Lennox–Gastaut syndrome (LGS). Various computational iEEG analysis methods have recently been introduced to confirm the visual inspection results. Of these methods, high gamma oscillation in iEEG has attracted interest because a series of studies have reported a close relationship between epileptogenic zones and cortical areas with high gamma oscillation. Meanwhile, frequency domain source imaging of EEG and MEG oscillations has proven to be a useful auxiliary tool for identifying rough locations of epileptogenic zones. To the best of our knowledge, however, frequency domain source imaging of high gamma iEEG oscillations has not been studied. In this study, we investigated whether the iEEG-based frequency domain source imaging of high gamma oscillation (60–100 Hz) would be a useful supplementary tool for identifying epileptogenic zones in patients with secondary generalized epilepsy. The method was applied to three successfully operated on LGS patients, whose iEEG contained some ictal events with distinct high gamma oscillations before seizure onset. The resultant cortical source distributions were compared with surgical resection areas and with high gamma spectral power distributions on the intracranial sensor plane. While the results of the sensor-level analyses contained many spurious activities, the results of frequency domain source imaging coincided better with the surgical resection areas, suggesting that the frequency domain source imaging of iEEG high gamma oscillations might help enhance the accuracy of pre-surgical evaluations of patients with secondary generalized epilepsy. (paper)

  13. Cerebral glucose utilization in pediatric neurological disorders determined by positron emission tomography

    International Nuclear Information System (INIS)

    Yanai, Kazuhiko; Tohoku Univ., Sendai; Iinuma, Kazuie; Miyabayashi, Shigeaki; Narisawa, Kuniaki; Tada, Keiya; Matsuzawa, Taiju; Tohoku Univ., Sendai; Ito, Masatoshi; Yamada, Kenji

    1987-01-01

    We measured local cerebral glucose utilization in 19 patients with Lennox-Gastaut syndrome (LG), partial seizures (PS), atypical and classical phenylketonuria (PKU), Leigh disease, and subacute sclerosing panencephalitis (SSPE), using positron emission tomography (PET). The mean values of regional glucose utilization in interictal scans of LG were significantly reduced in all brain regions when compared with that of PS (P<0.005). PET studies of glucose utilization in LG revealed more widespread hypometabolism than in PS. Two sibling with dihydropteridine reductase deficiency, a patient with classical PKU, and a boy with cytochrome c oxidase deficiency showed reduced glucose utilization in the caudate and putamen. A marked decrease in glucose utilization was found in the cortical gray matter of a patient with rapidly progressive SSPE, despite relatively preserved utilization in the caudate and putamen. The PET study of a patient with slowly progressive SSPE revealed patterns and values of glucose utilization similar to those of the control. Thus, PET provided a useful clue toward understanding brain dysfunction in LG, PS, PKU, Leigh disease, and SSPE. (orig.)

  14. Cerebral glucose utilization in pediatric neurological disorders determined by positron emission tomography

    Energy Technology Data Exchange (ETDEWEB)

    Yanai, Kazuhiko; Iinuma, Kazuie; Miyabayashi, Shigeaki; Narisawa, Kuniaki; Tada, Keiya; Matsuzawa, Taiju; Ito, Masatoshi; Yamada, Kenji

    1987-09-01

    We measured local cerebral glucose utilization in 19 patients with Lennox-Gastaut syndrome (LG), partial seizures (PS), atypical and classical phenylketonuria (PKU), Leigh disease, and subacute sclerosing panencephalitis (SSPE), using positron emission tomography (PET). The mean values of regional glucose utilization in interictal scans of LG were significantly reduced in all brain regions when compared with that of PS (P<0.005). PET studies of glucose utilization in LG revealed more widespread hypometabolism than in PS. Two sibling with dihydropteridine reductase deficiency, a patient with classical PKU, and a boy with cytochrome c oxidase deficiency showed reduced glucose utilization in the caudate and putamen. A marked decrease in glucose utilization was found in the cortical gray matter of a patient with rapidly progressive SSPE, despite relatively preserved utilization in the caudate and putamen. The PET study of a patient with slowly progressive SSPE revealed patterns and values of glucose utilization similar to those of the control. Thus, PET provided a useful clue toward understanding brain dysfunction in LG, PS, PKU, Leigh disease, and SSPE.

  15. CT findings of infant epilepsy

    International Nuclear Information System (INIS)

    Hojoh, Hiroatsu; Kataoka, Kenkichi; Nakagawa, Yoshihiro; Nakano, Shozo; Tomita, Yutaka.

    1982-01-01

    CT diagnosis of infantile epilepsy was evaluated. High incidence of abnormal CT findings in infantile spasms and Lennox-Gastaut syndrome was same as in other reports. Comparison between CT findings and neurological complications and that between CT findings and electroencephalogram findings revealed a stronger relationship existing in the former. This suggested that CT is more useful as a measure to detect underlying diseases which are due to organic change of the brain to cause epilepsy, rather than as that to disclose epileptic primary lesions of functional change. (Ueda, J.)

  16. Posterior reversible encephalopathy syndrome and acute post-streptococcal glomerulonephritis mimicking breakthrough seizures

    Directory of Open Access Journals (Sweden)

    Kamille Abdool

    2015-05-01

    Full Text Available We report the case of a 14-year-old boy with a past history of primary generalized seizures, who had been seizure-free for 2 years on sodium valproate and presented with generalized tonic clonic seizures suggestive of breakthrough seizures. Examination revealed hypertension, impetiginous lesions of the lower limbs, microscopic hematuria, elevated antistreptolysin O titre and low complement levels consistent with acute post-streptococcal glomerulonephritis. Cranial magnetic resonance imaging (MRI demonstrated changes consistent with posterior reversible encephalopathy syndrome. Hypertension was controlled with intravenous nitroglycerin followed by oral captopril and amlodipine. Brain MRI changes returned normal within 2 weeks. The nephritis went in to remission within 2 months and after 8 months the patient has been seizure free again. Posterior reversible encephalopathy syndrome appeared to have neither short nor intermediate effect on seizure control in this patient. The relationship between posterior reversible encephalopathy syndrome and seizures is reviewed.

  17. Deconstructing tolerance with clobazam

    Science.gov (United States)

    Wechsler, Robert T.; Sankar, Raman; Montouris, Georgia D.; White, H. Steve; Cloyd, James C.; Kane, Mary Clare; Peng, Guangbin; Tworek, David M.; Shen, Vivienne; Isojarvi, Jouko

    2016-01-01

    Objective: To evaluate potential development of tolerance to adjunctive clobazam in patients with Lennox-Gastaut syndrome. Methods: Eligible patients enrolled in open-label extension study OV-1004, which continued until clobazam was commercially available in the United States or for a maximum of 2 years outside the United States. Enrolled patients started at 0.5 mg·kg−1·d−1 clobazam, not to exceed 40 mg/d. After 48 hours, dosages could be adjusted up to 2.0 mg·kg−1·d−1 (maximum 80 mg/d) on the basis of efficacy and tolerability. Post hoc analyses evaluated mean dosages and drop-seizure rates for the first 2 years of the open-label extension based on responder categories and baseline seizure quartiles in OV-1012. Individual patient listings were reviewed for dosage increases ≥40% and increasing seizure rates. Results: Data from 200 patients were included. For patients free of drop seizures, there was no notable change in dosage over 24 months. For responder groups still exhibiting drop seizures, dosages were increased. Weekly drop-seizure rates for 100% and ≥75% responders demonstrated a consistent response over time. Few patients had a dosage increase ≥40% associated with an increase in seizure rates. Conclusions: Two-year findings suggest that the majority of patients do not develop tolerance to the antiseizure actions of clobazam. Observed dosage increases may reflect best efforts to achieve seizure freedom. It is possible that the clinical development of tolerance to clobazam has been overstated. ClinicalTrials.gov identifier: NCT00518713 and NCT01160770. Classification of evidence: This study provides Class III evidence that the majority of patients do not develop tolerance to clobazam over 2 years of treatment. PMID:27683846

  18. Deconstructing tolerance with clobazam: Post hoc analyses from an open-label extension study.

    Science.gov (United States)

    Gidal, Barry E; Wechsler, Robert T; Sankar, Raman; Montouris, Georgia D; White, H Steve; Cloyd, James C; Kane, Mary Clare; Peng, Guangbin; Tworek, David M; Shen, Vivienne; Isojarvi, Jouko

    2016-10-25

    To evaluate potential development of tolerance to adjunctive clobazam in patients with Lennox-Gastaut syndrome. Eligible patients enrolled in open-label extension study OV-1004, which continued until clobazam was commercially available in the United States or for a maximum of 2 years outside the United States. Enrolled patients started at 0.5 mg·kg -1 ·d -1 clobazam, not to exceed 40 mg/d. After 48 hours, dosages could be adjusted up to 2.0 mg·kg -1 ·d -1 (maximum 80 mg/d) on the basis of efficacy and tolerability. Post hoc analyses evaluated mean dosages and drop-seizure rates for the first 2 years of the open-label extension based on responder categories and baseline seizure quartiles in OV-1012. Individual patient listings were reviewed for dosage increases ≥40% and increasing seizure rates. Data from 200 patients were included. For patients free of drop seizures, there was no notable change in dosage over 24 months. For responder groups still exhibiting drop seizures, dosages were increased. Weekly drop-seizure rates for 100% and ≥75% responders demonstrated a consistent response over time. Few patients had a dosage increase ≥40% associated with an increase in seizure rates. Two-year findings suggest that the majority of patients do not develop tolerance to the antiseizure actions of clobazam. Observed dosage increases may reflect best efforts to achieve seizure freedom. It is possible that the clinical development of tolerance to clobazam has been overstated. NCT00518713 and NCT01160770. This study provides Class III evidence that the majority of patients do not develop tolerance to clobazam over 2 years of treatment. © 2016 American Academy of Neurology.

  19. Seizures and EEG features in 74 patients with genetic-dysmorphic syndromes.

    Science.gov (United States)

    Alfei, Enrico; Raviglione, Federico; Franceschetti, Silvana; D'Arrigo, Stefano; Milani, Donatella; Selicorni, Angelo; Riva, Daria; Zuffardi, Orsetta; Pantaleoni, Chiara; Binelli, Simona

    2014-12-01

    Epilepsy is one of the most common findings in chromosome aberrations. Types of seizures and severity may significantly vary both between different conditions and within the same aberration. Hitherto specific seizures and EEG patterns are identified for only few syndromes. We studied 74 patients with defined genetic-dysmorphic syndromes with and without epilepsy in order to assess clinical and electroencephalographic features, to compare our observation with already described electro-clinical phenotypes, and to identify putative electroencephalographic and/or seizure characteristics useful to address the diagnosis. In our population, 10 patients had chromosomal disorders, 19 microdeletion or microduplication syndromes, and 32 monogenic syndromes. In the remaining 13, syndrome diagnosis was assessed on clinical grounds. Our study confirmed the high incidence of epilepsy in genetic-dysmorphic syndromes. Moreover, febrile seizures and neonatal seizures had a higher incidence compared to general population. In addition, more than one third of epileptic patients had drug-resistant epilepsy. EEG study revealed poor background organization in 42 patients, an excess of diffuse rhythmic activities in beta, alpha or theta frequency bands in 34, and epileptiform patterns in 36. EEG was completely normal only in 20 patients. No specific electro-clinical pattern was identified, except for inv-dup15, Angelman, and Rett syndromes. Nevertheless some specific conditions are described in detail, because of notable differences from what previously reported. Regarding the diagnostic role of EEG, we found that--even without any epileptiform pattern--the generation of excessive rhythmic activities in different frequency bandwidths might support the diagnosis of a genetic syndrome. © 2014 Wiley Periodicals, Inc.

  20. Parental reporting of response to oral cannabis extracts for treatment of refractory epilepsy.

    Science.gov (United States)

    Press, Craig A; Knupp, Kelly G; Chapman, Kevin E

    2015-04-01

    Oral cannabis extracts (OCEs) have been used in the treatment of epilepsy; however, no studies demonstrate clear efficacy. We report on a cohort of pediatric patients with epilepsy who were given OCE and followed in a single tertiary epilepsy center. A retrospective chart review of children and adolescents who were given OCE for treatment of their epilepsy was performed. Seventy-five patients were identified of which 57% reported any improvement in seizure control and 33% reported a >50% reduction in seizures (responders). If the family had moved to CO for OCE treatment, the responder rate was 47% vs. 22% for children who already were in CO. The responder rate varied based on epilepsy syndrome: Dravet 23%, Doose 0%, and Lennox-Gastaut syndrome (LGS) 88.9%. The background EEG of the 8 responders where EEG data were available was not improved. Additional benefits reported included: improved behavior/alertness (33%), improved language (10%), and improved motor skills (10%). Adverse events (AEs) occurred in 44% of patients including increased seizures (13%) and somnolence/fatigue (12%). Rare adverse events included developmental regression, abnormal movements, status epilepticus requiring intubation, and death. Our retrospective study of OCE use in pediatric patients with epilepsy demonstrates that some families reported patient improvement with treatment; however, we also found a variety of challenges and possible confounding factors in studying OCE retrospectively in an open-labeled fashion. We strongly support the need for controlled, blinded studies to evaluate the efficacy and safety of OCE for treatment of pediatric epilepsies using accurate seizure counts, formal neurocognitive assessments, as well as EEG as a biomarker. This study provides Class III evidence that OCE is well tolerated by children and adolescents with epilepsy. Copyright © 2015 Elsevier Inc. All rights reserved.

  1. Tramadol: seizures, serotonin syndrome, and coadministered antidepressants.

    Science.gov (United States)

    Sansone, Randy A; Sansone, Lori A

    2009-04-01

    This ongoing column is dedicated to the challenging clinical interface between psychiatry and primary care-two fields that are inexorably linked.Tramadol (Ultram(®)) is a commonly prescribed analgesic because of its relatively lower risk of addiction and better safety profile in comparison with other opiates. However, two significant adverse reactions are known to potentially occur with tramadol-seizures and serotonin syndrome. These two adverse reactions may develop during tramadol monotherapy, but appear much more likely to emerge during misuse/overdose as well as with the coadministration of other drugs, particularly antidepressants. In this article, we review the data relating to tramadol, seizures, and serotonin syndrome. This pharmacologic intersection is of clear relevance to both psychiatrists and primary care clinicians.

  2. Corpus callosotomy in Colombia and some reflections on care and research among the poor in developing countries.

    Science.gov (United States)

    Fandiño-Franky, J; Torres, M; Nariño, D; Fandiño, J

    2000-01-01

    The authors present the results of a series of corpus callosotomies (CCS) in 97 patients performed from 1989 to 1997 at the Hospital Neurologico of the Liga Colombiana Contra La Epilepsia, Cartagena, Colombia. This study demonstrates the feasibility of performing these procedures in the developing world and analyzes the outcome and cost of treatment. Patients with medically intractable secondarily generalized epilepsy, bilateral nonfocal epileptic electroencephalogram (EEG), and absence of progressive encephalopathy were accepted as candidates (patients aged 0-30 years; 62 children, 19 girls and 43 boys, with mean age at surgery of 7.9 years; 35 adults, 19 women and 16 men, with mean age at surgery of 25.8 years). Preoperatively, the mean seizure frequency was 12.1 per day, or 364 per month (range, 0.06-200 per day, 1.8-6000 per month). Before surgery, 40% of patients were classified with generalized tonic-clonic seizures of different etiologies, or cryptogenic seizures; 36% had mixed seizures; 19% had Lennox-Gastaut Syndrome; and 5% had West Syndrome. Usually, routine EEG, computed tomography, and clinical findings sufficed for the surgical decision. The standard microsurgical technique performed was an anterior two-thirds CCS by the same surgeon under general anesthesia. In five cases, an additional frontal lobe excision after electrocorticography and subdural electrode monitoring was carried out in the same session. The results were evaluated after a mean follow-up of 35 months (range, 12-28). Two thirds of patients became seizure-free or were left with none or some disabling seizures. AED medication was eased slightly after surgery. The complication rate was low. The patients underwent postoperative psychosocial studies and neuropsychological rehabilitation and showed tendencies toward improvement. The direct cost of CCS in U.S. dollars (US$) ranged between 3,137 and 3,995 depending on the preoperative studies. Thus, CCS is well suited for selected patients in

  3. Trial of Cannabidiol for Drug-Resistant Seizures in the Dravet Syndrome.

    Science.gov (United States)

    Devinsky, Orrin; Cross, J Helen; Laux, Linda; Marsh, Eric; Miller, Ian; Nabbout, Rima; Scheffer, Ingrid E; Thiele, Elizabeth A; Wright, Stephen

    2017-05-25

    The Dravet syndrome is a complex childhood epilepsy disorder that is associated with drug-resistant seizures and a high mortality rate. We studied cannabidiol for the treatment of drug-resistant seizures in the Dravet syndrome. In this double-blind, placebo-controlled trial, we randomly assigned 120 children and young adults with the Dravet syndrome and drug-resistant seizures to receive either cannabidiol oral solution at a dose of 20 mg per kilogram of body weight per day or placebo, in addition to standard antiepileptic treatment. The primary end point was the change in convulsive-seizure frequency over a 14-week treatment period, as compared with a 4-week baseline period. The median frequency of convulsive seizures per month decreased from 12.4 to 5.9 with cannabidiol, as compared with a decrease from 14.9 to 14.1 with placebo (adjusted median difference between the cannabidiol group and the placebo group in change in seizure frequency, -22.8 percentage points; 95% confidence interval [CI], -41.1 to -5.4; P=0.01). The percentage of patients who had at least a 50% reduction in convulsive-seizure frequency was 43% with cannabidiol and 27% with placebo (odds ratio, 2.00; 95% CI, 0.93 to 4.30; P=0.08). The patient's overall condition improved by at least one category on the seven-category Caregiver Global Impression of Change scale in 62% of the cannabidiol group as compared with 34% of the placebo group (P=0.02). The frequency of total seizures of all types was significantly reduced with cannabidiol (P=0.03), but there was no significant reduction in nonconvulsive seizures. The percentage of patients who became seizure-free was 5% with cannabidiol and 0% with placebo (P=0.08). Adverse events that occurred more frequently in the cannabidiol group than in the placebo group included diarrhea, vomiting, fatigue, pyrexia, somnolence, and abnormal results on liver-function tests. There were more withdrawals from the trial in the cannabidiol group. Among patients with

  4. Regression of stroke-like lesions in MELAS-syndrome after seizure control.

    Science.gov (United States)

    Finsterer, Josef; Barton, Peter

    2010-12-01

    There are some indications that seizure activity promotes the development of stroke-like episodes, or vice versa, in patients with mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome or other syndromic mitochondrial disorders. A 41-year-old Caucasian female with MELAS syndrome, presenting with short stature, microcytic anaemia, increased blood-sedimentation rate, myopathy, hyper-gammaglobulinaemia, an iron-metabolism defect, migraine-like headaches, and stroke-like episodes, developed complex partial and generalised seizures at age 32 years. Valproic acid was ineffective but after switching to lamotrigine and lorazepam, she became seizure-free for five years and stroke-like episodes did not recur. Cerebral MRI initially showed enhanced gyral thickening and a non-enhanced T2-hyperintensity over the left parieto-temporo-occipital white matter and cortex and enhanced caudate heads. After two years without seizures, the non-enhanced hyperintense parieto-temporo-occipital lesion had disappeared, being attributed to consequent seizure control. The caudate heads, however, remained hyperintense throughout the observational period. This case indicates that adequate seizure control in a patient with MELAS syndrome may prevent the recurrence of stroke-like episodes and may result in the disappearance of stroke-like lesions on MRI.

  5. Klinefelter's Syndrome with Seizure, Pseudohypoparathyroidism Type Ib and Multiple Endocrine Dysfunctions

    Directory of Open Access Journals (Sweden)

    Chwen-Yi Yang

    2005-12-01

    Full Text Available Klinefelter's syndrome is rarely associated with hypocalcemia, especially pseudohypoparathyroidism (PHP type Ib. We describe a case of Klinefelter's syndrome associated with seizure, PHP type Ib and multiple endocrine dysfunctions. A 19-year-old Taiwanese male was admitted due to seizures with loss of consciousness. He had been diagnosed with Klinefelter's syndrome with seizure disorder and hypocalcemia 3 months previously. Physical examination revealed eunuchoidism but no osteodystrophy, while laboratory data revealed severe hypocalcemia, hyperphosphatemia, and elevated parathyroid hormone. Chromosomal study showed 47, XXY. Osteoporosis was found on chest and abdominal radiography. Dense calcification in the cerebrum and cerebellum was shown on brain computed tomography and magnetic resonance imaging. Elevation of the patient's serum calcium level was noted after vitamin D and calcium carbonate supplements were given. Klinefelter's syndrome is rarely associated with PHP type Ib; our patient's hypocalcemia improved after long-term aggressive treatment.

  6. Seizures in Fragile X Syndrome: Characteristics and Comorbid Diagnoses

    Science.gov (United States)

    Berry-Kravis, Elizabeth; Raspa, Melissa; Loggin-Hester, Lisa; Bishop, Ellen; Holiday, David; Bailey, Donald B., Jr.

    2010-01-01

    A national survey of caregivers of individuals with fragile X syndrome addressed characteristics of epilepsy and co-occurring conditions. Of the 1,394 individuals (1,090 males and 304 females) with the full mutation, 14% of males and 6% of females reported seizures. Seizures were more often partial, began between ages 4 and 10 years, and were…

  7. Brain serotonin content regulates the manifestation of tramadol-induced seizures in rats: disparity between tramadol-induced seizure and serotonin syndrome.

    Science.gov (United States)

    Fujimoto, Yohei; Funao, Tomoharu; Suehiro, Koichi; Takahashi, Ryota; Mori, Takashi; Nishikawa, Kiyonobu

    2015-01-01

    Tramadol-induced seizures might be pathologically associated with serotonin syndrome. Here, the authors investigated the relationship between serotonin and the seizure-inducing potential of tramadol. Two groups of rats received pretreatment to modulate brain levels of serotonin and one group was treated as a sham control (n = 6 per group). Serotonin modulation groups received either para-chlorophenylalanine or benserazide + 5-hydroxytryptophan. Serotonin, dopamine, and histamine levels in the posterior hypothalamus were then measured by microdialysis, while simultaneously infusing tramadol until seizure onset. In another experiment, seizure threshold with tramadol was investigated in rats intracerebroventricularly administered with either a serotonin receptor antagonist (methysergide) or saline (n = 6). Pretreatment significantly affected seizure threshold and serotonin fluctuations. The threshold was lowered in para-chlorophenylalanine group and raised in benserazide + 5-hydroxytryptophan group (The mean ± SEM amount of tramadol needed to induce seizures; sham: 43.1 ± 4.2 mg/kg, para-chlorophenylalanine: 23.2 ± 2.8 mg/kg, benserazide + 5-hydroxytryptophan: 59.4 ± 16.5 mg/kg). Levels of serotonin at baseline, and their augmentation with tramadol infusion, were less in the para-chlorophenylalanine group and greater in the benserazide + 5-hydroxytryptophan group. Furthermore, seizure thresholds were negatively correlated with serotonin levels (correlation coefficient; 0.71, P seizure threshold (P seizures, and that serotonin concentrations were negatively associated with seizure thresholds. Moreover, serotonin receptor antagonism precipitated seizure manifestation, indicating that tramadol-induced seizures are distinct from serotonin syndrome.

  8. Lennox–Gastaut syndrome: impact on the caregivers and families of patients

    Directory of Open Access Journals (Sweden)

    Gibson PA

    2014-10-01

    Full Text Available Patricia A Gibson Epilepsy Information Service, Wake Forest University School of Medicine, Winston-Salem, NC, USA Abstract: Lennox–Gastaut syndrome (LGS has a major impact on the health-related quality of life (HRQL of the affected children as well as their caregivers. The primary caregiver in the family is generally the mother, with support from the father and siblings. The burden of care and the effects of the disease on the child necessitate adjustments in virtually all aspects of the lives of their family. These adjustments inevitably affect the physical, emotional, social, and financial health of the whole family. Numerous sources of support for families can help to ease the burden of care. Improvements in the treatment of LGS, in addition to helping the child with LGS, would likely help improve the HRQL of the family members. This pilot parent survey was designed to explore the impact of epilepsy on caregiver HRQL. Parents of children with epilepsy who had contacted the Epilepsy Information Service at the Wake Forest University School of Medicine, Winston-Salem, NC, USA, were sent questionnaires comprising open- and closed-ended questions. A total of 200 surveys were distributed, with a return rate of 48%. The results revealed that 74% of the parents believed that having a child with epilepsy brought them and their partner closer together. However, when the parents were asked to explain the manner in which epilepsy affected their families, answers included continuous stress, major financial distress, and lack of time to spend with other children. Information and resources for the families of children with LGS could help improve the HRQL of both the patients and their relatives. Keywords: health-related quality of life, HRQL, epilepsy, relatives, siblings

  9. EEG features of absence seizures in idiopathic generalized epilepsy: Impact of syndrome, age, and state

    DEFF Research Database (Denmark)

    Sadleir, L.G.; Scheffer, I.E.; Smith, S.

    2009-01-01

    Purpose: Factors influencing the electroencephalography (EEG) features of absence seizures in newly presenting children with idiopathic generalized epilepsy (IGE) have not been rigorously studied. We examined how specific factors such as state, provocation, age, and epilepsy syndrome affect the EEG...... features of absence seizures. Methods: Children with untreated absence seizures were studied using video-EEG recording. The influence of state of arousal, provocation (hyperventilation, photic stimulation), age, and epilepsy syndrome on specific EEG features was analyzed. Results: Five hundred nine...... seizures were evaluated in 70 children with the following syndromes: childhood absence epilepsy (CAE) 37, CAE+ photoparoxysmal response (PPR) 10, juvenile absence epilepsy (JAE) 8, juvenile myoclonic epilepsy (JME) 6, and unclassified 9. Polyspikes occurred in all syndromes but were more common in JME...

  10. The ketogenic diet can be used successfully in combination with corticosteroids for epileptic encephalopathies.

    Science.gov (United States)

    Ville, Dorothée; Chiron, Catherine; Laschet, Jacques; Dulac, Olivier

    2015-07-01

    Hormonal therapy or ketogenic diet often permits overcoming the challenging periods of many epileptic encephalopathies (West and Lennox-Gastaut syndromes and encephalopathy with continuous spike-waves in slow sleep), but relapse affects over 20% of patients. We report here a monocenter pilot series of 42 consecutive patients in whom we combined oral steroids with the ketogenic diet for corticosteroid-resistant or -dependent epileptic encephalopathy. We retrospectively evaluated the effect on seizure frequency, interictal spike activity, neuropsychological course, and steroid treatment course. Twenty-three patients had West syndrome (WS), 13 had encephalopathy with continuous spike-waves in slow sleep (CSWS), and six others had miscellaneous epileptic encephalopathies. All patients succeeded to reach 0.8 to 1.6g/l ketone bodies in the urine following the usual KD regimen. For at least 6 months, 14/42 responded to the addition of the ketogenic diet: 4/23 with WS, 8/13 with CSWS, and 2/6 with miscellaneous epileptic encephalopathies. The addition of the KD allowed withdrawing steroids in all responders. Among them, 10/15 had been patients with steroid-dependent epileptic encephalopathy and 4/27 patients with steroid-resistant epileptic encephalopathy. Therefore, the ketogenic diet can be used successfully in combination with corticosteroids for epileptic encephalopathies. Patients presenting with steroid-dependent CSWS seem to be the best candidates. Copyright © 2015 Elsevier Inc. All rights reserved.

  11. Rufinamide from clinical trials to clinical practice in the United States and Europe.

    Science.gov (United States)

    Resnick, Trevor; Arzimanoglou, Alexis; Brown, Lawrence W; Flamini, Robert; Kerr, Michael; Kluger, Gerhard; Kothare, Sanjeev; Philip, Sunny; Harrison, Miranda; Narurkar, Milind

    2011-05-01

    Rufinamide is a triazole derivative structurally unrelated to other antiepileptic drugs that is indicated for the adjunctive treatment of seizures associated with Lennox-Gastaut syndrome (LGS) in patients aged ≥4 years. Originally granted orphan drug status, marketing authorisation was obtained on the basis of a randomised, double-blind, placebo-controlled trial conducted in 138 LGS patients. An open-label extension study subsequently demonstrated that rufinamide's efficacy and tolerability were maintained over the longer term (median duration of treatment, 432 days). Recently published reports from Europe and the United States have described the use of adjunctive rufinamide to treat LGS in clinical practice. These data complement the clinical trial results, by providing information on the efficacy and tolerability of rufinamide when used on an individualised basis in real-world practice, under less tightly restricted conditions in terms of patient population and dosing strategies. A comparison of the data reveals that a "lower and slower" dosing strategy tends to be adopted in clinical practice, in comparison with the clinical trial, which does not appear to compromise efficacy, but may provide improvements in tolerability. Individual case reports provide additional valuable information on how rufinamide is being used to treat different seizure types associated with LGS. Since clinical experience with rufinamide is currently at an early stage, there are still unanswered questions relating to its use, and it is likely that its place in the adjunctive treatment of LGS will evolve as further data emerge.

  12. SCN1A-related Dravet syndrome : Vaccinations and seizure precipitants in disease course and diagnosis

    NARCIS (Netherlands)

    Verbeek, N.E.

    2015-01-01

    Febrile seizures are well known adverse events following childhood vaccinations. If a seizure following vaccination is the first of an evolving epilepsy syndrome, the vaccination might be misinterpreted as the primary cause of the epilepsy. Dravet syndrome (formerly known as severe myoclonic

  13. Effect of vaccinations on seizure risk and disease course in Dravet syndrome

    NARCIS (Netherlands)

    Verbeek, N.E.; van der Maas, N.A.T.; Sonsma, A.C.M.; Ippel, E.; Bondt, P.E.V.D.; Hagebeuk, E.; Jansen, F.E.; Geesink, H.H.; Braun, K.P.; de Louw, A.; Augustijn, P.B.; Neuteboom, R.F.; Schieving, J.H.; Stroink, H.; Vermeulen, R.J.; Nicolai, J.; Brouwer, O.F.; van Kempen, M.; de Kovel, C.G.F.; Kemmeren, J.M.; Koeleman, B.P.C.; Knoers, N.V.; Lindhout, D.; Gunning, W.B.; Brilstra, E.H.

    2015-01-01

    Objective: To study the effect of vaccination-associated seizure onset on disease course and estimate the risk of subsequent seizures after infant pertussis combination and measles, mumps, and rubella (MMR) vaccinations in Dravet syndrome (DS). Methods: We retrospectively analyzed data from hospital

  14. Study on cranial computed tomography in infants and children with central nervous system disorders, 3

    International Nuclear Information System (INIS)

    Kumanomidou, Yoshiaki

    1981-01-01

    Studies by cranial computed tomography (CT) and electroencephalography (EEG) were made on patients with epilepsy ranging from 2 months to 20 years of age (mean age: 8 years and 7 months) and the results were as follows: 1) Patients with motor disturbance had CT-abnormality twice or more as frequent as patients without the disturbance.2) In groups without motor disturbance, the patients with infantile spasms, Lennox-Gastaut syndrome or complex partial epilepsy were rather frequently associated with abnormal CT-findings. 3) Patients with mental retardation showed CT-abnormalities more frequently than patients without the retardation. 4) Patients with uncontrolled seizures indicated CT-abnormalities more often than the controlled patients. 5) There was a definite correlation between background activity on EEG (BGA) and CT-findings, in which most patients with normal BGA showed normal CT-findings and most patients with abnormal BGA showed CT-abnormalities particularly when the BGA disclosed unilateral abnormalities. 6) In 70% of patients showing unilateral BGA abnormalities, the side of abnormal EEG was in accordance with side of CT-abnormalities. In 64% of patients showing hemispherial CT-abnormalities, on the other hand, the side of CT-abnormalities was the same as the side of EEG abnormalities. 7) Sides of seizure discharges on EEG were the same as sides of abnormalities on CT in 24% of patients and the localization of seizure discharges was identical to the location of CT-abnormalities in 16% of patients. In 33 patients with abnormality on CT, however, the side of CT-abnormalities was the same as sides of EEG abnormalities in 61% of the cases and the location of CT-abnormalities was the same as that localization of seizure discharges on EEG in 39%. (author)

  15. Seizure and EEG patterns in Wolf-Hirschhorn (4p-) syndrome.

    Science.gov (United States)

    Battaglia, Agatino; Carey, John C

    2005-08-01

    Wolf-Hirschhorn syndrome (WHS) is a well-characterized chromosomal disorder that occurs due to partial deletion of the short arm of chromosome 4 (4p-). Although, about 300 cases have been reported to date, limited data are available on electroclinical findings. Information given to parents at the time of diagnosis tends to be skewed to the extreme negative. To delineate the natural history of seizures and EEG patterns in WHS, and obtain better information on diagnosis or outcome in a clinical setting, we reviewed the available literature on electroclinical findings of WHS. 4p- syndrome is characterized by distinctive seizure and EEG patterns that facilitate the early diagnosis and management of such patients.

  16. Substantial and sustained seizure reduction with ketogenic diet in a patient with Ohtahara syndrome

    Directory of Open Access Journals (Sweden)

    Adithya Sivaraju

    2015-01-01

    Full Text Available Ketogenic diet has been shown to be efficacious in some epileptic encephalopathies but rarely reported as being useful in children with Ohtahara syndrome. This could possibly be attributed to the rarity of the disease and associated short survival period. We report on a 5-year-old child with Ohtahara syndrome, whose seizures failed to improve with all known medications, continued to show persistent suppression-burst pattern on the electroencephalography (EEG and had substantial reduction in seizure frequency for one year post-initiation of ketogenic diet. He has not had a single visit to the emergency room because of seizures in the last one year, and more importantly, there has been a clear improvement noted in his level of interaction and temperament. Patients with Ohtahara syndrome invariably have medically intractable seizures and catastrophic neurodevelopmental outcome. Ketogenic diet is a treatment modality that might be worth considering even in this group of patients.

  17. Chronic Granulomatous Herpes Encephalitis in a Child with Clinically Intractable Epilepsy

    Directory of Open Access Journals (Sweden)

    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.

  18. Seizures and pain uncertainty associated with parenting stress and Rett syndrome.

    Science.gov (United States)

    Byiers, Breanne J; Tervo, Raymond C; Feyma, Timothy J; Symons, Frank J

    2014-04-01

    Data were collected parenting stress, adaptive behavior, pain, and health issues from the caregivers of 35 girls and women with Rett syndrome (mean age = 20.3). A majority (60%) of parents reported stress in the clinical range on at least 1 subscale of the Parenting Stress Index-Short Form. Seizures and uncertainty about their daughter's gastrointestinal pain experience were significantly associated with higher levels of parenting stress. No other child factors (adaptive behavior, age, residential status) were significantly related to parenting stress. Factors related to chronic health concerns (seizures, ambiguous pain presentation) may be important when considering family stress issues in relation to general outcomes for girls with Rett syndrome and related developmental disorders.

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

    Directory of Open Access Journals (Sweden)

    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.

  20. Neonatal hypocalcemia, neonatal seizures, and intellectual disability in 22q11.2 deletion syndrome

    Science.gov (United States)

    Cheung, Evelyn Ning Man; George, Susan R.; Andrade, Danielle M.; Chow, Eva W. C.; Silversides, Candice K.; Bassett, Anne S.

    2015-01-01

    Purpose Hypocalcemia is a common endocrinological condition in 22q11.2 deletion syndrome. Neonatal hypocalcemia may affect neurodevelopment. We hypothesized that neonatal hypocalcemia would be associated with rare, more severe forms of intellectual disability in 22q11.2 deletion syndrome. Methods We used a logistic regression model to investigate potential predictors of intellectual disability severity, including neonatal hypocalcemia, neonatal seizures, and complex congenital heart disease, e.g., interrupted aortic arch, in 149 adults with 22q11.2 deletion syndrome. Ten subjects had moderate-to-severe intellectual disability. Results The model was highly significant (P < 0.0001), showing neonatal seizures (P = 0.0018) and neonatal hypocalcemia (P = 0.047) to be significant predictors of a more severe level of intellectual disability. Neonatal seizures were significantly associated with neonatal hypocalcemia in the entire sample (P < 0.0001), regardless of intellectual level. There was no evidence for the association of moderate- to-severe intellectual disability with other factors such as major structural brain malformations in this sample. Conclusion The results suggest that neonatal seizures may increase the risk for more severe intellectual deficits in 22q11.2 deletion syndrome, likely mediated by neonatal hypocalcemia. Neonatal hypocalcemia often remains unrecognized until the postseizure period, when damage to neurons may already have occurred. These findings support the importance of early recognition and treatment of neonatal hypocalcemia and potentially neonatal screening for 22q11.2 deletions. PMID:23765047

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

    Science.gov (United States)

    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.

  2. Dopey's seizure.

    Science.gov (United States)

    Dan, B; Christiaens, F

    1999-06-01

    Angelman syndrome is a neurogenetic condition namely characterized by developmental delay, virtual absence of expressive verbal language, peculiar organization of movement, seizures and happy demeanor. This syndrome has been recognized since 1965, but it seems that Walt Disney presented an original depiction of it in his first full-length animated film, including myoclonic jerks and an apparently generalized tonic-clonic seizure. Copyright 1999 BEA Trading Ltd.

  3. Prevalence of SCN1A-related dravet syndrome among children reported with seizures following vaccination: a population-based ten-year cohort study.

    Directory of Open Access Journals (Sweden)

    Nienke E Verbeek

    Full Text Available OBJECTIVES: To determine the prevalence of Dravet syndrome, an epileptic encephalopathy caused by SCN1A-mutations, often with seizure onset after vaccination, among infants reported with seizures following vaccination. To determine differences in characteristics of reported seizures after vaccination in children with and without SCN1A-related Dravet syndrome. METHODS: Data were reviewed of 1,269 children with seizures following immunization in the first two years of life, reported to the safety surveillance system of the Dutch national immunization program between 1 January 1997 and 31 December 2006. Selective, prospective follow-up was performed of children with clinical characteristics compatible with a diagnosis of Dravet syndrome. RESULTS: In 21.9% (n = 279 of children, a diagnosis of Dravet syndrome could not be excluded based on available clinical data (median age at follow-up 16 months. Additional follow-up data were obtained in 83.9% (n = 234 of these children (median age 8.5 years. 15 (1.2% of 1,269; 95%CI:0.6 to 1.8% children were diagnosed with SCN1A-related Dravet syndrome. Of all reported seizures following vaccinations in the first year of life, 2.5% (95%CI:1.3 to 3.6% were due to SCN1A-related Dravet syndrome, as were 5.9% of reported seizures (95%CI:3.1 to 8.7% after 2(nd or 3(rd DTP-IPV-Hib vaccination. Seizures in children with SCN1A-related Dravet syndrome occurred more often with a body temperature below 38.5°C (57.9% vs. 32.6%, p = 0.020 and reoccurred more often after following vaccinations (26.7% vs. 4.0%, p = 0.003, than in children without a diagnosis of SCN1A-related Dravet Syndrome. CONCLUSIONS: Although Dravet syndrome is a rare genetic epilepsy syndrome, 2.5% of reported seizures following vaccinations in the first year of life in our cohort occurred in children with this disorder. Knowledge on the specific characteristics of vaccination-related seizures in this syndrome might promote early diagnosis

  4. Continuous spike-waves during slow waves sleep: a clinical and electroencephalografic study in fifteen children Ponta-onda contínua do sono lento: estudo clínico e eletrencefalográfico em quinze crianças

    Directory of Open Access Journals (Sweden)

    ADRIANA A. F. DJABRAIAN

    1999-09-01

    Full Text Available We report on the clinical and EEG features of 15 patients with the syndrome of "continuous spike waves during slow wave sleep" (CSWSS. The differential diagnosis of CSWSS includes benign epilepsy of childhood with centro-temporal spikes, and Landau-Kleffner and Lennox-Gastaut syndromes. We found normal CT and MRI features in 6 cases, periventricular leukomalacia with and without diffuse brain atrophy in 4 cases and hydrocephalus in 1 case. There was no association between specific neurological findings and CSWSS. Nine of our cases had relatively focal discharges, like some cases from the literature. The occurrence of CSWSS appears to be age-related, generaly between the ages of 5 to 12 years, with a strong temporal relation to the neupsychological deterioration in its nature, severity and prognosis. We believe that this striking disorder has been overlooked and that routine sleep EEG studies on epileptic children may disclose additional cases of CSWSS.Relatamos as características clínicas e eletroencefalográficas de 15 patientes com a síndrome de ponta-onda contínua do sono não-REM (POCSNR. O diagnóstico diferencial da POCSNR inclue a epilepsia benigna da infância com pontas centro-temporais e as síndromes de Landau-Kleffner e Lennox-Gastaut. Encontramos TC e RNM de crânio normais em 6 casos, leucomalácia periventricular em 4 e hidrocefalia em 1. Não houve associação de achados neurológicos específicos e a POCSNR. Nove dos nossos casos tinham descargas relativamente focais, como alguns casos da literatura. A ocorrência da POCSNR parece ser idade-dependente, geralmente entre 5 e 12 anos, com forte relação temporal à deteriorização neurocognitiva, em sua natureza, severidade e prognóstico. Acreditamos que esta síndrome tem sido pouco diagnosticada e que a realização rotineira de EEG em sono em crianças epilépticas possa revelar novos casos de POCSNR.

  5. Mulberry Tumors in Retina and Nasal Hamartoma in a Patient With Tuberous Sclerosis

    Directory of Open Access Journals (Sweden)

    S. Reshadat

    2008-10-01

    Full Text Available Introduction: Tuberous Sclerosis (TS is an autosomal dominant disease that affects the brain, skin, eye, heart, kidney even bones. The commonest presentation is seizures in infancy or early childhood (in 80% of cases, mental retardation (in 44%of cases. Characteristic skin lesion includes facial angiofibromas, adenoma sebaceum, hypopigmented macules, shagreen patches ungual ungual fibromas, ash leaf spots, cafe'-au-lait spots.Case Report: A nine years old male was admitted in a pediatric hospital because of the status myoclonic seizures. Seizures had been started since infancy. In physical exam he had some hypopigmented macules, cafe'-au-lait spots and ash leaf lesions, frontal fibrosis and also shagreen patches. Patient was a case of mild mentally retardation with no any focal neurological deficit. Computed tomography scan of brain and MRI imaging revealed sub ependymal tubers with multiple calcification in both sides of parietal region. Electroencephal-ogram recording suggested abnormal spike, sharp wave discharges and lennox-Gastaut pattern. The diagnosis based on the history and physical exam and MRI were tuberous sclerosis. His foundoscopic exam revealed two prominent calcified mass around right optic disc in supratemporal arch, left eye was normal. Retinal angiography revealed the mulberry tumors and right phakoma of retina. Conclusion: Computed tomography also revaled the nasal hamartoma. Histopathologic examination confirmed the diagnosis of angiomyolipoma because the lesion was composed of smooth muscle bundles, mature adipose tissue and blood vessels of different sizes. He remained seizures free after treatment.

  6. Monitoring the autonomic nervous activity as the objective evaluation of music therapy for severely and multiply disabled children.

    Science.gov (United States)

    Orita, Makiko; Hayashida, Naomi; Shinkawa, Tetsuko; Kudo, Takashi; Koga, Mikitoshi; Togo, Michita; Katayama, Sotetsu; Hiramatsu, Kozaburo; Mori, Shunsuke; Takamura, Noboru

    2012-07-01

    Severely and multiply disabled children (SMDC) are frequently affected in more than one area of development, resulting in multiple disabilities. The aim of the study was to evaluate the efficacy of music therapy in SMDC using monitoring changes in the autonomic nervous system, by the frequency domain analysis of heart rate variability. We studied six patients with SMDC (3 patients with cerebral palsy, 1 patient with posttraumatic syndrome after head injury, 1 patient with herpes encephalitis sequelae, and 1 patient with Lennox-Gastaut syndrome characterized by frequent seizures, developmental delay and psychological and behavioral problems), aged 18-26 (mean 22.5 ± 3.5). By frequency domain method using electrocardiography, we measured the high frequency (HF; with a frequency ranging from 0.15 to 0.4 Hz), which represents parasympathetic activity, the low frequency/high frequency ratio, which represents sympathetic activity between the sympathetic and parasympathetic activities, and heart rate. A music therapist performed therapy to all patients through the piano playing for 50 min. We monitored each study participant for 150 min before therapy, 50 min during therapy, and 10 min after therapy. Interestingly, four of 6 patients showed significantly lower HF components during music therapy than before therapy, suggesting that these four patients might react to music therapy through the suppression of parasympathetic nervous activities. Thus, music therapy can suppress parasympathetic nervous activities in some patients with SMDC. The monitoring changes in the autonomic nervous activities could be a powerful tool for the objective evaluation of music therapy in patients with SMDC.

  7. Chromosomal microarray testing identifies a 4p terminal region associated with seizures in Wolf–Hirschhorn syndrome

    Science.gov (United States)

    South, Sarah T; Lortz, Amanda; Hensel, Charles H; Sdano, Mallory R; Vanzo, Rena J; Martin, Megan M; Peiffer, Andreas; Lambert, Christophe G; Calhoun, Amy; Carey, John C; Battaglia, Agatino

    2016-01-01

    Background Wolf–Hirschhorn syndrome (WHS) is a contiguous gene deletion syndrome involving variable size deletions of the 4p16.3 region. Seizures are frequently, but not always, associated with WHS. We hypothesised that the size and location of the deleted region may correlate with seizure presentation. Methods Using chromosomal microarray analysis, we finely mapped the breakpoints of copy number variants (CNVs) in 48 individuals with WHS. Seizure phenotype data were collected through parent-reported answers to a comprehensive questionnaire and supplemented with available medical records. Results We observed a significant correlation between the presence of an interstitial 4p deletion and lack of a seizure phenotype (Fisher's exact test p=3.59e-6). In our cohort, there were five individuals with interstitial deletions with a distal breakpoint at least 751 kbp proximal to the 4p terminus. Four of these individuals have never had an observable seizure, and the fifth individual had a single febrile seizure at the age of 1.5 years. All other individuals in our cohort whose deletions encompass the terminal 751 kbp region report having seizures typical of WHS. Additional examples from the literature corroborate these observations and further refine the candidate seizure susceptibility region to a region 197 kbp in size, starting 368 kbp from the terminus of chromosome 4. Conclusions We identify a small terminal region of chromosome 4p that represents a seizure susceptibility region. Deletion of this region in the context of WHS is sufficient for seizure occurrence. PMID:26747863

  8. Chromosomal microarray testing identifies a 4p terminal region associated with seizures in Wolf-Hirschhorn syndrome.

    Science.gov (United States)

    Ho, Karen S; South, Sarah T; Lortz, Amanda; Hensel, Charles H; Sdano, Mallory R; Vanzo, Rena J; Martin, Megan M; Peiffer, Andreas; Lambert, Christophe G; Calhoun, Amy; Carey, John C; Battaglia, Agatino

    2016-04-01

    Wolf-Hirschhorn syndrome (WHS) is a contiguous gene deletion syndrome involving variable size deletions of the 4p16.3 region. Seizures are frequently, but not always, associated with WHS. We hypothesised that the size and location of the deleted region may correlate with seizure presentation. Using chromosomal microarray analysis, we finely mapped the breakpoints of copy number variants (CNVs) in 48 individuals with WHS. Seizure phenotype data were collected through parent-reported answers to a comprehensive questionnaire and supplemented with available medical records. We observed a significant correlation between the presence of an interstitial 4p deletion and lack of a seizure phenotype (Fisher's exact test p=3.59e-6). In our cohort, there were five individuals with interstitial deletions with a distal breakpoint at least 751 kbp proximal to the 4p terminus. Four of these individuals have never had an observable seizure, and the fifth individual had a single febrile seizure at the age of 1.5 years. All other individuals in our cohort whose deletions encompass the terminal 751 kbp region report having seizures typical of WHS. Additional examples from the literature corroborate these observations and further refine the candidate seizure susceptibility region to a region 197 kbp in size, starting 368 kbp from the terminus of chromosome 4. We identify a small terminal region of chromosome 4p that represents a seizure susceptibility region. Deletion of this region in the context of WHS is sufficient for seizure occurrence. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  9. Effect of vaccinations on seizure risk and disease course in Dravet syndrome.

    Science.gov (United States)

    Verbeek, Nienke E; van der Maas, Nicoline A T; Sonsma, Anja C M; Ippel, Elly; Vermeer-de Bondt, Patricia E; Hagebeuk, Eveline; Jansen, Floor E; Geesink, Huibert H; Braun, Kees P; de Louw, Anton; Augustijn, Paul B; Neuteboom, Rinze F; Schieving, Jolanda H; Stroink, Hans; Vermeulen, R Jeroen; Nicolai, Joost; Brouwer, Oebele F; van Kempen, Marjan; de Kovel, Carolien G F; Kemmeren, Jeanet M; Koeleman, Bobby P C; Knoers, Nine V; Lindhout, Dick; Gunning, W Boudewijn; Brilstra, Eva H

    2015-08-18

    To study the effect of vaccination-associated seizure onset on disease course and estimate the risk of subsequent seizures after infant pertussis combination and measles, mumps, and rubella (MMR) vaccinations in Dravet syndrome (DS). We retrospectively analyzed data from hospital medical files, child health clinics, and the vaccination register for children with DS and pathogenic SCN1A mutations. Seizures within 24 hours after infant whole-cell, acellular, or nonpertussis combination vaccination or within 5 to 12 days after MMR vaccination were defined as "vaccination-associated." Risks of vaccination-associated seizures for the different vaccines were analyzed in univariable and in multivariable logistic regression for pertussis combination vaccines and by a self-controlled case series analysis using parental seizure registries for MMR vaccines. Disease courses of children with and without vaccination-associated seizure onset were compared. Children who had DS (n = 77) with and without vaccination-associated seizure onset (21% and 79%, respectively) differed in age at first seizure (median 3.7 vs 6.1 months, p risk of subsequent vaccination-associated seizures was significantly lower for acellular pertussis (9%; odds ratio 0.18, 95% confidence interval [CI] 0.05-0.71) and nonpertussis (8%; odds ratio 0.11, 95% CI 0.02-0.59) than whole-cell pertussis (37%; reference) vaccines. Self-controlled case series analysis showed an increased incidence rate ratio of seizures of 2.3 (95% CI 1.5-3.4) within the risk period of 5 to 12 days following MMR vaccination. Our results suggest that vaccination-associated earlier seizure onset does not alter disease course in DS, while the risk of subsequent vaccination-associated seizures is probably vaccine-specific. © 2015 American Academy of Neurology.

  10. An Atypical Presentation of Subacute Encephalopathy with Seizures in Chronic Alcoholism Syndrome.

    Science.gov (United States)

    Kim, Tae-Kyoung; Jung, Eui Sung; Park, Jong-Moo; Kang, Kyusik; Lee, Woong-Woo; Lee, Jung-Ju

    2016-06-01

    Subacute encephalopathy with seizures in chronic alcoholism syndrome is a rare clinical manifestation in patients with chronic alcohol abuse. We report the case of a patient with chronic alcoholism who presented with partial nonconvulsive status epilepticus associated with a thalamic lesion.

  11. An Atypical Presentation of Subacute Encephalopathy with Seizures in Chronic Alcoholism Syndrome

    OpenAIRE

    Kim, Tae-Kyoung; Jung, Eui Sung; Park, Jong-Moo; Kang, Kyusik; Lee, Woong-Woo; Lee, Jung-Ju

    2016-01-01

    Subacute encephalopathy with seizures in chronic alcoholism syndrome is a rare clinical manifestation in patients with chronic alcohol abuse. We report the case of a patient with chronic alcoholism who presented with partial nonconvulsive status epilepticus associated with a thalamic lesion.

  12. [Cryptogenic West syndrome: Clinical profile, response to treatment and prognostic factors].

    Science.gov (United States)

    Calderón Romero, María; Arce Portillo, Elena; López Lobato, Mercedes; Muñoz Cabello, Beatriz; Blanco Martínez, Bárbara; Madruga Garrido, Marcos; Alonso Luego, Olga

    2017-12-06

    West syndrome (WS) is an age-dependent epileptic encephalopathy in which the prognosis varies according to the, not always identified, underlying origin. To define the profile of cryptogenic (a least studied isolated sub-group) WS, in Spain. To study its outcome, response to different treatments, and to establish prognostic factors. The study included a review of the medical records of 16 patients diagnosed with cryptogenic WS during the period, 2000-2015. The mean follow-up time was 6.6 years, with a minimum of 2 years. The large majority (11/16) were male. The mean age at onset was 6 months, and 6/16 had a family history of idiopathic epilepsy. The first line treatment with vigabatrin had an electrical-clinical response in 5/16 patients, with the remaining cases responding to adrenocorticotropic hormone (ACTH). Almost half (44%) of the patients progressed to other types of epilepsy, with no difference between those treated with vigabatrin or ACTH. A greater number of adverse effects were obtained with ACTH, with no retinal involvement being observed with vigabatrin. The aetiological cause was found in 2/16. Being female, late onset, and early control of the hypsarrhythmia, were factors of a good prognosis. The overall prognosis of cryptogenic WS was more serious than expected. Although the incidence of Lennox-Gastaut syndrome was low, the progression to focal epilepsy was the most common, with it appearing within the first 2 years of the diagnosis. The initial response to vigabatrin was lower than expected, but the long-term result was comparable to ACTH. Copyright © 2017. Publicado por Elsevier España, S.L.U.

  13. The Lack of Effect of Food on the Pharmacokinetics of ZX008 (Fenfluramine Oral Solution): Results of a Single-dose, Two-period Crossover Study.

    Science.gov (United States)

    Gammaitoni, Arnold; Smith, Steven; Boyd, Brooks

    2018-06-22

    Fenfluramine is being developed as a low-dose adjunctive treatment for seizures in patients with Dravet syndrome and other epileptic encephalopathies, including Lennox-Gastaut syndrome. Most patients with Dravet syndrome receive multiple antiepileptic drugs, making it challenging for caregivers to track correct administration times. The present Phase I study was conducted to determine the effect of food on the pharmacokinetic properties of fenfluramine. Healthy nonsmoking subjects aged 18 to 50years were enrolled in an open-label, crossover, Phase I pharmacokinetic and safety profile study and received 2 single 0.8-mg/kg doses of ZX008 (fenfluramine hydrochloride oral solution), 1 after a 10-hour overnight fast and the other 30 minutes after the start of consumption of a high-fat breakfast, in a randomly assigned order. A washout period of at least 9days separated the 2 treatment periods. Venous blood samples were taken before each dose and periodically for 72hours after each dose for determination of concentrations of fenfluramine and its active metabolite norfenfluramine. Plasma pharmacokinetic parameters were estimated for each subject by noncompartmental analysis. In the 13 subjects completing both treatment periods, food had no effect on the rate or extent of absorption and bioavailability of fenfluramine as assessed by fed vs fasted adjusted geometric mean observed plasma C max (59.1vs 56.7 ng/mL; NS) and AUC 0-∞ (1640vs 1600 ng · h/mL; NS). Additionally, there was no impact of food on systemic exposure of norfenfluramine. Seven subjects reported at least 1 treatment-emergent adverse event; all treatment-emergent adverse events were mild in severity. The bioequivalence and tolerability of single 0.8-mg/kg oral doses of ZX008 in the fed and fasted states support ZX008 administration without regard to meals. Copyright © 2018 The Author(s). Published by Elsevier Inc. All rights reserved.

  14. [Reflex seizures, cinema and television].

    Science.gov (United States)

    Olivares-Romero, Jesús

    2015-12-16

    In movies and television series are few references to seizures or reflex epilepsy even though in real life are an important subgroup of total epileptic syndromes. It has performed a search on the topic, identified 25 films in which they appear reflex seizures. Most seizures observed are tonic-clonic and visual stimuli are the most numerous, corresponding all with flashing lights. The emotions are the main stimuli in higher level processes. In most cases it is not possible to know if a character suffers a reflex epilepsy or suffer reflex seizures in the context of another epileptic syndrome. The main conclusion is that, in the movies, the reflex seizures are merely a visual reinforcing and anecdotal element without significant influence on the plot.

  15. Right hemispheric reversible cerebral vasoconstriction syndrome in a patient with left hemispheric partial seizures.

    Science.gov (United States)

    Perez, Gina S; McCaslin, Justin; Shamim, Sadat

    2017-04-01

    We report a right-handed 19-year-old girl who developed reversible cerebral vasoconstriction syndrome (RCVS) lateralized to the right hemisphere with simultaneous new-onset left hemispheric seizures. RCVS, typically more diffuse, was lateralized to one of the cerebral hemispheres.

  16. De novo mutations in synaptic transmission genes including DNM1 cause epileptic encephalopathies

    DEFF Research Database (Denmark)

    2014-01-01

    in five individuals and de novo mutations in GABBR2, FASN, and RYR3 in two individuals each. Unlike previous studies, this cohort is sufficiently large to show a significant excess of de novo mutations in epileptic encephalopathy probands compared to the general population using a likelihood analysis (p...... = 8.2 × 10(-4)), supporting a prominent role for de novo mutations in epileptic encephalopathies. We bring statistical evidence that mutations in DNM1 cause epileptic encephalopathy, find suggestive evidence for a role of three additional genes, and show that at least 12% of analyzed individuals have...... analyzed exome-sequencing data of 356 trios with the "classical" epileptic encephalopathies, infantile spasms and Lennox Gastaut syndrome, including 264 trios previously analyzed by the Epi4K/EPGP consortium. In this expanded cohort, we find 429 de novo mutations, including de novo mutations in DNM1...

  17. Seizures as the first manifestation of chromosome 22q11.2 deletion syndrome in a 40-year old man: a case report

    Directory of Open Access Journals (Sweden)

    Tonelli Adriano R

    2007-12-01

    Full Text Available Abstract Background The microdeletion of chromosome 22q11.2 is the most common human deletion syndrome. It typically presents early in life and is rarely considered in adult patients. As part of the manifestations of this condition, patients can have parathyroid glandular involvement ranging from hypocalcemic hypoparathyroidism to normocalcemia with normal parathryroid hormone levels. The first manifestation of the syndrome might be seizures due to profound hypocalcemia. Case presentation A 40-year-old man without significant past medical history presented with a new-onset generalized tonic-clonic seizure. He had no personal history of hypocalcemia or seizures. Physical examination was remarkable for short stature, hypertelorism, prominent forehead and nasal voice. His initial laboratory examination showed hypocalcemia (Calcium 5.2 mg/dl and Calcium ionized 0.69 mmol/l with hypoparathyroidism (Parathyroid hormone intact Conclusion Microdeletion of chromosome 22q11.2 is among the most clinically variable syndromes, with more than 180 features associated with the deletion. It has a variable phenotypical expression, requiring a high level of awareness for its early diagnosis. Seizures, related to marked hypocalcemia due to idiopathic hypoparathyroidism, might be the presenting feature in an adult patient with this syndrome.

  18. 22q11.2 deletion syndrome lowers seizure threshold in adult patients without epilepsy.

    Science.gov (United States)

    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.

  19. Estudo clínico-eletrencefalográfico longitudinal em pacientes epilépticos tratados com Ro 5-4023 Longitudinal clinical and electroencephalographieal studies in epileptic patients treated with Ro 5-4023

    Directory of Open Access Journals (Sweden)

    Michel P. Lison

    1970-03-01

    Full Text Available Estudaram-se as modificações do quadro clínico e do quadro eletrencefalográfico em 22 pacientes com manifestações epilépticas diárias (5 com ausências típicas, 3 com crises psicomotoras, 5 com ausências mioclônicas, 1 com mioclonias de ação e 8 com síndrome de Lennox, tratados com 7-nitro-5-(2clorofenil-3H-1,4 benzodiazepina 2 (1H-one ou Ro 5-4023. Vinte pacientes tinham sido submetidos a terapêuticas anticonvulsivantes usuais sem resultados apreciáveis. Com o uso de Ro 5-4023 as crises foram controladas desde o primeiro dia em 14 pacientes. Em mais 6 doentes houve redução rápida e significativa da intensidade e da freqüência das crises. Destes, um não mais apresentou manifestações a partir da segunda semana de tratamento. Recidivas parciais ocorreram em 8 pacientes. Destaca-se o amplo campo de ação da droga, que age nas epilepsias generalizadas (ausências típicas e ausências mioclônicas, em epilepsias parciais, particularmente crises psicomotoras da infância com ponta-ondas lentas, nas encefalopatias epilépticas graves da infância, de tipo Lennox, e também em síndrome epiléptico degenerativo. Os efeitos colaterais, presentes em 11 pacientes, foram passageiros em 9. Precipitação de crises generalizadas tônico-clônicas ocorreu em dois pacientes; contudo há indícios que esse fenômeno é mais freqüente com outros derivados benzodiazepínicos. As modificações do EEG incluiram desaparecimento ou diminuição das descargas bilaterais e difusas, espontâneas e/ou desencadeadas pela estimulação luminosa intermitente, aparecimento de ritmos rápidos e difusos, persistência ou aparecimento de anormalidades focais lentas ou de tipo irritativo.Twenty two epileptic patients, with daily seizures (5 had typical absences, 3 had psychomotor seizures, 5 had myoclonic absences, one had intentional myoclonia and 8 had Lennox syndrome were treated with 7-nitro-5-(2chlorophenyl-3H-1,4 benzodiazepine-2(1H-one or Ro 5

  20. First-drug treatment failures in 42 Turkish children with idiopathic childhood occipital epilepsies.

    Science.gov (United States)

    Incecik, Faruk; Herguner, Ozlem M; Altunbasak, Sakir

    2015-01-01

    The early and late benign occipital epilepsies of childhood (BOEC) are described as two discrete electro-clinical syndromes, eponymously known as Panayiotopoulos and Gastaut syndromes. The purpose of this study was to identify predictors of failure to respond to the initial antiepileptic drug (AED). A total of 42 children with BOEC were enrolled. Predictive factors were analyzed by survival methods. Among the 42, 25 patients (59.5%) were boys and 17 (40.5%) were girls and the mean age at the seizure onset was 7.46 ± 2.65 years (4-14 years). Of the 42 patients, 34 (81.0%) were treated relatively successfully with the first AED treatment, and 8 (19.0%) were not responded initial AED treatment. There was no correlation between response to initial AED treatment and sex, consanguinity, epilepsy history of family, age of seizure onset, frequency of seizures, history of status epilepticus, duration of starting first treatment, findings on electroencephalogram. However, history of febrile seizure and type of BOEC were significantly associated with failure risk. Factors predicting failure to respond to the AED were history of febrile seizure and type of BOEC in children with BOEC.

  1. Treating seizures in Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Ng, Marcus C; Westover, M Brandon; Cole, Andrew J

    2014-01-01

    Seizures are known to occur in Creutzfeldt-Jakob disease (CJD). In the setting of a rapidly progressive condition with no effective therapy, determining appropriate treatment for seizures can be difficult if clinical morbidity is not obvious yet the electroencephalogram (EEG) demonstrates a worrisome pattern such as status epilepticus. Herein, we present the case of a 39-year-old man with CJD and electrographic seizures, discuss how this case challenges conventional definitions of seizures, and discuss a rational approach toward treatment. Coincidentally, our case is the first report of CJD in a patient with Stickler syndrome.

  2. Genetic and phenotypic heterogeneity suggest therapeutic implications in SCN2A-related disorders.

    Science.gov (United States)

    Wolff, Markus; Johannesen, Katrine M; Hedrich, Ulrike B S; Masnada, Silvia; Rubboli, Guido; Gardella, Elena; Lesca, Gaetan; Ville, Dorothée; Milh, Mathieu; Villard, Laurent; Afenjar, Alexandra; Chantot-Bastaraud, Sandra; Mignot, Cyril; Lardennois, Caroline; Nava, Caroline; Schwarz, Niklas; Gérard, Marion; Perrin, Laurence; Doummar, Diane; Auvin, Stéphane; Miranda, Maria J; Hempel, Maja; Brilstra, Eva; Knoers, Nine; Verbeek, Nienke; van Kempen, Marjan; Braun, Kees P; Mancini, Grazia; Biskup, Saskia; Hörtnagel, Konstanze; Döcker, Miriam; Bast, Thomas; Loddenkemper, Tobias; Wong-Kisiel, Lily; Baumeister, Friedrich M; Fazeli, Walid; Striano, Pasquale; Dilena, Robertino; Fontana, Elena; Zara, Federico; Kurlemann, Gerhard; Klepper, Joerg; Thoene, Jess G; Arndt, Daniel H; Deconinck, Nicolas; Schmitt-Mechelke, Thomas; Maier, Oliver; Muhle, Hiltrud; Wical, Beverly; Finetti, Claudio; Brückner, Reinhard; Pietz, Joachim; Golla, Günther; Jillella, Dinesh; Linnet, Karen M; Charles, Perrine; Moog, Ute; Õiglane-Shlik, Eve; Mantovani, John F; Park, Kristen; Deprez, Marie; Lederer, Damien; Mary, Sandrine; Scalais, Emmanuel; Selim, Laila; Van Coster, Rudy; Lagae, Lieven; Nikanorova, Marina; Hjalgrim, Helle; Korenke, G Christoph; Trivisano, Marina; Specchio, Nicola; Ceulemans, Berten; Dorn, Thomas; Helbig, Katherine L; Hardies, Katia; Stamberger, Hannah; de Jonghe, Peter; Weckhuysen, Sarah; Lemke, Johannes R; Krägeloh-Mann, Ingeborg; Helbig, Ingo; Kluger, Gerhard; Lerche, Holger; Møller, Rikke S

    2017-05-01

    Mutations in SCN2A, a gene encoding the voltage-gated sodium channel Nav1.2, have been associated with a spectrum of epilepsies and neurodevelopmental disorders. Here, we report the phenotypes of 71 patients and review 130 previously reported patients. We found that (i) encephalopathies with infantile/childhood onset epilepsies (≥3 months of age) occur almost as often as those with an early infantile onset (<3 months), and are thus more frequent than previously reported; (ii) distinct phenotypes can be seen within the late onset group, including myoclonic-atonic epilepsy (two patients), Lennox-Gastaut not emerging from West syndrome (two patients), and focal epilepsies with an electrical status epilepticus during slow sleep-like EEG pattern (six patients); and (iii) West syndrome constitutes a common phenotype with a major recurring mutation (p.Arg853Gln: two new and four previously reported children). Other known phenotypes include Ohtahara syndrome, epilepsy of infancy with migrating focal seizures, and intellectual disability or autism without epilepsy. To assess the response to antiepileptic therapy, we retrospectively reviewed the treatment regimen and the course of the epilepsy in 66 patients for which well-documented medical information was available. We find that the use of sodium channel blockers was often associated with clinically relevant seizure reduction or seizure freedom in children with early infantile epilepsies (<3 months), whereas other antiepileptic drugs were less effective. In contrast, sodium channel blockers were rarely effective in epilepsies with later onset (≥3 months) and sometimes induced seizure worsening. Regarding the genetic findings, truncating mutations were exclusively seen in patients with late onset epilepsies and lack of response to sodium channel blockers. Functional characterization of four selected missense mutations using whole cell patch-clamping in tsA201 cells-together with data from the literature-suggest that

  3. Why do seizures occur when they do? Situations perceived to be associated with increased or decreased seizure likelihood in people with epilepsy and intellectual disability.

    Science.gov (United States)

    Illingworth, Josephine L; Watson, Peter; Ring, Howard

    2014-10-01

    Seizure precipitants are commonly reported in the general population of people with epilepsy. However, there has been little research in this area in people with epilepsy and intellectual disability (ID). We conducted a survey of the situations associated with increased or decreased seizure likelihood in this population. The aim of the research was to identify situations of increased seizure likelihood (SISLs) and situations of decreased seizure likelihood (SDSLs) reported by carers of people with an ID and epilepsy. Three study groups were investigated: two groups comprising individuals with ID associated with a specific genetic diagnosis - Rett syndrome or fragile X syndrome - and one group consisting of individuals with a range of other etiologies. Responses relating to 100 people were received: 44 relating to people with Rett syndrome, 25 to people with fragile X syndrome, and 31 to people whose ID had some other etiologies. Ninety-eight percent of the respondents reported at least one SISL, and 60% reported at least one SDSL. Having more seizure types and greater seizure frequency were associated with a higher number of SISLs reported. The most commonly reported SISLs and SDSLs for each of the three groups are presented. The most common SISL overall was illness, which was reported as an SISL by 71% of the respondents. There was less consensus with regard to SDSLs. These findings provide a greater understanding of when seizures occur in those with ID and epilepsy, with possible implications for adjunctive behavioral management of seizures in those with treatment-refractory epilepsy. Copyright © 2014. Published by Elsevier Inc.

  4. First-drug treatment failures in 42 Turkish children with idiopathic childhood occipital epilepsies

    Directory of Open Access Journals (Sweden)

    Faruk Incecik

    2015-01-01

    Full Text Available Background: The early and late benign occipital epilepsies of childhood (BOEC are described as two discrete electro-clinical syndromes, eponymously known as Panayiotopoulos and Gastaut syndromes. The purpose of this study was to identify predictors of failure to respond to the initial antiepileptic drug (AED. Materials and Methods: A total of 42 children with BOEC were enrolled. Predictive factors were analyzed by survival methods. Results: Among the 42, 25 patients (59.5% were boys and 17 (40.5% were girls and the mean age at the seizure onset was 7.46 ± 2.65 years (4-14 years. Of the 42 patients, 34 (81.0% were treated relatively successfully with the first AED treatment, and 8 (19.0% were not responded initial AED treatment. There was no correlation between response to initial AED treatment and sex, consanguinity, epilepsy history of family, age of seizure onset, frequency of seizures, history of status epilepticus, duration of starting first treatment, findings on electroencephalogram. However, history of febrile seizure and type of BOEC were significantly associated with failure risk. Conclusions: Factors predicting failure to respond to the AED were history of febrile seizure and type of BOEC in children with BOEC.

  5. Clobazam and its active metabolite N-desmethylclobazam display significantly greater affinities for α₂- versus α₁-GABA(A-receptor complexes.

    Directory of Open Access Journals (Sweden)

    Henrik Sindal Jensen

    Full Text Available Clobazam (CLB, a 1,5-benzodiazepine (BZD, was FDA-approved in October 2011 for the adjunctive treatment of seizures associated with Lennox-Gastaut syndrome (LGS in patients 2 years and older. BZDs exert various CNS effects through allosteric modulation of GABAA receptors. The structurally distinct, 1,4-BZD clonazepam (CLN is also approved to treat LGS. The precise mechanisms of action and clinical efficacy of both are unknown. Data show that the GABAA α₁-subunit-selective compound zolpidem [ZOL] exhibits hypnotic/sedative effects. Conversely, data from knock-in mice carrying BZD binding site mutations suggest that the α₂ subunit mediates anticonvulsant effects, without sedative actions. Hence, the specific pattern of interactions across the GABAA receptor complexes of BZDs might be reflected in their clinical efficacies and adverse effect profiles. In this study, GABAA-receptor binding affinities of CLB, N-desmethylclobazam (N-CLB, the major metabolite of CLB, CLN, and ZOL were characterized with native receptors from rat-brain homogenates and on cloned receptors from HEK293 cells transfected with combinations of α (α₁, α₂, α₃, or α₅, β₂, and γ₂ subtypes. Our results demonstrate that CLB and N-CLB have significantly greater binding affinities for α₂- vs. α₁-receptor complexes, a difference not observed for CLN, for which no distinction between α₂ and α₁ receptors was observed. Our experiments with ZOL confirmed the high preference for α₁ receptors. These results provide potential clues to a new understanding of the pharmacologic modes of action of CLB and N-CLB.

  6. A survey of antiepileptic drug responses identifies drugs with potential efficacy for seizure control in Wolf-Hirschhorn syndrome.

    Science.gov (United States)

    Ho, Karen S; Markham, Leah M; Twede, Hope; Lortz, Amanda; Olson, Lenora M; Sheng, Xiaoming; Weng, Cindy; Wassman, E Robert; Newcomb, Tara; Wassman, E Robert; Carey, John C; Battaglia, Agatino

    2018-04-01

    Seizures are present in over 90% of infants and children with Wolf-Hirschhorn syndrome (WHS). When present, they significantly affect quality of life. The goal of this study was to use caregiver reports to describe the comparative efficacies of commonly used antiepileptic medications in a large population of individuals with WHS. A web-based, confidential caregiver survey was developed to capture seizure semiology and a chronologic record of seizure treatments as well as responses to each treatment. Adverse events for each drug were also cataloged. We received 141 complete survey responses (47% response rate) describing the seizures of individuals ranging in age from 4months to 61years (90 females: 51 males). Using the Early Childhood Epilepsy Severity Scale (E-Chess), WHS-associated seizures are demonstrably severe regardless of deletion size. The best-performing antiepileptic drugs (AEDs) for controlling seizures in this cohort were broad spectrum drugs clobazam, levetiracetam, and lamotrigine; whereas, the three commonly used carboxamide class drugs: carbamazepine, phenytoin, and oxcarbazepine, were reported to have little effect on, or even exacerbate, seizures. The carboxamide class drugs, along with phenobarbital and topiramate, were also associated with the highest rate of intolerance due to cooccurrence of adverse events. Levetiracetam, clobazam, and clonazepam demonstrated higher tolerability and comparatively less severe adverse events (Wilcoxon rank sum comparison between performance of levetiracetam and carboxamide class drugs gives a psyndromes which may have complex seizure etiologies. Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.

  7. A Neonate with persistent hypoglycemia and seizures.

    African Journals Online (AJOL)

    MBY

    disorder was diagnosed and managed with limited success as the episodes hydroglycemic seizures persisted. ... the presence of hyperinsulinemia as the cause of the hypoglycemic dependent seizures. Case Presentation. A three day old girl was admitted to the neonatal .... the Prader-Willi syndrome, has been reported.

  8. Prolonged QT Syndrome and Seizure Secondary to Alkaline Earth Metal Deficiency: A Case Report

    Directory of Open Access Journals (Sweden)

    A. McKinney

    2011-01-01

    Full Text Available Introduction. Alkaline earth metal deficiency is recognized as a cause of both seizure and long QT syndrome. Their deficiency can have significant repercussions on the function of cells, tissues, and organs of the body. An understanding of the role of electrolytes allows an appreciation of the significance of depleted levels on cell function. Case Report. A 65-year-old lady was admitted with symptoms of chest discomfort, vomiting, increased stoma output, and dizziness. Two days following admission she suffered a tonic-clonic seizure. ECG review demonstrated a prolonged QTc interval, raising the possibility of an underlying Torsades de Pointes as the precipitant. This was attributed to electrolyte disturbance arising as a result of multiple aetiologies. Discussion. This paper highlights the multisystem effects of electrolyte disturbance, with emphasis upon its role in precipitating cardiac arrhythmia and neurological symptoms.

  9. Epilepsy in fragile-X-syndrome mimicking panayiotopoulos syndrome: Description of three patients.

    Science.gov (United States)

    Bonanni, Paolo; Casellato, Susanna; Fabbro, Franco; Negrin, Susanna

    2017-10-01

    Fragile-X-syndrome is the most common cause of inherited intellectual disability. Epilepsy is reported to occur in 10-20% of individuals with Fragile-X-syndrome. A frequent seizure/electroencephalogram (EEG) pattern resembles that of benign rolandic epilepsy. We describe the clinical features, EEG findings and evolution in three patients affected by Fragile-X-syndrome and epilepsy mimicking Panayiotopoulos syndrome. Age at seizure onset was between 4 and about 7 years. Seizures pattern comprised a constellation of autonomic symptoms with unilateral deviation of the eyes and ictal syncope. Duration of the seizures could be brief or lengthy. Interictal EEGs revealed functional multifocal abnormalities. The evolution was benign in all patients with seizures remission before the age of 14. This observation expands the spectrum of benign epileptic phenotypes present in Fragile-X-syndrome and may be quite helpful in guiding anticonvulsant management and counseling families as to expectations regarding seizure remission. © 2017 Wiley Periodicals, Inc.

  10. Wolf-Hirschhorn (4p-) syndrome with West syndrome.

    Science.gov (United States)

    Motoi, Hirotaka; Okanishi, Tohru; Kanai, Sotaro; Yokota, Takuya; Yamazoe, Tomohiro; Nishimura, Mitsuyo; Fujimoto, Ayataka; Yamamoto, Takamichi; Enoki, Hideo

    2016-01-01

    Wolf-Hirschhorn syndrome (WHS) is a chromosome disorder (4p-syndrome) which is characterized by craniofacial features and epileptic seizures. Here, we report a case of WHS with West syndrome, in whom the seizures were refractory to several antiepileptic drugs but were responsive to the addition of lamotrigine. The patient had epileptic spasms at age seven months. The interictal electroencephalogram was hypsarrhythmic. After adding lamotrigine, seizures decreased remarkably, and spasms disappeared. We have identified and described the very rare case of a girl with WHS who also developed West syndrome. In this case, adding lamotrigine to her medications effectively treated the spasms.

  11. Emotion-induced myoclonic absence-like seizures in a patient with inv-dup(15) syndrome: a clinical, EEG, and molecular genetic study.

    Science.gov (United States)

    Aguglia, U; Le Piane, E; Gambardella, A; Messina, D; Russo, C; Sirchia, S M; Porta, G; Quattrone, A

    1999-09-01

    We have described a clinical EEG and molecular genetic study of a 9-year-old boy with inv-dup(15) syndrome in whom seizures were induced by emotionally gratifying stimuli. The reflex seizures began 5-20 s after the onset of repeated cheek-kissing from his mother or after viewing of pleasant or funny events. They were characterized by bilateral discharges involving mainly the temporal regions and evolving into myoclonic absence-like seizures. Nonemotional stimuli, such as a pinch, sucking or rubbing his cheeks, or the sound of the kiss alone, failed to provoke seizures. The seizures were resistant to antiepileptic (AED) treatments. Molecular genetic investigations revealed a correct methylation pattern of the chromosomes 15, and three copies (two maternal and one paternal) of the segment 15q11-q13, including the GABRb3 gene. We hypothesize that an overexpression of cerebral gamma-aminobutyric acid (GABA)-mediated inhibition accounts for the severe epilepsy that we observed in this patient.

  12. Fyodor Dostoevsky and his falling sickness: a critical analysis of seizure semiology.

    Science.gov (United States)

    Seneviratne, Udaya

    2010-08-01

    Fyodor Dostoevsky is a great Russian writer who had epilepsy. As a consequence, there are many references to seizure-related phenomena in his work. His epilepsy syndrome has been a focus of debate. The goal of this article is to delineate his epilepsy syndrome based on a semiological description of seizures, which could be considered one of the most reliable pieces of circumstantial evidence available. It was hypothesized that seizure-related descriptions in his books were based on his own personal experience. The semiology of seizures and related phenomena was compiled from Dostoevsky's own work, his letters to family and friends, and reminiscences of his wife and friend. Those descriptions were analyzed in detail to elicit localizing and lateralizing features of seizures. On the basis of this evidence, it was postulated that Dostoevsky had a partial epilepsy syndrome most probably arising from the dominant temporal lobe. Crown Copyright 2010. Published by Elsevier Inc. All rights reserved.

  13. Oxaliplatin-Induced Tonic-Clonic Seizures

    Directory of Open Access Journals (Sweden)

    Ahmad K. Rahal

    2015-01-01

    Full Text Available Oxaliplatin is a common chemotherapy drug used for colon and gastric cancers. Common side effects are peripheral neuropathy, hematological toxicity, and allergic reactions. A rare side effect is seizures which are usually associated with posterior reversible leukoencephalopathy syndrome (PRES. A 50-year-old male patient presented with severe abdominal pain. CT scan of the abdomen showed acute appendicitis. Appendectomy was done and pathology showed mixed adenoneuroendocrine carcinoma. Adjuvant chemotherapy was started with Folinic acid, Fluorouracil, and Oxaliplatin (FOLFOX. During the third cycle of FOLFOX, the patient developed tonic-clonic seizures. Laboratory workup was within normal limits. EEG and MRI of the brain showed no acute abnormality. The patient was rechallenged with FOLFOX but he had tonic-clonic seizures for the second time. His chemotherapy regimen was switched to Folinic acid, Fluorouracil, and Irinotecan (FOLFIRI. After 5 cycles of FOLFIRI, the patient did not develop any seizures, making Oxaliplatin the most likely culprit for his seizures. Oxaliplatin-induced seizures rarely occur in the absence of PRES. One case report has been described in the literature. We present a rare case of tonic-clonic seizures in a patient receiving Oxaliplatin in the absence of PRES.

  14. Epileptic Seizures Versus Syncope: Pathophysiology and Clinical Approach

    Directory of Open Access Journals (Sweden)

    Marios Charalambous

    2017-02-01

    Full Text Available Generalised epileptic seizures and syncope are two syndromes with similar clinical manifestation and their differentiation can be quite challenging. The aim of this review is to use an evidence-based approach in differentiating these two syndromes through the comprehension of the pathophysiological mechanisms involved and their clinical signs. Both syndromes affect regions of the forebrain and consciousness level, although, different mechanisms are involved. Syncope is a paroxysmal event secondary to a short-term decrease in cerebral perfusion, oxygenation or essential nutrients delivery. Generalised epileptic seizure activity is defined as the clinical manifestation of transient paroxysmal disturbances in brain function secondary to an imbalance between excitatory and inhibitory neurotransmitters. Clinical criteria, including precipitating events, clinical signs preceding, during and following the episodes and event duration, can be used to differentiate the two syndromes. Although these criteria might be useful for the practitioner, definite conclusions should be precluded due to the lack of original research articles and weak evidence on this specific field.Application: The review might be a useful tool for the general practitioner and clinical scientist as it will aid towards the differentiation of two syndromes, i.e. generalised epileptic seizures and syncope, with similar clinical presentation.

  15. Shaken baby syndrome manifesting as infantile spasms seizure type

    Directory of Open Access Journals (Sweden)

    Der-Shiun Wang

    2014-01-01

    Full Text Available The diagnosis of child maltreatment leading to head injury is challenging. Here, we present the case of a 3-month-old female infant who presented with focal seizures that lasted for several minutes. After admission, she began to show intermittent clusters of head nods, irritable crying, arching, writhing, stiffening, and jerking of both arms. These results and electroencephalography findings were attributed as the diagnosis of infantile spasms (IS. Brain computed tomography and magnetic resonance imaging (MRI revealed the presence of chronic subdural hematoma mixed with acute ischemic injuries. Examination of the eye fundus confirmed the presence of retinal hemorrhage. Therefore, all evidence pointed to a diagnosis of shaken baby syndrome (SBS. Based on this case, we suggest that physicians should consider a diagnosis of SBS for children with new-onset IS and that should be evaluated, diagnosed, and treated as promptly as possible.

  16. Unusual 4p16.3 deletions suggest an additional chromosome region for the Wolf-Hirschhorn syndrome-associated seizures disorder

    NARCIS (Netherlands)

    Zollino, M.; Orteschi, D.; Ruiter, M.; Pfundt, R.P.; Steindl, K.; Cafiero, C.; Ricciardi, S.; Contaldo, I.; Chieffo, D.; Ranalli, D.; Acquafondata, C.; Murdolo, M.; Marangi, G.; Asaro, A.; Battaglia, D.

    2014-01-01

    OBJECTIVE: Seizure disorder is one of the most relevant clinical manifestations in Wolf-Hirschhorn syndrome (WHS) and it acts as independent prognostic factor for the severity of intellectual disability (ID). LETM1, encoding a mitochondrial protein playing a role in K(+) /H(+) exchange and in Ca(2+)

  17. Basal Ganglia Calcification with Tetanic Seizure Suggest Mitochondrial Disorder.

    Science.gov (United States)

    Finsterer, Josef; Enzelsberger, Barbara; Bastowansky, Adam

    2017-04-09

    BACKGROUND Basal ganglia calcification (BGC) is a rare sporadic or hereditary central nervous system (CNS) abnormality, characterized by symmetric or asymmetric calcification of the basal ganglia. CASE REPORT We report the case of a 65-year-old Gypsy female who was admitted for a tetanic seizure, and who had a history of polyneuropathy, restless-leg syndrome, retinopathy, diabetes, hyperlipidemia, osteoporosis with consecutive hyperkyphosis, cervicalgia, lumbalgia, struma nodosa requiring thyroidectomy and consecutive hypothyroidism, adipositas, resection of a vocal chord polyp, arterial hypertension, coronary heart disease, atheromatosis of the aorta, peripheral artery disease, chronic obstructive pulmonary disease, steatosis hepatis, mild renal insufficiency, long-term hypocalcemia, hyperphosphatemia, impingement syndrome, spondylarthrosis of the lumbar spine, and hysterectomy. History and clinical presentation suggested a mitochondrial defect which also manifested as hypoparathyroidism or Fanconi syndrome resulting in BGC. After substitution of calcium, no further tetanic seizures occurred. CONCLUSIONS Patients with BGC should be investigated for a mitochondrial disorder. A mitochondrial disorder may also manifest as tetanic seizure.

  18. Seizure disorders in 43 cattle.

    Science.gov (United States)

    D'Angelo, A; Bellino, C; Bertone, I; Cagnotti, G; Iulini, B; Miniscalco, B; Casalone, C; Gianella, P; Cagnasso, A

    2015-01-01

    Large animals have a relatively high seizure threshold, and in most cases seizures are acquired. No published case series have described this syndrome in cattle. To describe clinical findings and outcomes in cattle referred to the Veterinary Teaching Hospital of the University of Turin (Italy) because of seizures. Client-owned cattle with documented evidence of seizures. Medical records of cattle with episodes of seizures reported between January 2002 and February 2014 were reviewed. Evidence of seizures was identified based on the evaluation of seizure episodes by the referring veterinarian or 1 of the authors. Animals were recruited if physical and neurologic examinations were performed and if diagnostic laboratory test results were available. Forty-three of 49 cases fulfilled the inclusion criteria. The mean age was 8 months. Thirty-one animals were male and 12 were female. Piedmontese breed accounted for 39/43 (91%) animals. Seizures were etiologically classified as reactive in 30 patients (70%) and secondary or structural in 13 (30%). Thirty-six animals survived, 2 died naturally, and 5 were euthanized for reasons of animal welfare. The definitive cause of reactive seizures was diagnosed as hypomagnesemia (n = 2), hypocalcemia (n = 12), and hypomagnesemia-hypocalcemia (n = 16). The cause of structural seizures was diagnosed as cerebrocortical necrosis (n = 8), inflammatory diseases (n = 4), and lead (Pb) intoxication (n = 1). The study results indicate that seizures largely are reported in beef cattle and that the cause can be identified and successfully treated in most cases. Copyright © 2015 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

  19. Novel PIGT Variant in Two Brothers: Expansion of the Multiple Congenital Anomalies-Hypotonia Seizures Syndrome 3 Phenotype

    OpenAIRE

    Skauli, Nadia; Wallace, Sean; Chiang, Samuel C. C.; Bar?y, Tuva; Holmgren, Asbj?rn; Stray-Pedersen, Asbj?rg; Bryceson, Yenan T.; Str?mme, Petter; Frengen, Eirik; Misceo, Doriana

    2016-01-01

    Biallelic PIGT variants were previously reported in seven patients from three families with Multiple Congenital Anomalies-Hypotonia Seizures Syndrome 3 (MCAHS3), characterized by epileptic encephalopathy, hypotonia, global developmental delay/intellectual disability, cerebral and cerebellar atrophy, craniofacial dysmorphisms, and skeletal, ophthalmological, cardiac, and genitourinary abnormalities. We report a novel homozygous PIGT missense variant c.1079G>T (p.Gly360Val) in two brothers w...

  20. Seizure Associated Takotsubo Syndrome: A Rare Combination

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    Htay Htay Kyi

    2017-01-01

    Full Text Available Takotsubo cardiomyopathy (TC is increasingly recognized in neurocritical care population especially in postmenopausal females. We are presenting a 61-year-old African American female with past medical history of epilepsy, bipolar disorder, and hypertension who presented with multiple episodes of seizures due to noncompliance with antiepileptic medications. She was on telemetry which showed ST alarm. Electrocardiogram (ECG was ordered and showed ST elevation in anterolateral leads and troponins were positive. Subsequently Takotsubo cardiomyopathy was diagnosed by left ventriculography findings and absence of angiographic evidence of obstructive coronary artery disease. Echocardiogram showed apical hypokinesia, ejection fraction of 40%, and systolic anterior motion of mitral valve with hyperdynamic left ventricle, in the absence of intracoronary thrombus formation in the angiogram. Electroencephalography showed evidence of generalized tonic-clonic seizure. She was treated with supportive therapy. This case illustrates importance of ECG in all patients with seizure irrespective of cardiac symptoms as TC could be the cause of Sudden Unexpected Death in Epilepsy (SUDEP and may be underdiagnosed and so undertreated.

  1. Photogenic partial seizures.

    Science.gov (United States)

    Hennessy, M J; Binnie, C D

    2000-01-01

    To establish the incidence and symptoms of partial seizures in a cohort of patients investigated on account of known sensitivity to intermittent photic stimulation and/or precipitation of seizures by environmental visual stimuli such as television (TV) screens or computer monitors. We report 43 consecutive patients with epilepsy, who had exhibited a significant EEG photoparoxysmal response or who had seizures precipitated by environmental visual stimuli and underwent detailed assessment of their photosensitivity in the EEG laboratory, during which all were questioned concerning their ictal symptoms. All patients were considered on clinical grounds to have an idiopathic epilepsy syndrome. Twenty-eight (65%) patients reported visually precipitated attacks occurring initially with maintained consciousness, in some instances evolving to a period of confusion or to a secondarily generalized seizure. Visual symptoms were most commonly reported and included positive symptoms such as coloured circles or spots, but also blindness and subjective symptoms such as "eyes going funny." Other symptoms described included nonspecific cephalic sensations, deja-vu, auditory hallucinations, nausea, and vomiting. No patient reported any clear spontaneous partial seizures, and there were no grounds for supposing that any had partial epilepsy excepting the ictal phenomenology of some or all of the visually induced attacks. These findings provide clinical support for the physiological studies that indicate that the trigger mechanism for human photosensitivity involves binocularly innervated cells located in the visual cortex. Thus the visual cortex is the seat of the primary epileptogenic process, and the photically triggered discharges and seizures may be regarded as partial with secondary generalization.

  2. Epilepsy and PET

    International Nuclear Information System (INIS)

    Shimizu, Hiroyuki; Ishijima, Buichi

    1984-01-01

    The glucose metabolism of interictal epileptic foci in human brains were analyzed by positron emission tomography. The seizure patterns of 29 epileptic patients were as follows; complex partial 13 cases, elementary partial 9 cases, and generalized 7 cases. 11 C was produced by a JSW medical cyclotron BC105 and was randomly tagged to glucose prepared by photosynthesis. Data sampling by PET was started 15 minutes after peroral administration of 11 C-glucose to the patients. Three slices with 1.75 cm distance were obtained by a single scanning. In temporal lobe epilepsy, three slices were selected as 2.0 cm, 3.75 cm and 5.5 cm above orbitomeatal line. The basal ganglia were scanned 4.5 -- 5.0 cm and the motor and sensory strips were 5.0 -- 9.0 cm above OML. The glucose metabolic rate was expressed with color scales and qualitatively estimated. The results disclosed an obvious hypometabolic zone around a focus area in 22 cases (76%) out of the 29 subjects. This hypometabolic zone was observed in 12 cases (92%) of 13 complex partial, 9 cases (78%) of 9 elementary partial, and 3 cases (43%) of 7 generalized seizure patterns. In temporal lobe epilepsy, the location of the hypometabolic zone was different according to the clinical symptoms. The patients with automatism, pseudoabsence, autonomic, and emotional symptoms had its foci in the mesial portion of the temporal lobe. On the other hand, the patients with psychical seizure revealed its low metabolic area in the lateral temporal cortex. In the elementary partial epilepsy, the hypoactive zones were observed in the motor, sensory, and visual cortical area in accordance with the clinical symptoms. Very interestingly, an explicit cortical focus was discovered in two cases of the generalized epilepsy. In these cases the mechanism of secondary generalization was supposed to proceed in the expression of their clinical symptoms. In one Lennox-Gastaut case, a unilateral temporal lobe was involved as the seizure focus. (J.P.N.)

  3. Out-of-body experiences associated with seizures

    Directory of Open Access Journals (Sweden)

    Bruce eGreyson

    2014-02-01

    Full Text Available Alterations of consciousness are critical factors in the diagnosis of epileptic seizures. With these alterations in consciousness, some persons report sensations of separating from the physical body, experiences that may in rare cases resemble spontaneous out-of-body experiences. This study was designed to identify and characterize these out-of-body-like subjective experiences associated with seizure activity. 55% of the patients in this study recalled some subjective experience in association with their seizures. Among our sample of 100 patients, 7 reported out-of-body experiences associated with their seizures. We found no differentiating traits that were associated with patients’ reports of out-of-body experiences, in terms of either demographics; medical history, including age of onset and duration of seizure disorder, and seizure frequency; seizure characteristics, including localization, lateralization, etiology, and type of seizure, and epilepsy syndrome; or ability to recall any subjective experiences associated with their seizures. Reporting out-of-body experiences in association with seizures did not affect epilepsy-related quality of life. It should be noted that even in those patients who report out-of-body experiences, such sensations are extremely rare events that do not occur routinely with their seizures. Most patients who reported out-of-body experiences described one or two experiences that occurred an indeterminate number of years ago, which precludes the possibility of associating the experience with the particular characteristics of that one seizure or with medications taken or other conditions at the time.

  4. Seizure drawings: insight into the self-image of children with epilepsy.

    Science.gov (United States)

    Stafstrom, Carl E; Havlena, Janice

    2003-02-01

    Epilepsy is a chronic disorder that is associated with numerous psychological challenges, especially in children. Drawings have been underutilized as a method to obtain insight into psychological issues in children with epilepsy. We asked 105 children with epilepsy, ages 5 to 18 years, to draw a picture of what it is like to have a seizure. Across ages and epilepsy syndromes, the drawings showed evidence of impaired self-concept, low self-esteem, and a sense of helplessness and vulnerability. Overall, the drawings of human figures were less developed than expected for chronological age. In some drawings, indicators of underlying depression were found. When considered by epilepsy syndrome or seizure type, some specific artistic features were noted. Children with simple partial (motor) seizures drew distorted body parts, especially limbs. Those with complex partial seizures depicted sensory symptoms and mental status changes such as confusion. Children with generalized tonic-clonic seizures showed shaking extremities. Drawings by children with absence seizures illustrated mainly staring. In conclusion, drawings are a powerful method to examine the self-concept of children with epilepsy and gain insight into their feelings about themselves and their world.

  5. Seizures as the first manifestation of chromosome 22q11.2 deletion syndrome in a 40-year old man: a case report

    OpenAIRE

    Tonelli, Adriano R; Kosuri, Kalyan; Wei, Sainan; Chick, Davoren

    2007-01-01

    Abstract Background The microdeletion of chromosome 22q11.2 is the most common human deletion syndrome. It typically presents early in life and is rarely considered in adult patients. As part of the manifestations of this condition, patients can have parathyroid glandular involvement ranging from hypocalcemic hypoparathyroidism to normocalcemia with normal parathryroid hormone levels. The first manifestation of the syndrome might be seizures due to profound hypocalcemia. Case presentation A 4...

  6. Subacute encephalopathy with epileptic seizures in alcoholism (SESA): case report.

    Science.gov (United States)

    Otto, F G; Kozian, R

    2001-10-01

    The case of a 66-year-old patient is reported in view of the rarity of his condition: a case of subacute encephalopathy with seizures in alcoholics (SESA syndrome), described first in 1981 by Niedermeyer, et al. Wernicke-type aphasia, epileptic seizures (generalized tonic-clonic) and PLEDs EEG pattern dominated the neurological picture, in addition to hepatomegaly and rhabdomyolysis. This condition differs from all other known CNS complications in chronic alcoholism and is withdrawal-independent. It is prognostically favorable as far as the syndrome as such is concerned.

  7. Why are seizures rare in rapid eye movement sleep? Review of the frequency of seizures in different sleep stages.

    Science.gov (United States)

    Ng, Marcus; Pavlova, Milena

    2013-01-01

    Since the formal characterization of sleep stages, there have been reports that seizures may preferentially occur in certain phases of sleep. Through ascending cholinergic connections from the brainstem, rapid eye movement (REM) sleep is physiologically characterized by low voltage fast activity on the electroencephalogram, REMs, and muscle atonia. Multiple independent studies confirm that, in REM sleep, there is a strikingly low proportion of seizures (~1% or less). We review a total of 42 distinct conventional and intracranial studies in the literature which comprised a net of 1458 patients. Indexed to duration, we found that REM sleep was the most protective stage of sleep against focal seizures, generalized seizures, focal interictal discharges, and two particular epilepsy syndromes. REM sleep had an additional protective effect compared to wakefulness with an average 7.83 times fewer focal seizures, 3.25 times fewer generalized seizures, and 1.11 times fewer focal interictal discharges. In further studies REM sleep has also demonstrated utility in localizing epileptogenic foci with potential translation into postsurgical seizure freedom. Based on emerging connectivity data in sleep, we hypothesize that the influence of REM sleep on seizures is due to a desynchronized EEG pattern which reflects important connectivity differences unique to this sleep stage.

  8. Complex Regional Pain Syndrome Revived by Epileptic Seizure Then Disappeared Soon during Treatment with Regional Intravenous Nerve Blockade: A Case Report

    Directory of Open Access Journals (Sweden)

    Masahiko Sumitani

    2011-01-01

    Full Text Available We present a case of complex regional pain syndrome (CRPS, in which symptoms, including burning pain and severe allodynia, were alleviated by using a regional intravenous nerve blockade (Bier block combined with physiotherapy, but reappeared following an epileptic seizure. Symptoms disappeared again following control of epileptic discharges, as revealed by single-photon emission computed tomography (SPECT and electroencephalography (EEG results. Although systemic toxicity of a local anesthetic applied by Bier block was suspected as a cause of the first seizure, the patient did not present any other toxic symptoms, and seizures repeatedly occurred after Bier block cessation; the patient was then diagnosed as having temporal symptomatic epilepsy. This case suggests that symptoms of CRPS may be sustained by abnormal brain conditions, and our findings contribute to the understanding of how the central nervous system participates in maintaining pain and allodynia associated with CRPS.

  9. Management Of Post Stroke Seizures

    Directory of Open Access Journals (Sweden)

    Kavian Ghandehari

    2017-02-01

    Full Text Available The incidence of seizures in relation to stroke is 8.9%, with a frequency of 10.6 and 8.6% in haemorrhagic and ischaemic stroke, respectively. In subarachnoid haemorrhage the incidence is 8.5%. Due to the fact that infarcts are significantly more frequent than haemorrhages, seizures are mainly related to occlusive vascular disease of the brain. The general view is to consider stroke-related seizures as harmless complications in the course of a prolonged vascular disease involving the heart and brain. Seizures can be classified as those of early and those of late onset in a paradigm comparable to post-traumatic epilepsy, with an arbitrary dividing point of two weeks after the event. Most early-onset seizures occur during the first day after the stroke. Late-onset seizures occur three times more often than early-onset ones. A first late-onset epileptic event is most likely to take place between six months and two years after the stroke. However, up to 28% of patients develop their first seizure several years later. Simple partial seizures, with or without secondary generalisation, account for about 50% of total seizures, while complex partial spells, with or without secondary generalisation, and primary generalised tonic–clonic insults account for approximately 25% each. Status epilepticus occurs in 12% of stroke patients, but the recurrence rate after an initial status epilepticus is not higher than after a single seizure. Inhibitory seizures, mimicking transient ischaemic attacks, are observed in 7.1% of cases. The only clinical predictor of late-onset seizures is the initial presentation of partial anterior circulation syndrome due to a territorial infarct. Patients with total anterior circulation syndrome have less chance of developing epileptic spells, not only due to their shorter life expectancy but also due to the fact that the large infarcts are sharply demarcated in these patients. The optimal timing and type of antiepileptic drug

  10. Seizure-related factors and non-verbal intelligence in children with epilepsy. A population-based study from Western Norway.

    Science.gov (United States)

    Høie, B; Mykletun, A; Sommerfelt, K; Bjørnaes, H; Skeidsvoll, H; Waaler, P E

    2005-06-01

    To study the relationship between seizure-related factors, non-verbal intelligence, and socio-economic status (SES) in a population-based sample of children with epilepsy. The latest ILAE International classifications of epileptic seizures and syndromes were used to classify seizure types and epileptic syndromes in all 6-12 year old children (N=198) with epilepsy in Hordaland County, Norway. The children had neuropediatric and EEG examinations. Of the 198 patients, demographic characteristics were collected on 183 who participated in psychological studies including Raven matrices. 126 healthy controls underwent the same testing. Severe non-verbal problems (SNVP) were defined as a Raven score at or Raven percentile group, whereas controls were highly over-represented in the higher percentile groups. SNVP were present in 43% of children with epilepsy and 3% of controls. These problems were especially common in children with remote symptomatic epilepsy aetiology, undetermined epilepsy syndromes, myoclonic seizures, early seizure debut, high seizure frequency and in children with polytherapy. Seizure-related characteristics that were not usually associated with SNVP were idiopathic epilepsies, localization related (LR) cryptogenic epilepsies, absence and simple partial seizures, and a late debut of epilepsy. Adjusting for socio-economic status factors did not significantly change results. In childhood epilepsy various seizure-related factors, but not SES factors, were associated with the presence or absence of SNVP. Such deficits may be especially common in children with remote symptomatic epilepsy aetiology and in complex and therapy resistant epilepsies. Low frequencies of SNVP may be found in children with idiopathic and LR cryptogenic epilepsy syndromes, simple partial or absence seizures and a late epilepsy debut. Our study contributes to an overall picture of cognitive function and its relation to central seizure characteristics in a childhood epilepsy population

  11. A hypothesis regarding the molecular mechanism underlying dietary soy-induced effects on seizure propensity.

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    Cara Jean Westmark

    2014-09-01

    Full Text Available Numerous neurological disorders including fragile X syndrome, Down syndrome, autism and Alzheimer’s disease are comorbid with epilepsy. We have observed elevated seizure propensity in mouse models of these disorders dependent on diet. Specifically, soy-based diets exacerbate audiogenic-induced seizures in juvenile mice. We have also found potential associations between the consumption of soy-based infant formula and seizure incidence, epilepsy comorbidity and autism diagnostic scores in autistic children by retrospective analyses of medical record data. In total, these data suggest that consumption of high levels of soy protein during postnatal development may affect neuronal excitability. Herein, we present our theory regarding the molecular mechanism underlying soy-induced effects on seizure propensity. We hypothesize that soy phytoestrogens interfere with metabotropic glutamate receptor signaling through an estrogen receptor-dependent mechanism, which results in elevated production of key synaptic proteins and decreased seizure threshold.

  12. Experimental febrile seizures are precipitated by a hyperthermia-induced respiratory alkalosis.

    Science.gov (United States)

    Schuchmann, Sebastian; Schmitz, Dietmar; Rivera, Claudio; Vanhatalo, Sampsa; Salmen, Benedikt; Mackie, Ken; Sipilä, Sampsa T; Voipio, Juha; Kaila, Kai

    2006-07-01

    Febrile seizures are frequent during early childhood, and prolonged (complex) febrile seizures are associated with an increased susceptibility to temporal lobe epilepsy. The pathophysiological consequences of febrile seizures have been extensively studied in rat pups exposed to hyperthermia. The mechanisms that trigger these seizures are unknown, however. A rise in brain pH is known to enhance neuronal excitability. Here we show that hyperthermia causes respiratory alkalosis in the immature brain, with a threshold of 0.2-0.3 pH units for seizure induction. Suppressing alkalosis with 5% ambient CO2 abolished seizures within 20 s. CO2 also prevented two long-term effects of hyperthermic seizures in the hippocampus: the upregulation of the I(h) current and the upregulation of CB1 receptor expression. The effects of hyperthermia were closely mimicked by intraperitoneal injection of bicarbonate. Our work indicates a mechanism for triggering hyperthermic seizures and suggests new strategies in the research and therapy of fever-related epileptic syndromes.

  13. Prevalence of Pathogenic Copy Number Variation in Adults With Pediatric-Onset Epilepsy and Intellectual Disability.

    Science.gov (United States)

    Borlot, Felippe; Regan, Brigid M; Bassett, Anne S; Stavropoulos, D James; Andrade, Danielle M

    2017-11-01

    23.1 deletion, 9p24.3-p23 deletion, 10q11.22-q11.23 duplication, 12p13.33-13.2 duplication, 13q34 deletion, and 16p13.2 duplication. Five genes are of particular interest given their potential pathogenicity in the corresponding phenotypes and least tolerability to variation: ABAT, KIAA2022, COL4A1, CACNA1C, and SMARCA2. ABAT duplication was associated with Lennox-Gastaut syndrome and KIAA2022 deletion with Jeavons syndrome. The high prevalence of pathogenic CNVs in this study highlights the importance of microarray analysis in adults with unexplained childhood-onset epilepsy and intellectual disability. Additional studies and comparison with similar cases are required to evaluate the effects of deletions and duplications that overlap specific genes.

  14. DOORS syndrome: phenotype, genotype and comparison with Coffin-Siris syndrome.

    Science.gov (United States)

    Campeau, Philippe M; Hennekam, Raoul C

    2014-09-01

    DOORS syndrome (Deafness, Onychodystrophy, Osteodystrophy, mental Retardation, Seizures) is characterized mainly by sensorineural deafness, shortened terminal phalanges with small nails of hands and feet, intellectual deficiency, and seizures. Half of the patients with all clinical features have mutations in TBC1D24. We review here the manifestations of patients clinically diagnosed with DOORS syndrome. In this cohort of 32 families (36 patients) we detected 13 individuals from 10 families with TBC1D24 mutations. Subsequent whole exome sequencing in the cohort showed the same de novoSMARCB1 mutation (c.1130G>A), known to cause Coffin-Siris syndrome, in two patients. Distinguishing features include retinal anomalies, Dandy-Walker malformation, scoliosis, rocker bottom feet, respiratory difficulties and absence of seizures, and 2-oxoglutaric aciduria in the patients with the SMARCB1 mutation. We briefly discuss the heterogeneity of the DOORS syndrome phenotype and the differential diagnosis of this condition. © 2014 Wiley Periodicals, Inc.

  15. Postpartum seizures with posterior reversible encephalopathy syndrome following cesarean delivery for triplets

    Directory of Open Access Journals (Sweden)

    Anita Chhabra

    2014-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a recently described clinicoradiologic entity that is associated with several medical conditions like hypertensive encephalopathy and eclampsia. It presents with rapid onset of symptoms including headache, seizures, altered consciousness, and visual disturbance. It is often, but not always associated with high blood pressure. We present a case of 23-year-old patient, with unremarkable antenatal period, who developed convulsions in the immediate postpartum period following elective cesarean delivery of her triplets performed under regional anesthesia. The magnetic resonance imaging brain revealed vasogenic edema suggestive of PRES. She was managed with supportive treatment including mechanical ventilation in the intensive care unit. She recovered completely without neurological sequelae and discharged on the 8 th postoperative day. This case report highlights the importance of awareness, prompt diagnosis and treatment to improve the outcome in this potentially life-threatening, but reversible condition.

  16. On the nature of seizure dynamics

    Science.gov (United States)

    Stacey, William C.; Quilichini, Pascale P.; Ivanov, Anton I.

    2014-01-01

    Seizures can occur spontaneously and in a recurrent manner, which defines epilepsy; or they can be induced in a normal brain under a variety of conditions in most neuronal networks and species from flies to humans. Such universality raises the possibility that invariant properties exist that characterize seizures under different physiological and pathological conditions. Here, we analysed seizure dynamics mathematically and established a taxonomy of seizures based on first principles. For the predominant seizure class we developed a generic model called Epileptor. As an experimental model system, we used ictal-like discharges induced in vitro in mouse hippocampi. We show that only five state variables linked by integral-differential equations are sufficient to describe the onset, time course and offset of ictal-like discharges as well as their recurrence. Two state variables are responsible for generating rapid discharges (fast time scale), two for spike and wave events (intermediate time scale) and one for the control of time course, including the alternation between ‘normal’ and ictal periods (slow time scale). We propose that normal and ictal activities coexist: a separatrix acts as a barrier (or seizure threshold) between these states. Seizure onset is reached upon the collision of normal brain trajectories with the separatrix. We show theoretically and experimentally how a system can be pushed toward seizure under a wide variety of conditions. Within our experimental model, the onset and offset of ictal-like discharges are well-defined mathematical events: a saddle-node and homoclinic bifurcation, respectively. These bifurcations necessitate a baseline shift at onset and a logarithmic scaling of interspike intervals at offset. These predictions were not only confirmed in our in vitro experiments, but also for focal seizures recorded in different syndromes, brain regions and species (humans and zebrafish). Finally, we identified several possible biophysical

  17. Seizures

    Science.gov (United States)

    Secondary seizures; Reactive seizures; Seizure - secondary; Seizure - reactive; Convulsions ... or kidney failure Very high blood pressure ( malignant hypertension ) Venomous bites and stings ( snake bite ) Withdrawal from ...

  18. DOORS Syndrome: Phenotype, Genotype and Comparison With Coffin-Siris Syndrome

    NARCIS (Netherlands)

    Campeau, Philippe M.; Hennekam, Raoul C.; Aftimos, Salim; Banka, Siddharth; Begleiter, Michael L.; Bilo, Leonilda; Blair, Edward; Burrage, Lindsay C.; Liu, David S.; de Bie, Isabelle; Félix, Têmis Maria; Giltay, Jacques C.; Gibbs, Richard A.; Giuliano, Fabienne; Hadzsiev, Kinga; Hori, Mutsuki; Kariminejad, Ariana; Kayserili, Hülya; Kerr, Bronwyn; Lee, Brendan H.; Lu, James T.; Male, Alison; Meenakshi, Girish; Mey, Antje; Murray, Mitzi L.; Nair, Lal D. V.; Nampoothiri, Sheela; Newman, William G.; Peluso, Silvio; Peters, Heidi; Powell, R.; Repetto, Gabriela M.; Rump, Patrick; Santos-Simarro, Fernando; Stewart, Fiona; van Bever, Yolande; van den Ende, Jenneke; Wieczorek, Dagmar; Wisniewska, Marzena; Sisodiya, Sanjay M.

    2014-01-01

    DOORS syndrome (Deafness, Onychodystrophy, Osteodystrophy, mental Retardation, Seizures) is characterized mainly by sensorineural deafness, shortened terminal phalanges with small nails of hands and feet, intellectual deficiency, and seizures. Half of the patients with all clinical features have

  19. Anticonvulsant Efficacy in Sturge-Weber Syndrome

    Science.gov (United States)

    Kaplan, Emma H.; Kossoff, Eric H.; Bachur, Catherine D.; Gholston, Milton; Hahn, Jihoon; Widlus, Matthew; Comi, Anne M.

    2016-01-01

    OBJECTIVE We analyzed individuals with epilepsy due to Sturge-Weber syndrome to determine which anticonvulsants provided optimal seizure control and which resulted in the fewest side effects. METHODS One-hundred-eight records from a single center were retrospectively analyzed for Sturge-Weber syndrome brain involvement, epilepsy, Sturge-Weber syndrome neuroscores, and currently used anticonvulsants. RESULTS Of the fourteen anticonvulsants that had been employed, the most often used agents were oxcarbazepine or carbamazepine, and levetiracetam. Individuals whose seizures at the most recent visit were fully controlled (seizure-free) for 6 months or longer were more likely to have ever tried, or currently used, oxcarbazepine or carbamazepine than those with uncontrolled seizures. Thirty-nine of 69 individuals (56.5%) were seizure-free with oxcarbazepine or carbamazepine history versus 11 of 35 individuals (31.4%) who had not taken these agents (P anticonvulsants versus 12 of 42 (28.6%) not taking them (P < 0.01). Patients with seizure control for 6 months or longer were less likely to have ever tried, or to currently be taking, levetiracetam than those without control. Sixteen of 56 individuals (28.6%) were seizure-free with levetiracetam history versus 34 of 48 (70.8%) without it (P < 0.001); 14 of 43 individuals (32.6%) were seizure-free and currently taking levetiracetam versus 36 of 61 (59.0%) not taking it (P < 0.01). When topiramate was added as second-line medication, five of nine patients (55.6%) experienced decreased seizure severity, and worsening of glaucoma was not reported. CONCLUSIONS Carbamazepine and oxcarbazepine were associated with better seizure control than levetiracetam in this Sturge-Weber syndrome cohort and so may be preferred as the initial therapy. When used as adjunctive therapy, topiramate was effective in this limited analysis without a clear increased incidence of glaucoma. PMID:26997037

  20. New onset epilepsy in Prader-Willi syndrome: semiology and literature review.

    Science.gov (United States)

    Benson, Leslie A; Maski, Kiran P; Kothare, Sanjeev V; Bourgeois, Blaise F

    2010-10-01

    Prader-Willi syndrome is a chromosomal disorder caused by absence of expression of the paternal active genes in the 15q11∼q13 chromosome region; it is associated with an increased incidence of epilepsy and narcolepsy. Presented here is the case of a 2.5-year-old boy with Prader-Willi syndrome and a history of neonatal superior sagittal sinus thrombosis with new onset of atonic seizures with electrographic onset from the parasagittal region. It is postulated that microscarring from neonatal venous sinus thrombosis, history of febrile seizures, and Prader-Willi syndrome are factors predisposing him to epilepsy. The importance of video electroencephalography with electromyography electrodes is emphasized for Prader-Willi syndrome patients with drop episodes, to differentiate cataplexy from seizures. This being a novel report of a Prader-Willi syndrome patient with atonic seizures, the literature on seizure semiology among patients with Prader-Willi syndrome is reviewed. Copyright © 2010 Elsevier Inc. All rights reserved.

  1. Stability of extemporaneously prepared rufinamide oral suspensions.

    Science.gov (United States)

    Hutchinson, David J; Liou, Yayin; Best, Robert; Zhao, Fang

    2010-03-01

    Rufinamide is an oral antiepileptic drug indicated for adjunctive therapy in treating generalized seizures associated with Lennox-Gastaut syndrome. Currently, rufinamide is available as 200-mg and 400-mg tablets. A liquid dosage form does not exist at the present time. Lack of a suspension formulation may present an administration problem for many children and adults who are unable to swallow tablets. The availability of a liquid dosage form will provide an easy and accurate way to measure and administer the medication. To determine the stability of both sugar-containing and sugar-free rufinamide suspensions over a 90-day period. A suspension of rufinamide 40 mg/mL was prepared by grinding twelve 400-mg tablets of rufinamide tablets in a glass mortar. Sixty milliliters of Ora-Plus and 60 mL of either Ora-Sweet or Ora-Sweet SF (sugar free) were mixed and added to the powder to make a final volume of 120 mL. Three identical samples of each formulation were prepared and placed in 60-mL amber plastic bottles and were stored at room temperature. A 1-mL sample was withdrawn from each of the 6 bottles with a micropipette immediately after preparation and at 7, 14, 28, 56, and 90 days. After further dilution to an expected concentration of 0.4 mg/mL, the samples were assayed using high-performance liquid chromatography. Stability was defined as the retention of at least 90% of the initial concentration. At least 90% of the initial rufinamide concentration remained throughout the 90-day study period in both preparations. There were no detectable changes in color, odor, taste, and pH and no visible microbial growth. Extemporaneously compounded suspensions of rufinamide 40 mg/mL in a 1:1 mixture of Ora-Plus and Ora-Sweet or Ora-Sweet SF were stable for at least 90 days when stored in 59-mL amber polypropylene plastic bottles at room temperature.

  2. 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

  3. Creatine Deficiency Syndrome could be Missed Easily: A Case Report of Guanidinoacetate Methyltransferase Deficiency Presented with Neurodevelopmental Delay, Seizures, and Behavioral Changes, but Normal Structural MRI.

    Science.gov (United States)

    Pacheva, Iliyana; Ivanov, Ivan; Penkov, Marin; Kancheva, Daliya; Jordanova, Albena; Ivanova, Mariya

    2016-09-01

    A case with GAMT deficiency (homozygous c.64dupG mutation) presented with neurodevelopmental delay, rare seizures, behavioral disturbances, and mild hypotonia, posing diagnostic challenges. Metabolic investigations showed low creatinine in plasma and urine (guanidinoacetate couldn't be investigated) and slightly elevated lactate. MRI was normal. Correct diagnosis was possible only after MR spectroscopy was performed at age 5½ years. A homozygous c.64dupG mutation of the GAMT gene was identified in the proband. In conclusion, every case with neurodevelopmental delay or arrest, especially when accompanied by seizures, behavioral impairment, muscle hypotonia or extrapyramidal symptoms should undergo MRI with MR spectroscopy. Normal structural MRI doesn't exclude a creatine deficiency syndrome. Biochemical investigations of guanidinoacetate, creatine, and creatinine in body fluid should be done to diagnose cerebral creatine deficiency syndromes and to specify the deficient enzyme. Thus, a treatable disease will not be missed. © 2016 by the Association of Clinical Scientists, Inc.

  4. GABAergic Neuron-Specific Loss of Ube3a Causes Angelman Syndrome-Like EEG Abnormalities and Enhances Seizure Susceptibility.

    Science.gov (United States)

    Judson, Matthew C; Wallace, Michael L; Sidorov, Michael S; Burette, Alain C; Gu, Bin; van Woerden, Geeske M; King, Ian F; Han, Ji Eun; Zylka, Mark J; Elgersma, Ype; Weinberg, Richard J; Philpot, Benjamin D

    2016-04-06

    Loss of maternal UBE3A causes Angelman syndrome (AS), a neurodevelopmental disorder associated with severe epilepsy. We previously implicated GABAergic deficits onto layer (L) 2/3 pyramidal neurons in the pathogenesis of neocortical hyperexcitability, and perhaps epilepsy, in AS model mice. Here we investigate consequences of selective Ube3a loss from either GABAergic or glutamatergic neurons, focusing on the development of hyperexcitability within L2/3 neocortex and in broader circuit and behavioral contexts. We find that GABAergic Ube3a loss causes AS-like increases in neocortical EEG delta power, enhances seizure susceptibility, and leads to presynaptic accumulation of clathrin-coated vesicles (CCVs)-all without decreasing GABAergic inhibition onto L2/3 pyramidal neurons. Conversely, glutamatergic Ube3a loss fails to yield EEG abnormalities, seizures, or associated CCV phenotypes, despite impairing tonic inhibition onto L2/3 pyramidal neurons. These results substantiate GABAergic Ube3a loss as the principal cause of circuit hyperexcitability in AS mice, lending insight into ictogenic mechanisms in AS. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. Absence seizure

    Science.gov (United States)

    Seizure - petit mal; Seizure - absence; Petit mal seizure; Epilepsy - absence seizure ... Elsevier; 2016:chap 101. Marcdante KJ, Kliegman RM. Seizures (paroxysmal disorders). In: Marcdante KJ, Kliegman RM, eds. Nelson Essentials ...

  6. Dravet syndrome

    Directory of Open Access Journals (Sweden)

    Incorpora Gemma

    2009-09-01

    Full Text Available Abstract "Dravet syndrome" (DS previously named severe myoclonic epilepsy of infancy (SMEI, or epilepsy with polymorphic seizures, is a rare disorder characterized by an early, severe, generalized, epileptic encephalopathy. DS is characterized by febrile and afebrile seizures beginning in the 1st year of life followed by different types of seizures (either focal or generalized, which are typically resistant to antiepileptic drugs. A developmental delay from the 2nd to 3rd year of life becomes evident, together with motor disturbances and personality disorders. Beside the classic syndrome, there are milder cases which have been called severe myoclonic epilepsy borderline (SMEB. DS is caused by a mutation in the neuronal sodium channel gene, SCN1A , that is also mutated in generalized epilepsy with FS+ (GEFS+.

  7. Progressive seizures in a patient with congenital coagulopathies.

    Science.gov (United States)

    Berryman, Rosanna; Imam, Ibrahim; Whitfield, Peter C; Mukonoweshuro, William; Salih, Isam

    2011-06-30

    Dural arteriovenous fistula (DAVF) is a rare complication of cerebral venous sinus thrombosis (CVST). DAVFs develop as a result of direct arterial to venous sinus communications evolving in response to an occluded sinus. The authors present a patient with Down's syndrome who developed progressive, uncontrolled seizures and chronic CVST secondary to factor V Leiden deficiency. Brain MRI and computerised tomographic venography revealed a complex secondary DAVF, which once embolised resulted in a dramatic reduction in seizures and improvement in the clinical state. DAVFs should be considered as a potential complication in patients with persistent features of CVST.

  8. Soy infant formula and seizures in children with autism: a retrospective study.

    Directory of Open Access Journals (Sweden)

    Cara J Westmark

    Full Text Available Seizures are a common phenotype in many neurodevelopmental disorders including fragile X syndrome, Down syndrome and autism. We hypothesized that phytoestrogens in soy-based infant formula were contributing to lower seizure threshold in these disorders. Herein, we evaluated the dependence of seizure incidence on infant formula in a population of autistic children. Medical record data were obtained on 1,949 autistic children from the SFARI Simplex Collection. An autism diagnosis was determined by scores on the ADI-R and ADOS exams. The database included data on infant formula use, seizure incidence, the specific type of seizure exhibited and IQ. Soy-based formula was utilized in 17.5% of the study population. Females comprised 13.4% of the subjects. There was a 2.6-fold higher rate of febrile seizures [4.2% versus 1.6%, OR = 2.6, 95% CI = 1.3-5.3], a 2.1-fold higher rate of epilepsy comorbidity [3.6% versus 1.7%, OR = 2.2, 95% CI = 1.1-4.7] and a 4-fold higher rate of simple partial seizures [1.2% versus 0.3%, OR = 4.8, 95% CI = 1.0-23] in the autistic children fed soy-based formula. No statistically significant associations were found with other outcomes including: IQ, age of seizure onset, infantile spasms and atonic, generalized tonic clonic, absence and complex partial seizures. Limitations of the study included: infant formula and seizure data were based on parental recall, there were significantly less female subjects, and there was lack of data regarding critical confounders such as the reasons the subjects used soy formula, age at which soy formula was initiated and the length of time on soy formula. Despite these limitations, our results suggest that the use of soy-based infant formula may be associated with febrile seizures in both genders and with a diagnosis of epilepsy in males in autistic children. Given the lack of data on critical confounders and the retrospective nature of the study, a prospective study is

  9. Computed Tomography Findings in Patients with Seizure Disorder

    Directory of Open Access Journals (Sweden)

    Sumnima Acharya

    2016-06-01

    Full Text Available Introduction: Seizure occurs in up to 10% of the population, whereas epilepsy is a chronic disease characterized by recurrent seizures that may affect up to 2% of the population. Modern neuroimaging is useful in diagnosis of  abnormalities underlying the epilepsies, but the information provided by imaging techniques can also contribute to proper classification of certain epileptic disorders and can delineate the genetics of some underlying syndromes. Neuroimaging is even more important for those patients who have medically intractable seizures. This study was carried out to establish different etiologies of seizures, to correlate the clinical data and radiological findings in cases of seizure, and to identify the common etiologies in different types of seizures. Methods: This was a retrospective hospital-based study conducted in the Department of Radiodiagnosis of Lumbini Medical College Teaching Hospital. Records of patients of past two years, admitted in any department of the hospital with history of seizure disorder and underwent a Computed Tomography  (CT of brain were included. The CT patterns were assessed and the data were tabulated and statistically analyzed. Results: There were a total of 480 cases out of which 263 (55% were male and 217 (45% were female with M:F ratio of 1.2:1. Generalized seizure was more frequent than partial seizure in both gender. In 274 cases of generalized seizures, CT scan findings were abnormal in 151 cases and normal finding observed in 123 cases. In 206 cases of partial seizures, 125 cases were abnormal and 81 having normal CT scan findings. Age wise distribution showed highest number (n=218 of cases in young group (60 yr. The most common cause of seizure  was  calcified granuloma (n=79, 16.5% followed by neurocysticercosis (NCC, n=64, 13%. Diffuse cerebral edema, sub-arachnoid hemorrhage, and hydrocephalus was seen only in lower age group particularly among 1-20 years. Infarct and diffuse cortical

  10. Refractory Seizures in Tramadol Poisoning: A Case Report

    Directory of Open Access Journals (Sweden)

    Mohammad Majidi

    2014-09-01

    Full Text Available Background: Tramadol, an analgesic drug abused by opioid addicts, is also abused accidentally or for suicidal purposes. Tramadol poisoning can induce CNS depression, seizures, coma, and ultimately death. Case: In this report, a 30-year-old male was admitted to the emergency department due to suicidal attempt with ingestion of 14000 mg (140 tablet 100 mg of tramadol. He had history of suicidal attempts in past years as well as depression in his past medical history, but he had not abused tramadol and other drugs in his history. There was no history of epilepsy or head trauma in. He presented with generalized seizures two hours post ingestion, and, then, he was referred to hospital four hours later. Generalized seizures were poorly controlled by multiple medications. Due to respiratory arrest, endotracheal tube was inserted and he was admitted to the ICU immediately. At admission, he experienced hypovolemic shock, hypoglycemia, coma, apnea, refractory seizures, muscle spasms, acute respiratory distress syndrome, coagolative disorder, rhabdomyolysis, and acute renal failure. Despite medical managements, he died 38 days after ingestion. Conclusion: In this report, despite using inhalational anesthetic drugs, seizures continued and were very poorly controlled. Cause of death in this patient can be seen as the side effects of tramadol poisoning.

  11. Thalamus lesions in chronic and acute seizure disorders

    Energy Technology Data Exchange (ETDEWEB)

    Tschampa, Henriette J; Greschus, Susanne; Urbach, Horst [University of Bonn, Department of Radiology (Neuroradiology), Bonn (Germany); Sassen, Robert; Bien, Christian G [University of Bonn, Department of Epileptology, Bonn (Germany)

    2011-04-15

    Transient signal changes in the pulvinar have been described following status epilepticus. However, we observed persistent thalamus changes after seizures. The purpose of this study was to characterize thalamus changes in patients with seizure disorders and to correlate imaging findings with clinical features. We searched among 5,500 magnetic resonance imaging (MRI) exams performed in patients with seizures and identified 43 patients. The MRI scans of these patients were reviewed and correlated with clinical data. We identified four patterns of thalamus lesions: (a) fluid attenuated inversion recovery-hyperintense pulvinar lesions (20 patients), as known from status epilepticus. Ten patients in this group had a status epilepticus. Among the remaining patients, three had frequent seizures and seven had sporadic seizures. Twelve patients had follow-up exams for a median of 11 months. The lesions had persisted in 11/12 cases in the last available exam and were reversible in one case only. In seven cases, cone-shaped thalamus atrophy resulted, (b) linear defects in the medial and anterior thalamus (five patients), accompanied by atrophy of the mamillary body and the fornix in patients with chronic epilepsy, (c) extensive bilateral thalamus lesions in two patients with a syndrome caused by mutation in the mitochondrial polymerase gamma, and (d) other thalamus lesions not associated with the seizure disorder (16 patients). The spectrum of thalamus lesions in patients with seizure disorders is wider than previously reported. Postictal pulvinar lesions can persist and may result in thalamic atrophy. Linear defects in the anterior thalamus are associated with limbic system atrophy. (orig.)

  12. Thalamus lesions in chronic and acute seizure disorders

    International Nuclear Information System (INIS)

    Tschampa, Henriette J.; Greschus, Susanne; Urbach, Horst; Sassen, Robert; Bien, Christian G.

    2011-01-01

    Transient signal changes in the pulvinar have been described following status epilepticus. However, we observed persistent thalamus changes after seizures. The purpose of this study was to characterize thalamus changes in patients with seizure disorders and to correlate imaging findings with clinical features. We searched among 5,500 magnetic resonance imaging (MRI) exams performed in patients with seizures and identified 43 patients. The MRI scans of these patients were reviewed and correlated with clinical data. We identified four patterns of thalamus lesions: (a) fluid attenuated inversion recovery-hyperintense pulvinar lesions (20 patients), as known from status epilepticus. Ten patients in this group had a status epilepticus. Among the remaining patients, three had frequent seizures and seven had sporadic seizures. Twelve patients had follow-up exams for a median of 11 months. The lesions had persisted in 11/12 cases in the last available exam and were reversible in one case only. In seven cases, cone-shaped thalamus atrophy resulted, (b) linear defects in the medial and anterior thalamus (five patients), accompanied by atrophy of the mamillary body and the fornix in patients with chronic epilepsy, (c) extensive bilateral thalamus lesions in two patients with a syndrome caused by mutation in the mitochondrial polymerase gamma, and (d) other thalamus lesions not associated with the seizure disorder (16 patients). The spectrum of thalamus lesions in patients with seizure disorders is wider than previously reported. Postictal pulvinar lesions can persist and may result in thalamic atrophy. Linear defects in the anterior thalamus are associated with limbic system atrophy. (orig.)

  13. A case report of reversible generalized seizures in a patient with Waardenburg syndrome associated with a novel nonsense mutation in the penultimate exon of SOX10.

    Science.gov (United States)

    Suzuki, Noriomi; Mutai, Hideki; Miya, Fuyuki; Tsunoda, Tatsuhiko; Terashima, Hiroshi; Morimoto, Noriko; Matsunaga, Tatsuo

    2018-05-23

    Waardenburg syndrome type 1 (WS1) can be distinguished from Waardenburg syndrome type 2 (WS2) by the presence of dystopia canthorum. About 96% of WS1 are due to PAX3 mutations, and SOX10 mutations have been reported in 15% of WS2. This report describes a patient with WS1 who harbored a novel SOX10 nonsense mutation (c.652G > T, p.G218*) in exon 3 which is the penultimate exon. The patient had mild prodromal neurological symptoms that were followed by severe attacks of generalized seizures associated with delayed myelination of the brain. The immature myelination recovered later and the neurological symptoms could be improved. This is the first truncating mutation in exon 3 of SOX10 that is associated with neurological symptoms in Waardenburg syndrome. Previous studies reported that the neurological symptoms that associate with WS are congenital and irreversible. These findings suggest that the reversible neurological phenotype may be associated with the nonsense mutation in exon 3 of SOX10. When patients of WS show mild prodromal neurological symptoms, the clinician should be aware of the possibility that severe attacks of generalized seizures may follow, which may be associated with the truncating mutation in exon 3 of SOX10.

  14. Seizure ending signs in patients with dyscognitive focal seizures.

    Science.gov (United States)

    Gavvala, Jay R; Gerard, Elizabeth E; Macken, Mícheál; Schuele, Stephan U

    2015-09-01

    Signs indicating the end of a focal seizure with loss of awareness and/or responsiveness but without progression to focal or generalized motor symptoms are poorly defined and can be difficult to determine. Not recognizing the transition from ictal to postictal behaviour can affect seizure reporting accuracy by family members and may lead to delayed or a lack of examination during EEG monitoring, erroneous seizure localization and inadequate medical intervention for prolonged seizure duration. Our epilepsy monitoring unit database was searched for focal seizures without secondary generalization for the period from 2007 to 2011. The first focal seizure in a patient with loss of awareness and/or responsiveness and/or behavioural arrest, with or without automatisms, was included. Seizures without objective symptoms or inadequate video-EEG quality were excluded. A total of 67 patients were included, with an average age of 41.7 years. Thirty-six of the patients had seizures from the left hemisphere and 29 from the right. All patients showed an abrupt change in motor activity and resumed contact with the environment as a sign of clinical seizure ending. Specific ending signs (nose wiping, coughing, sighing, throat clearing, or laughter) were seen in 23 of 47 of temporal lobe seizures and 7 of 20 extra-temporal seizures. Seizure ending signs are often subtle and the most common finding is a sudden change in motor activity and resumption of contact with the environment. More distinct signs, such as nose wiping, coughing or throat clearing, are not specific to temporal lobe onset. A higher proportion of seizures during sleep went unexamined, compared to those during wakefulness. This demonstrates that seizure semiology can be very subtle and arousals from sleep during monitoring should alert staff. Patient accounts of seizure frequency appear to be unreliable and witness reports need to be taken into account. [Published with video sequences].

  15. Seizures and Teens: Stress, Sleep, & Seizures

    Science.gov (United States)

    Shafer, Patricia Osborne

    2007-01-01

    Most parents are used to erratic sleep patterns and mood swings in their teenagers. When these occur in an adolescent with seizures, however, the parent may wonder if sleep and mood problems are related to seizures. Sorting out the cause and effects of sleep in an adolescent with seizures can be confusing. Since stress can be a contributor to both…

  16. Termination of seizure clusters is related to the duration of focal seizures.

    Science.gov (United States)

    Ferastraoaru, Victor; Schulze-Bonhage, Andreas; Lipton, Richard B; Dümpelmann, Matthias; Legatt, Alan D; Blumberg, Julie; Haut, Sheryl R

    2016-06-01

    Clustered seizures are characterized by shorter than usual interseizure intervals and pose increased morbidity risk. This study examines the characteristics of seizures that cluster, with special attention to the final seizure in a cluster. This is a retrospective analysis of long-term inpatient monitoring data from the EPILEPSIAE project. Patients underwent presurgical evaluation from 2002 to 2009. Seizure clusters were defined by the occurrence of at least two consecutive seizures with interseizure intervals of <4 h. Other definitions of seizure clustering were examined in a sensitivity analysis. Seizures were classified into three contextually defined groups: isolated seizures (not meeting clustering criteria), terminal seizure (last seizure in a cluster), and intracluster seizures (any other seizures within a cluster). Seizure characteristics were compared among the three groups in terms of duration, type (focal seizures remaining restricted to one hemisphere vs. evolving bilaterally), seizure origin, and localization concordance among pairs of consecutive seizures. Among 92 subjects, 77 (83%) had at least one seizure cluster. The intracluster seizures were significantly shorter than the last seizure in a cluster (p = 0.011), whereas the last seizure in a cluster resembled the isolated seizures in terms of duration. Although focal only (unilateral), seizures were shorter than seizures that evolved bilaterally and there was no correlation between the seizure type and the seizure position in relation to a cluster (p = 0.762). Frontal and temporal lobe seizures were more likely to cluster compared with other localizations (p = 0.009). Seizure pairs that are part of a cluster were more likely to have a concordant origin than were isolated seizures. Results were similar for the 2 h definition of clustering, but not for the 8 h definition of clustering. We demonstrated that intracluster seizures are short relative to isolated seizures and terminal seizures. Frontal

  17. Seizures in Chédiak-Higashi syndrome: case report Crises convulsivas na síndrome de Chédiak-Higashi: relato de caso

    Directory of Open Access Journals (Sweden)

    GABRIEL R. DE FREITAS

    1999-06-01

    Full Text Available Chédiak-Higashi syndrome is a rare hematological disease characterized by increased fusion of cytoplasmatic granules. Neurological symptoms occur in approximately half of the patients. We describe the clinical, eletrophysiological, hematological and radiological findings in a girl who had Chédiak-Higashi syndrome and seizures.A síndrome de Chédiak-Higashi é uma doença hematológica rara caracterizada pelo aumento da fusão dos grânulos citoplasmáticos. Manifestações neurológicas ocorrem em cerca de metade dos pacientes. Relatamos o caso de uma criança com síndrome de Chédiak-Higashi e crises convulsivas.

  18. Identifying seizure clusters in patients with psychogenic nonepileptic seizures.

    Science.gov (United States)

    Baird, Grayson L; Harlow, Lisa L; Machan, Jason T; Thomas, Dave; LaFrance, W C

    2017-08-01

    The present study explored how seizure clusters may be defined for those with psychogenic nonepileptic seizures (PNES), a topic for which there is a paucity of literature. The sample was drawn from a multisite randomized clinical trial for PNES; seizure data are from participants' seizure diaries. Three possible cluster definitions were examined: 1) common clinical definition, where ≥3 seizures in a day is considered a cluster, along with two novel statistical definitions, where ≥3 seizures in a day are considered a cluster if the observed number of seizures statistically exceeds what would be expected relative to a patient's: 1) average seizure rate prior to the trial, 2) observed seizure rate for the previous seven days. Prevalence of clusters was 62-68% depending on cluster definition used, and occurrence rate of clusters was 6-19% depending on cluster definition. Based on these data, clusters seem to be common in patients with PNES, and more research is needed to identify if clusters are related to triggers and outcomes. Copyright © 2017 Elsevier Inc. All rights reserved.

  19. How do the many etiologies of West syndrome lead to excitability and seizures? The corticotropin releasing hormone excess hypothesis.

    Science.gov (United States)

    Brunson, K L; Eghbal-Ahmadi, M; Baram, T Z

    2001-11-01

    West syndrome (WS) is associated with diverse etiological factors. This fact has suggested that there must be a 'final common pathway' for these etiologies, which operates on the immature brain to result in WS only at the maturational state present during infancy. Any theory for the pathogenesis of WS has to account for the unique features of this disorder. For example, how can a single entity have so many etiologies? Why does WS arise only in infancy, even when a known insult had occurred prenatally, and why does it disappear? Why is WS associated with lasting cognitive dysfunction? And, importantly, why do these seizures--unlike most others--respond to treatment by a hormone, ACTH? The established hormonal role of ACTH in human physiology is to function in the neuroendocrine cascade of the responses to all stressful stimuli, including insults to the brain. As part of this function, ACTH is known to suppress the production of corticotropin releasing hormone (CRH), a peptide that is produced in response to diverse insults and stressors.The many etiologies of WS all lead to activation of the stress response, including increased production and secretion of the stress-neurohormone CRH. CRH has been shown, in infant animal models, to cause severe seizures and death of neurons in areas involved with learning and memory. These effects of CRH are restricted to the infancy period because the receptors for CRH, which mediate its action on neurons, are most abundant during this developmental period. ACTH administration is known to inhibit production and release of CRH via a negative feedback mechanism. Therefore, the efficacy of ACTH for WS may depend on its ability to decrease the levels of the seizure-promoting stress-neurohormone CRH.This CRH-excess theory for the pathophysiology of WS is consistent not only with the profile of ACTH effects, but also with the many different 'causes' of WS, with the abnormal ACTH levels in the cerebrospinal fluid of affected infants and

  20. Febrile Seizure Simulation

    Directory of Open Access Journals (Sweden)

    Victor Cisneros

    2017-01-01

    Full Text Available Audience: This simulation session is appropriate for medical students, community physicians, or residents in emergency medicine, neurology, pediatrics, or family medicine. Introduction: Febrile seizures are the most common form of seizures in childhood; they are thought to occur in 2-5% of all children.1-3 Febrile seizures are defined as a seizure in association with a febrile illness in children without a central nervous system infection, previous afebrile seizure, known brain disorder, or electrolyte abnormalities. 1,2 They typically occur between 6 months and 18 months of age though they can occur up to 5 years of age.3 Febrile seizures are categorized as: simple (generalized seizure lasting less than 15 minutes in a child aged 6 months to 5 years, and less than 1 in a 24 hour period or complex (a focal seizure or generalized seizure lasting greater than 15 minutes, or multiple seizures in a 24 hour period. 1,3 Treatment for febrile seizures is based on treating the underlying cause of the fever and giving reassurance and education to the parents.2 Mortality is extremely rare, and there is no difference in the patient’s cognitive abilities after a febrile seizure, even when the seizure is prolonged.1 Objectives: At the end of this simulation session, the learner will be able to: 1 discuss the management of febrile seizures 2 discuss when placement of an advanced airway is indicated in the management of a febrile seizure 3 list the risk factors for febrile seizures 4 prepare a differential diagnosis for the causes of febrile seizures 5 educate family members on febrile seizures. Methods: This educational session is a high-fidelity simulation.

  1. Detection of brain atrophy due to ACTH or corticosteroid therapy with computed tomography

    International Nuclear Information System (INIS)

    Tamai, Isamu; Takei, Tadao; Oota, Hideomi; Maekawa, Kihei.

    1981-01-01

    Adrenocorticotropic hormone (ACTH) or corticosteroids seemed to cause brain atrophy in intants. We studied the atrophy which was caused by these drugs with computed tomography (CT). 1) Nine cases of infantile spasms examined before, during and after ACTH therepy with CT. Brain atrophy on CT was observed immediately after the completion of ACTH therapy. The brain atrophy receded slightly after several months. It was more marked in younger patients, in cases treated by hight doses of ACTH and in cases where brain atrophy had already been obserbed before ACTH therapy. 2) Twenty cases of infantile spasms or Lennox Gastaut syndrome were examined after ACTH therapy with CT. Brain atrophy was observed in twelve cases. Main features of brain atrophy were the enlargement of sylvian fissure and the widening of subarachnoid space at the frontal or temporal region. Mental retardation was observed in eighteen cases. 3) Two cases of nephrotic syndrome were treated with pulse therapy of prednisolone. CT was carried out before and after treatment. Atrophy of cerebrum was observed in these cases. 4) A case of infantile spasms treated with anticonvulsants without ACTH was studied by electroencephalography (EEG) and CT. The abnormal pattern of EEG was markedly corrected, while brain atrophy on CT was not observed after the therapy. Because of these observations the use of ACTH has to be reconsidered. ACTH should be the drug of second choice for the therapy of infantile spasms and should be used in case other anticonvulsants have no effect. ACTH should be used at lower dosages and for shorter periods of time. (author)

  2. Detection of brain atrophy due to ACTH or corticosteroid therapy with computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Tamai, I.; Takei, T. (National Sagamihara Hospital, Kanagawa (Japan)); Oota, H.; Maekawa, K.

    1981-07-01

    Adrenocorticotropic hormone (ACTH) or corticosteroids seemed to cause brain atrophy in infants. We studied the atrophy which was caused by these drugs with computed tomography (CT). 1) Nine cases of infantile spasms examined before, during and after ACTH therapy with CT. Brain atrophy on CT was observed immediately after the completion of ACTH therapy. The brain atrophy receded slightly after several months. It was more marked in younger patients, in cases treated by high doses of ACTH and in cases where brain atrophy had already been observed before ACTH therapy. 2) Twenty cases of infantile spasms or Lennox Gastaut syndrome were examined after ACTH therapy with CT. Brain atrophy was observed in twelve cases. Main features of brain atrophy were the enlargement of sylvian fissure and the widening of subarachnoid space at the frontal or temporal region. Mental retardation was observed in eighteen cases. 3) Two cases of nephrotic syndrome were treated with pulse therapy of prednisolone. CT was carried out before and after treatment. Atrophy of cerebrum was observed in these cases. 4) A case of infantile spasms treated with anticonvulsants without ACTH was studied by electroencephalography (EEG) and CT. The abnormal pattern of EEG was markedly corrected, while brain atrophy on CT was not observed after the therapy. Because of these observations the use of ACTH has to be reconsidered. ACTH should be the drug of second choice for the therapy of infantile spasms and should be used in case other anticonvulsants have no effect. ACTH should be used at lower dosages and for shorter periods of time.

  3. A novel CDKL5 mutation in a 47,XXY boy with the early-onset seizure variant of Rett syndrome.

    Science.gov (United States)

    Sartori, Stefano; Di Rosa, Gabriella; Polli, Roberta; Bettella, Elisa; Tricomi, Giovanni; Tortorella, Gaetano; Murgia, Alessandra

    2009-02-01

    Mutations of the cyclin-dependent kinase-like 5 gene (CDKL5), reported almost exclusively in female subjects, have been recently found to be the cause of a phenotype overlapping Rett syndrome with early-onset epileptic encephalopathy. We describe the first CDKL5 mutation detected in a male individual with 47,XXY karyotype. This previously unreported, de novo, mutation truncates the large CDKL5 COOH-terminal region, thought to be crucial for the proper sub-cellular localization of the CDKL5 protein. The resulting phenotype is characterized by a severe early-onset epileptic encephalopathy, global developmental delay, and profound intellectual and motor impairment with features reminiscent of Rett syndrome. In light of the data presented we discuss the possible phenotypic modulatory effects of the supernumerary wild type X allele and pattern of X chromosome inactivation and stress the importance of considering the causal involvement of CDKL5 in developmentally delayed males with early-onset seizures. (c) 2009 Wiley-Liss, Inc.

  4. Dextromethorphan in the treatment of early myoclonic encephalopathy evolving into migrating partial seizures in infancy

    Directory of Open Access Journals (Sweden)

    Yin-Hsuan Chien

    2012-05-01

    Full Text Available Epileptic encephalopathy with suppression-burst in electroencephalography (EEG can evolve into a few types of epileptic syndromes. We present here an unusual case of early myoclonic encephalopathy that evolved into migrating partial seizures in infancy. A female neonate initially had erratic myoclonus movements, hiccups, and a suppression-burst pattern in EEG that was compatible with early myoclonic encephalopathy. The seizures were controlled with dextromethorphan (20 mg/kg, and a suppression-burst pattern in EEG was reverted to relatively normal background activity. However, at 72 days of age, alternating focal tonic seizures, compatible with migrating partial seizures in infancy, were demonstrated by the 24-hour EEG recording. The seizures responded poorly to dextromethorphan. To our knowledge, this is the first reported case of early myoclonic encephalopathy evolving into migrating partial seizure in infancy. Whether it represents another age-dependent epilepsy evolution needs more clinical observation.

  5. IB-12LUNG MASS AND IMMUNOSUPPRESSANT RESPONSIVE SEIZURES: VGKC AUTOIMMUNITY MASQUERADING AS ETIOLOGY OR ACTING AS REACTIVE MARKER?

    OpenAIRE

    Umemura, Yoshie; Bujarski, Krzysztof; Ronan, Lara

    2014-01-01

    Voltage gated potassium channel complex antibody (VGKC Ab) has been associated with many neurological illnesses including seizures. VGKC Ab related seizures are less responsive to antiepileptic drugs alone, and often require immunosuppression to achieve seizure freedom. Recently, specific antigenic targets such as LGI1 and CASPR2 within VGKC have been found and have been associated with specific syndromes. It has also been noted that the term "VGKC Ab" itself is problematic because it groups ...

  6. The startle syndromes : Physiology and treatment

    NARCIS (Netherlands)

    Dreissen, Yasmine E. M.; Tijssen, Marina A. J.

    2012-01-01

    Startle syndromes are paroxysmal and show stimulus sensitivity, placing them in the differential diagnosis of epileptic seizures. Startle syndromes form a heterogeneous group of disorders with three categories: hyperekplexia (HPX), stimulus-induced disorders, and neuropsychiatric syndromes. HPX is

  7. The startle syndromes: Physiology and treatment

    NARCIS (Netherlands)

    Dreissen, Yasmine E. M.; Tijssen, Marina A. J.

    2012-01-01

    Startle syndromes are paroxysmal and show stimulus sensitivity, placing them in the differential diagnosis of epileptic seizures. Startle syndromes form a heterogeneous group of disorders with three categories: hyperekplexia (HPX), stimulus-induced disorders, and neuropsychiatric syndromes. HPX is

  8. Febrile seizures

    Science.gov (United States)

    ... proper care. Occasionally, a provider will prescribe a medicine called diazepam to prevent or treat febrile seizures that occur more than once. However, no drug is completely effective in preventing febrile seizures. Alternative Names Seizure - fever induced; Febrile convulsions Patient Instructions ...

  9. Automated seizure detection systems and their effectiveness for each type of seizure.

    Science.gov (United States)

    Ulate-Campos, A; Coughlin, F; Gaínza-Lein, M; Fernández, I Sánchez; Pearl, P L; Loddenkemper, T

    2016-08-01

    Epilepsy affects almost 1% of the population and most of the approximately 20-30% of patients with refractory epilepsy have one or more seizures per month. Seizure detection devices allow an objective assessment of seizure frequency and a treatment tailored to the individual patient. A rapid recognition and treatment of seizures through closed-loop systems could potentially decrease morbidity and mortality in epilepsy. However, no single detection device can detect all seizure types. Therefore, the choice of a seizure detection device should consider the patient-specific seizure semiologies. This review of the literature evaluates seizure detection devices and their effectiveness for different seizure types. Our aim is to summarize current evidence, offer suggestions on how to select the most suitable seizure detection device for each patient and provide guidance to physicians, families and researchers when choosing or designing seizure detection devices. Further, this review will guide future prospective validation studies. Copyright © 2016. Published by Elsevier Ltd.

  10. Association of adherence to epilepsy quality standards with seizure control.

    Science.gov (United States)

    Moura, Lidia M V R; Mendez, Diego Yacaman; Jesus, Jonathan De; Andrade, Rogger A; Weissman, Joel S; Vickrey, Barbara G; Hoch, Daniel B

    2015-11-01

    We assessed the relationship between adherence to epilepsy quality measures (EQM) and seizure control over 2-3 years in a retrospective cohort study. 6150 patients were identified at two large academic medical centers with a primary or secondary diagnosis of epilepsy, were 18-85 years old and seen in outpatient general neurology or epilepsy units between June 2011 and May 2014. Patients were included if: their initial visit was between June 2011 and June 2012, treatment was with ≥1 anti-seizure drug, there was ≥1 visit per year during the timeframe, and seizure frequency was documented at initial and final visits, yielding 162 patients/1055 visits from which socio-demographic, clinical and care quality data were abstracted. Quality care was assessed as (1) percent adherence to up to 8 eligible EQM, and (2) defect-free care (DFC: adherence to all eligible EQM). Seizure control (SC) was defined as ≥50% reduction in average seizures/month between initial and final visits. Chi-square and t-test compared care quality with seizure control. Logistic regression was used to assess the relationships between SC, quality of care and subspecialist involvement. Care quality, reflected by documentation of seizure frequency, addressing therapeutic interventions, and referral to a comprehensive epilepsy center, all exceeded 80% adherence. Care quality as reflected by documentation of seizure type, etiology or syndrome; assessment of side effects, counseling about epilepsy safety and women's issues, and screening for psychiatric disorders ranged from 40 to 57%. Mean EQM adherence across all applicable measures was associated with greater seizure control (p=0.0098). DFC was low (=8%) and did not covary with seizure control (p=0.55). The SC and non-SC groups only differed on epilepsy etiology (p=0.04). Exploratory analysis showed that mean quality scores are associated with seizure control (OR=4.9 [1.3-18.5], p=0.017) while controlling for the effect of subspecialty involvement

  11. Deep Recurrent Neural Networks for seizure detection and early seizure detection systems

    Energy Technology Data Exchange (ETDEWEB)

    Talathi, S. S. [Lawrence Livermore National Lab. (LLNL), Livermore, CA (United States)

    2017-06-05

    Epilepsy is common neurological diseases, affecting about 0.6-0.8 % of world population. Epileptic patients suffer from chronic unprovoked seizures, which can result in broad spectrum of debilitating medical and social consequences. Since seizures, in general, occur infrequently and are unpredictable, automated seizure detection systems are recommended to screen for seizures during long-term electroencephalogram (EEG) recordings. In addition, systems for early seizure detection can lead to the development of new types of intervention systems that are designed to control or shorten the duration of seizure events. In this article, we investigate the utility of recurrent neural networks (RNNs) in designing seizure detection and early seizure detection systems. We propose a deep learning framework via the use of Gated Recurrent Unit (GRU) RNNs for seizure detection. We use publicly available data in order to evaluate our method and demonstrate very promising evaluation results with overall accuracy close to 100 %. We also systematically investigate the application of our method for early seizure warning systems. Our method can detect about 98% of seizure events within the first 5 seconds of the overall epileptic seizure duration.

  12. Seizures

    Science.gov (United States)

    ... may be diagnosed with epilepsy , also known as seizure disorder. Seizure Basics Usually, electrical activity in the brain involves ... times. Fortunately, fainting rarely is a sign of epilepsy. Most kids recover very quickly (seconds to minutes) ...

  13. Angelman Syndrome

    Science.gov (United States)

    ... therapy for seizures is usually necessary. Physical and occupational therapies, communication therapy, and behavioral therapies are important in allowing individuals with Angelman syndrome to reach their maximum developmental potential. × Treatment There ...

  14. Seizures

    Science.gov (United States)

    ... wake up between them. Seizures can have many causes, including medicines, high fevers, head injuries and certain diseases. People who have recurring seizures due to a brain disorder have epilepsy. NIH: National Institute of Neurological Disorders and Stroke

  15. Seizure semiology and electroencephalography in young children with lesional temporal lobe epilepsy.

    Science.gov (United States)

    Lv, Rui-Juan; Sun, Zhen-Rong; Cui, Tao; Shao, Xiao-Qiu

    2014-02-01

    This study aimed to discuss the clinical features of seizure semiology and electroencephalography (EEG) in young children with lesional temporal lobe epilepsy (TLE). Children with lesional TLE received presurgical evaluation for intractable epilepsy. They were followed up for more than one year after temporal lobectomy. We reviewed the medical history and video-EEG monitoring of children with TLE to analyze the semiology of seizures and EEG findings and compared the semiology of seizures and EEG findings of childhood TLE and adult TLE. A total of 84 seizures were analyzed in 11 children (aged 23-108 months). The age of seizure onset was from 1 month to 26 months (a mean of 17.6 months). All of the patients exhibited prominent motor manifestations including epileptic spasm, tonic seizure, and unilateral clonic seizure. Seven children manifested behavioral arrest similar to an automotor seizure in adult TLE but with a shorter duration and higher frequency. The automatisms were typically orofacial, whereas manual automatisms were rarely observed. The EEG recordings revealed that diffuse discharge patterns were more common in younger children, whereas focal or unilateral patterns were more typical in older children. All of the patients were seizure-free after temporal lobectomy with more than one-year follow-up. All of the children had a mental development delay or regression; however, there was improvement after surgery, especially in those with surgery performed early. In contrast to TLE in adults, young children with lesional TLE probably represent a distinct nosological and probably less homogeneous syndrome. Although they had generalized clinical and electrographic features, resective epilepsy surgery should be considered as early as possible to obtain seizure control and improvement in mental development. Copyright © 2013 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  16. Treating acute seizures with benzodiazepines: does seizure duration matter?

    Science.gov (United States)

    Naylor, David E

    2014-10-01

    Several clinical trials have shown improved seizure control and outcome by early initiation of treatment with benzodiazepines, before arrival in the emergency department and before intravenous access can be established. Here, evidence is provided and reviewed for rapid treatment of acute seizures in order to avoid the development of benzodiazepine pharmacoresistance and the emergence of self-sustaining status epilepticus. Alterations in the physiology, pharmacology, and postsynaptic level of GABA-A receptors can develop within minutes to an hour and hinder the ability of synaptic inhibition to stop seizures while also impairing the efficacy of GABAergic agents, such as benzodiazepines, to boost impaired inhibition. In addition, heightened excitatory transmission further exacerbates the inhibitory/excitatory balance and makes seizure control even more resistant to treatment. The acute increase in the surface expression of NMDA receptors during prolonged seizures also may cause excitotoxic injury, cell death, and other pathological expressions and re-arrangements of receptor subunits that all contribute to long-term sequelae such as cognitive impairment and chronic epilepsy. In conclusion, a short window of opportunity exists when seizures are maximally controlled by first-line benzodiazepine treatment. After that, multiple pathological mechanisms quickly become engaged that make seizures increasingly more difficult to control with high risk for long-term harm.

  17. The effects of early-life seizures on hippocampal dendrite development and later-life learning and memory.

    Science.gov (United States)

    Casanova, J R; Nishimura, Masataka; Swann, John W

    2014-04-01

    Severe childhood epilepsy is commonly associated with intellectual developmental disabilities. The reasons for these cognitive deficits are likely multifactorial and will vary between epilepsy syndromes and even among children with the same syndrome. However, one factor these children have in common is the recurring seizures they experience - sometimes on a daily basis. Supporting the idea that the seizures themselves can contribute to intellectual disabilities are laboratory results demonstrating spatial learning and memory deficits in normal mice and rats that have experienced recurrent seizures in infancy. Studies reviewed here have shown that seizures in vivo and electrographic seizure activity in vitro both suppress the growth of hippocampal pyramidal cell dendrites. A simplification of dendritic arborization and a resulting decrease in the number and/or properties of the excitatory synapses on them could help explain the observed cognitive disabilities. There are a wide variety of candidate mechanisms that could be involved in seizure-induced growth suppression. The challenge is designing experiments that will help focus research on a limited number of potential molecular events. Thus far, results suggest that growth suppression is NMDA receptor-dependent and associated with a decrease in activation of the transcription factor CREB. The latter result is intriguing since CREB is known to play an important role in dendrite growth. Seizure-induced dendrite growth suppression may not occur as a single process in which pyramidal cells dendrites simply stop growing or grow slower compared to normal neurons. Instead, recent results suggest that after only a few hours of synchronized epileptiform activity in vitro dendrites appear to partially retract. This acute response is also NMDA receptor dependent and appears to be mediated by the Ca(+2)/calmodulin-dependent phosphatase, calcineurin. An understanding of the staging of seizure-induced growth suppression and the

  18. Ohtahara Syndrome

    Science.gov (United States)

    ... are more often affected than girls. View Full Definition Treatment Antiepileptic drugs are used to control seizures, but are unfortunately ... Other therapies are symptomatic and supportive. × ... Definition Ohtahara syndrome is a neurological disorder characterized by ...

  19. Intraoperative seizures and seizures outcome in patients underwent awake craniotomy.

    Science.gov (United States)

    Yuan, Yang; Peizhi, Zhou; Xiang, Wang; Yanhui, Liu; Ruofei, Liang; Shu, Jiang; Qing, Mao

    2016-11-25

    Awake craniotomies (AC) could reduce neurological deficits compared with patients under general anesthesia, however, intraoperative seizure is a major reason causing awake surgery failure. The purpose of the study was to give a comprehensive overview the published articles focused on seizure incidence in awake craniotomy. Bibliographic searches of the EMBASE, MEDLINE,were performed to identify articles and conference abstracts that investigated the intraoperative seizure frequency of patients underwent AC. Twenty-five studies were included in this meta-analysis. Among the 25 included studies, one was randomized controlled trials and 5 of them were comparable studies. The pooled data suggested the general intraoperative seizure(IOS) rate for patients with AC was 8%(fixed effect model), sub-group analysis identified IOS rate for glioma patients was 8% and low grade patients was 10%. The pooled data showed early seizure rates of AC patients was 11% and late seizure rates was 35%. This systematic review and meta-analysis shows that awake craniotomy is a safe technique with relatively low intraoperative seizure occurrence. However, few RCTs were available, and the acquisition of further evidence through high-quality RCTs is highly recommended.

  20. Ketogenic diet treatment for pediatric super-refractory status epilepticus.

    Science.gov (United States)

    Appavu, Brian; Vanatta, Lisa; Condie, John; Kerrigan, John F; Jarrar, Randa

    2016-10-01

    We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm. A retrospective review was performed at Phoenix Children's Hospital on patients with super-refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015. Ten children with super-refractory status epilepticus, ages 2-16 years, were identified. 4/10 patients had immune mediated encephalitis, including Rasmussen encephalitis, anti-N-methyl-d-aspartate receptor encephalitis, and post-infectious mycoplasma encephalitis. Other etiologies included Lennox Gastaut Syndrome, non-ketotic hyperglycinemia, PCDH19 and GABRG2 genetic epilepsy, New Onset Refractory Status Epilepticus, and Febrile Infection-Related Epilepsy Syndrome. 4/10 patients' EEG features suggested focal with status epilepticus, and 6/10 suggested generalized with status epilepticus. Median hospital length was 61days and median ICU length was 27days. The median number of antiepileptic medications prior to diet initiation was 3.0 drugs, and the median after ketogenic diet treatment was 3.5 drugs. Median duration of status epilepticus prior to KD was 18days. 9/10 patients had resolution of super-refractory status epilepticus in a median of 7days after diet initiation. 8/9 patients were weaned off anesthesia within 15days of diet initiation, and within 1day of achieving ketonuria. 1/10 patients experienced side effects on the diet requiring supplementation. Most patients achieved resolution of status epilepticus on KD therapy, suggesting it could be an effective therapy that can be utilized early in the treatment of children with super refractory status epilepticus. Copyright © 2016. Published by Elsevier Ltd.

  1. Dyke-Davidoff-Masson Syndrome. An unusual cause of status epilepticus.

    Science.gov (United States)

    Zawar, Ifrah; Khan, Ashfa A; Sultan, Tipu; Rathore, Ahsan W

    2015-10-01

    The Dyke-Davidoff-Masson Syndrome (DDMS) results from an insult to the growing brain in utero or early infancy, which lead to loss of neurons compromising the growth of the brain. Clinical presentation includes seizures, hemiparesis, facial asymmetry, and learning disability. Radiological findings include cerebral atrophy on one side. Here, we present a case with status epilepticus who had underlying DDMS. It is a rare syndrome and uncommon cause for status epilepticus. Infections of CNS, hypoxic ischemic encephalopathy, intracranial bleed, trauma, congenital vascular malformations are the common causes of this syndrome. Diagnosis is established after clinical history, examination, and MRI. Intractable seizures can be controlled with appropriate anticonvulsants. Subsequently, these children may require physiotherapy, speech therapy, and occupational therapy in addition to the anticonvulsant medication. Outcome is better if the seizures are controlled.

  2. Seizure development after stroke.

    Science.gov (United States)

    Misirli, H; Ozge, A; Somay, G; Erdoğan, N; Erkal, H; Erenoğlu, N Y

    2006-12-01

    Although there have been many studies on seizures following stroke, there is still much we do not know about them. In this study, we evaluated the characteristics of seizures in stroke patients. There were 2267 patients with a first-ever stroke, and after excluding 387 patients, 1880 were available for analysis. Of these 1880 patients, we evaluated 200 patients with seizures and 400 patients without seizures. We investigated the seizures according to age, gender, stroke type, the aetiology of ischaemic stroke and the localisation of the lesion. The seizures were classified as early onset and late onset and the seizure type as partial, generalised or secondarily generalised. Seizures occurred in 200 (10.6%) of 1880 strokes. The number of patients with seizures were 138 (10.6%) in ischaemic stroke group and 62 (10.7%) in haemorrhagic stroke group. Patients with ischaemic strokes had 41 embolic (29.7%) and 97 thrombotic (70.3%) origin, and these were not statistically significant in comparison with controls. Cortical involvement for the development of seizures was the most important risk factor (odds ratios = 4.25, p < 0.01). It was concluded that embolic strokes, being younger than 65 years old, and cortical localisation of stroke were important risks for developing seizures.

  3. Athletes with seizure disorders.

    Science.gov (United States)

    Knowles, Byron Don; Pleacher, Michael D

    2012-01-01

    Individuals with seizure disorders have long been restricted from participation in certain sporting activities. Those with seizure disorders are more likely than their peers to have a sedentary lifestyle and to develop obesity. Regular participation in physical activity can improve both physical and psychosocial outcomes for persons with seizure disorders. Seizure activity often is reduced among those patients who regularly engage in aerobic activity. Recent literature indicates that the diagnosis of seizure disorders remains highly stigmatizing in the adolescent population. Persons with seizure disorders may be more accepted by peer groups if they are allowed to participate in sports and recreational activities. Persons with seizure disorders are encouraged to participate in regular aerobic activities. They may participate in team sports and contact or collision activities provided that they utilize appropriate protective equipment. There seems to be no increased risk of injury or increasing seizure activity as the result of such participation. Persons with seizure disorders still are discouraged from participating in scuba diving and skydiving. The benefits of participation in regular sporting activity far outweigh any risk to the athlete with a seizure disorder who chooses to participate in sports.

  4. Angelman syndrome, cause of epilepsy in infants

    International Nuclear Information System (INIS)

    Sykora, P.; Vicenova, A.; Svecova, L.; Kolnikova, M.

    2014-01-01

    Several chromosomal syndromes include brain dysfunction symptoms as mental retardation, developmental speech disorders and epilepsy. Authors present a case report of Angelman syndrome – neuro behavioral disorder associated with deletion in the maternal chromosome 15q 11-g13 causing mutation of the UBE3A gene. The main features consist of psychomotor retardation, developmental speech disorder, ataxia, tremor, hyperactivity, clapping hands, inadequate laughter and happiness, attention deficit and epilepsy. The later starts before the 3rd year of age in form of atypical absences, myoclonic and generalized tonic-clonic seizures. EEG typically shows episodes of slow activity with sharp waves occipitally. Prognosis is poor. Genetic syndromes importantly contribute to the etiology of epilepsy with early seizures. (author)

  5. Failure of the Nemo trial: bumetanide is a promising agent to treat many brain disorders but not newborn seizures

    Directory of Open Access Journals (Sweden)

    Yehezkel eBen-Ari

    2016-04-01

    Full Text Available The diuretic bumetanide failed to treat acute seizures due to hypoxic ischemic encephalopathy (HIE in newborn babies and was associated with hearing loss (NEMO trial; 1. On the other hand, clinical and experimental observations suggest that the diuretic might provide novel therapy for many brain disorders including autistic spectrum disorder, schizophrenia, Rett syndrome and Parkinson disease. Here, we discuss the differences between the pathophysiology of severe recurrent seizures in the neonates and neurological and psychiatric disorders stressing the uniqueness of severe seizures in newborn in comparison to other disorders.

  6. Seizure recurrence after a first febrile seizure: a multivariate approach

    NARCIS (Netherlands)

    Offringa, M.; Derksen-Lubsen, G.; Bossuyt, P. M.; Lubsen, J.

    1992-01-01

    The results are presented of a follow-up study of 155 Dutch children after the first febrile seizure. Of these initially untreated children 37 per cent had had at least one, 30 per cent at least two and 17 per cent at least three subsequent seizures. The vulnerable period for recurrent seizures

  7. Seizure Disorders in Pregnancy

    Science.gov (United States)

    ... If I have a seizure disorder, can it cause problems during pregnancy? • What risks are associated with having a seizure ... If I have a seizure disorder, can it cause problems during pregnancy? Seizure disorders can affect pregnancy in several ways: • ...

  8. Genetics Home Reference: ring chromosome 14 syndrome

    Science.gov (United States)

    ... be something about the ring structure itself that causes epilepsy. Seizures may occur because certain genes on the ... mapping of telomeric 14q32 deletions: search for the cause of seizures. Am J Med Genet A. ... L, Elia M, Vigevano F. Epilepsy in ring 14 chromosome syndrome. Epilepsy Behav. 2012 ...

  9. Intravenous levetiracetam terminates refractory status epilepticus in two patients with migrating partial seizures in infancy.

    Science.gov (United States)

    Cilio, Maria Roberta; Bianchi, Roberto; Balestri, Martina; Onofri, Alfredo; Giovannini, Simona; Di Capua, Matteo; Vigevano, Federico

    2009-09-01

    To evaluate the efficacy and tolerability of intravenous (IV) levetiracetam in refractory status epilepticus of migrating partial seizures in infancy (MPSI). IV levetiracetam was infused in two infants, first as a loading dose of 60mg/kg in 30min, then at 30mg/kg twice a day. Both infants were continuously monitored with video-EEG before, during and after the drug trial. Blood count, liver enzymes, serum creatinine, ammonia and lactate blood levels were performed repeatedly before and after the IV levetiracetam administration. Follow-up was of 16 and 10 months. EEG monitoring allowed the diagnosis of MPSI, showing the typical seizures pattern in both patients. IV levetiracetam was effective in stopping status epilepticus in both infants. Levetiracetam also prevented the recurrence of status epilepticus during follow-up. No adverse reactions were observed during the infusion phase or during follow-up. MPSI is a newly recognized epileptic syndrome characterized by early onset of intractable partial seizures arisingly independently and sequentially from both hemispheres, migrating from one region of the brain to another and from one hemisphere to another. We report the efficacy of intravenous levetiracetam in resolving refractory status epilepticus in two infants with this new epilepsy syndrome.

  10. Rufinamide, an antiepileptic drug, improves cognition and increases neurogenesis in the aged gerbil hippocampal dentate gyrus via increasing expressions of IGF-1, IGF-1R and p-CREB.

    Science.gov (United States)

    Chen, Bai Hui; Ahn, Ji Hyeon; Park, Joon Ha; Song, Minah; Kim, Hyunjung; Lee, Tae-Kyeong; Lee, Jae Chul; Kim, Young-Myeong; Hwang, In Koo; Kim, Dae Won; Lee, Choong-Hyun; Yan, Bing Chun; Kang, Il Jun; Won, Moo-Ho

    2018-04-25

    Rufinamide is a novel antiepileptic drug and commonly used in the treatment of Lennox-Gastaut syndrome. In the present study, we investigated effects of rufinamide on cognitive function using passive avoidance test and neurogenesis in the hippocampal dentate gyrus using Ki-67 (a marker for cell proliferation), doublecortin (DCX, a marker for neuroblast) and BrdU/NeuN (markers for newly generated mature neurons) immunohistochemistry in aged gerbils. Aged gerbils (24-month old) were treated with 1 mg/kg and 3 mg/kg rufinamide for 4 weeks. Treatment with 3 mg/kg rufinamide, not 1 mg/kg rufinamide, significantly improved cognitive function and increased neurogenesis, showing that proliferating cells (Ki-67-immunoreactive cells), differentiating neuroblasts (DCX-immunoreactive neuroblasts) and mature neurons (BrdU/NeuN-immunoreactive cells) in the aged dentate gyrus compared with those in the control group. When we examined its mechanisms, rufinamide significantly increased immunoreactivities of insulin-like growth factor-1 (IGF-1), its receptor (IGF-1R), and phosphorylated cAMP response element binding protein (p-CREB). However, rufinamide did not show any increase in immunoreactivities of brain-derived neurotrophic factor and its receptor. Therefore, our results indicate that rufinamide can improve cognitive function and increase neurogenesis in the hippocampus of the aged gerbil via increasing expressions of IGF-1, IGF-1R and p-CREB. Copyright © 2018 Elsevier B.V. All rights reserved.

  11. Improved Spatial Ability Correlated with Left Hemisphere Dysfunction in Turner's Syndrome. Implications for Mechanism.

    Science.gov (United States)

    Rovet, Joanne F.

    This study contrasts the performance of a 17-year-old female subject with Turner's syndrome before and after developing left temporal lobe seizures, as a means of identifying the mechanism responsible for the Turner's syndrome spatial impairment. The results revealed a deficit in spatial processing before onset of the seizure disorder. Results…

  12. Risk factor for febrile seizures

    Directory of Open Access Journals (Sweden)

    Odalović Dragica

    2014-01-01

    Full Text Available Febrile seizures are the most frequent neurological disorder in the childhood. According to American Academy of Pediatrics (AAP, they have been defined as seizures provoked by high temperature in children aged between 6 months and 5 years, without previous history of afebrile seizures, intracranial infections and other possible causes of seizures. Seizures can be typical and atypical, according to the characteristics. Pathogenesis of this disorder has not been clarified yet, and it is believed to be a combination of genetic factors, high body temperature and brain maturation. The risk factors for recurrence of febrile seizures are: age in which seizures appeared for the first time, epilepsy in the first degree relative, febrile seizures in the first degree relative, frequent diseases with fever and low body temperature on the beginning of seizures. The frequency of recurrent seizures The risk for occurrence of epilepsy in children with simple seizures is about 1-1.5%, which is slightly higher compared to general population, while it increases to 4-15% in patients with complex seizures. However, there is no evidence that therapy prevents occurrence of epilepsy. When the prevention of recurrent seizures is considered, it is necessary to separate simple from complex seizures. The aim of this paper was to analyze the most important risk factors for febrile seizures, and to evaluate their impact on occurrence of recurrent seizures. Our study included 125 children with febrile seizures, aged from 6 months to 5 years. The presence of febrile seizures and epilepsy in the first degree relative has been noted in 22% of children. Typical febrile seizures were observed in 76% of cases, and atypical in 24%. Most patients had only one seizure (73.6%. Children, who had seizure earlier in life, had more frequent recurrences. Both risk factors were present in 25% of patients, while 68% of patients had only one risk factor. For the children with febrile disease

  13. Generaliserede kramper som debutsymptom ved Gitelmans syndrom

    DEFF Research Database (Denmark)

    Hvelplund, Carolina; Jeppesen, Eva Mosfeldt; Mortensen, Henrik B

    2009-01-01

    Gitelman's syndrome is a rare autosomal recessive syndrome presenting with hypocalciuria, hypomagnesiemia and hypokalemic metabolic alkalosis. This case reports a patient admitted with generalized seizures with the above-mentioned biochemical abnormalities, thus representing a rare onset of Gitel...

  14. Cognitive Dysfunctions in Epileptic Syndromes

    Directory of Open Access Journals (Sweden)

    Semih Ayta

    2014-05-01

    Full Text Available Some children with epilepsy display a low level of intelligence, learning disabilities, attention deficit hyperactivity disorder, mood disorders and anxiety. Besides specific learning disabilities like reading, writing, arithmetics, learning problems may involve other major areas of intellectual functions such as speech and language, attention, memory, fine motor coordination. Even in the presence of common pathology that leads to epilepsy and mental dysfunctions, seizures cause additional cognitive problems. Age at seizure onset, type of seizures and epileptic syndromes are some variables that determine the effect of epilepsy on cognition. As recurrent seizures may have some negative impact on the developing brain, the use of antiepileptic drugs should be considered not only to aim reducing seizures but also to prevent possible seizure-induced cortical dysfunctions. Epilepsy is a disorder requiring a complicated psychological adjustment for the patients and indeed is a disease that affects the whole family. Thus, the management of epilepsy must include educational, psychotherapeutic and behavioral interventions as well as drug treatment.

  15. Binge drinking during pregnancy and risk of seizures in childhood: a study based on the Danish National Birth Cohort

    DEFF Research Database (Denmark)

    Sun, Yuelian; Strandberg-Larsen, Katrine; Vestergaard, Mogens

    2009-01-01

    Seizures are often found in children with fetal alcohol syndrome, but it is not known whether binge drinking during pregnancy by nonalcoholic women is associated with an increased risk of seizure disorders in children. The authors conducted a population-based cohort study of 80,526 liveborn...... singletons in the Danish National Birth Cohort (1996-2002). Information on maternal binge drinking (intake of > or = 5 drinks on a single occasion) was collected in 2 computer-assisted telephone interviews during pregnancy. Children were followed for up to 8 years. Information on neonatal seizures, epilepsy......, and febrile seizures was retrieved from the Danish National Hospital Register. Results showed that exposure to binge drinking episodes during pregnancy was not associated with an increased risk of seizure disorders in children, except for those exposed at 11-16 gestational weeks. These children had a 3...

  16. EEG analysis of seizure patterns using visibility graphs for detection of generalized seizures.

    Science.gov (United States)

    Wang, Lei; Long, Xi; Arends, Johan B A M; Aarts, Ronald M

    2017-10-01

    The traditional EEG features in the time and frequency domain show limited seizure detection performance in the epileptic population with intellectual disability (ID). In addition, the influence of EEG seizure patterns on detection performance was less studied. A single-channel EEG signal can be mapped into visibility graphs (VGS), including basic visibility graph (VG), horizontal VG (HVG), and difference VG (DVG). These graphs were used to characterize different EEG seizure patterns. To demonstrate its effectiveness in identifying EEG seizure patterns and detecting generalized seizures, EEG recordings of 615h on one EEG channel from 29 epileptic patients with ID were analyzed. A novel feature set with discriminative power for seizure detection was obtained by using the VGS method. The degree distributions (DDs) of DVG can clearly distinguish EEG of each seizure pattern. The degree entropy and power-law degree power in DVG were proposed here for the first time, and they show significant difference between seizure and non-seizure EEG. The connecting structure measured by HVG can better distinguish seizure EEG from background than those by VG and DVG. A traditional EEG feature set based on frequency analysis was used here as a benchmark feature set. With a support vector machine (SVM) classifier, the seizure detection performance of the benchmark feature set (sensitivity of 24%, FD t /h of 1.8s) can be improved by combining our proposed VGS features extracted from one EEG channel (sensitivity of 38%, FD t /h of 1.4s). The proposed VGS-based features can help improve seizure detection for ID patients. Copyright © 2017 Elsevier B.V. All rights reserved.

  17. Automatic epileptic seizure detection using scalp EEG and advanced artificial intelligence techniques.

    Science.gov (United States)

    Fergus, Paul; Hignett, David; Hussain, Abir; Al-Jumeily, Dhiya; Abdel-Aziz, Khaled

    2015-01-01

    The epilepsies are a heterogeneous group of neurological disorders and syndromes characterised by recurrent, involuntary, paroxysmal seizure activity, which is often associated with a clinicoelectrical correlate on the electroencephalogram. The diagnosis of epilepsy is usually made by a neurologist but can be difficult to be made in the early stages. Supporting paraclinical evidence obtained from magnetic resonance imaging and electroencephalography may enable clinicians to make a diagnosis of epilepsy and investigate treatment earlier. However, electroencephalogram capture and interpretation are time consuming and can be expensive due to the need for trained specialists to perform the interpretation. Automated detection of correlates of seizure activity may be a solution. In this paper, we present a supervised machine learning approach that classifies seizure and nonseizure records using an open dataset containing 342 records. Our results show an improvement on existing studies by as much as 10% in most cases with a sensitivity of 93%, specificity of 94%, and area under the curve of 98% with a 6% global error using a k-class nearest neighbour classifier. We propose that such an approach could have clinical applications in the investigation of patients with suspected seizure disorders.

  18. Gelastic seizures associated with hypothalamic hamartomas. An update in the clinical presentation, diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    José F. Tellez-Zenteno

    2008-10-01

    Full Text Available José F. Tellez-Zenteno1, Cesar Serrano-Almeida2, Farzad Moien-Afshari11Division of Neurology, University of Saskatchewan, Saskatoon, Saskatchewan, Canada; 2Department of Clinical Neurosciences, University of Calgary, Calgary, Alberta, CanadaAbstract: Gelastic seizures are epileptic events characterized by bouts of laughter. Laughter-like vocalization is usually combined with facial contraction in the form of a smile. Autonomic features such as flushing, tachycardia, and altered respiration are widely recognized. Conscious state may not be impaired, although this is often difficult to asses particularly in young children. Gelastic seizures have been associated classically to hypothalamic hamartomas, although different extrahypothalamic localizations have been described. Hypothalamic hamartomas are rare congenital lesions presenting with the classic triad of gelastic epilepsy, precocious puberty and developmental delay. The clinical course of patients with gelastic seizures associated with hypothalamic hamartomas is progressive, commencing with gelastic seizures in infancy, deteriorating into more complex seizure disorder resulting in intractable epilepsy. Electrophysiological, radiological, and pathophysiological studies have confirmed the intrinsic epileptogenicity of the hypothalamic hamartoma. Currently the most effective surgical approach is the trancallosal anterior interforniceal approach, however newer approaches including the endoscopic and other treatment such as radiosurgery and gamma knife have been used with success. This review focuses on the syndrome of gelastic seizures associated with hypothalamic hamartomas, but it also reviews other concepts such as status gelasticus and some aspects of gelastic seizures in other locations.Keywords: epilepsy, gelastic seizures, epilepsy surgery, hypothalamic hamartoma, intractable epilepsy

  19. Neurodevelopmental comorbidities and seizure control 24 months after a first unprovoked seizure in children.

    Science.gov (United States)

    Jason, Eva Åndell; Tomson, Torbjörn; Carlsson, Sofia; Tedroff, Kristina; Åmark, Per

    2018-07-01

    To follow children with newly diagnosed unprovoked seizures to determine (1) whether the prevalence of neurodevelopmental comorbidities and cerebral palsy (CP) changed after the initial seizure, and (2) the association between studied comorbidities and seizures 13-24 months after seizure onset or initiation of treatment. Analyses were based on 750 children (28 days-18 years) with a first unprovoked seizure (index) included in a population-based Incidence Registry in Stockholm between 2001 and 2006. The children were followed for two years and their medical records were examined for a priori defined neurodevelopmental/psychiatric comorbidities and CP and seizure frequency. Baseline information was collected from medical records from before, and up to six months after, the index seizure. Odds ratios (OR) of repeated seizures 13-24 months after the first seizure or after initiation of anti-epileptic drug treatment was calculated by logistic regression and adjusted for age and sex. At baseline, 32% of the children had neurodevelopmental/psychiatric comorbidities or CP compared to 35%, 24 months later. Children with such comorbidities more often experienced seizures 13-24 months after the index seizure (OR 2.87, CI 2.07-3.99) with the highest OR in those with CP or attention deficit hyperactivity disorder (ADHD). Children diagnosed at age neurodevelopmental comorbidities and CP in children with epilepsy tend to be present already at seizure onset and that such comorbidities are strong indicators of poor outcome regarding seizure control with or without treatment. Copyright © 2018 Elsevier B.V. All rights reserved.

  20. Novel PIGT Variant in Two Brothers: Expansion of the Multiple Congenital Anomalies-Hypotonia Seizures Syndrome 3 Phenotype

    Directory of Open Access Journals (Sweden)

    Nadia Skauli

    2016-11-01

    Full Text Available Biallelic PIGT variants were previously reported in seven patients from three families with Multiple Congenital Anomalies-Hypotonia Seizures Syndrome 3 (MCAHS3, characterized by epileptic encephalopathy, hypotonia, global developmental delay/intellectual disability, cerebral and cerebellar atrophy, craniofacial dysmorphisms, and skeletal, ophthalmological, cardiac, and genitourinary abnormalities. We report a novel homozygous PIGT missense variant c.1079G>T (p.Gly360Val in two brothers with several of the typical features of MCAHS3, but in addition, pyramidal tract neurological signs. Notably, they are the first patients with MCAHS3 without skeletal, cardiac, or genitourinary anomalies. PIGT encodes a crucial subunit of the glycosylphosphatidylinositol (GPI transamidase complex, which catalyzes the attachment of proteins to GPI-anchors, attaching the proteins to the cell membrane. In vitro studies in cells from the two brothers showed reduced levels of GPI-anchors and GPI-anchored proteins on the cell surface, supporting the pathogenicity of the novel PIGT variant.

  1. "Capgras" Delusions Involving Belongings, Not People, and Evolving Visual Hallucinations Associated with Occipital Lobe Seizures.

    Science.gov (United States)

    Lilly, Brandon; Maynard, Erika; Melvin, Kelly; Holroyd, Suzanne

    2018-01-01

    Capgras syndrome is characterized by the delusional belief that a familiar person has been replaced by a visually similar imposter or replica. Rarely, the delusional focus may be objects rather than people. Numerous etiologies have been described for Capgras to include seizures. Similarly, visual hallucinations, both simple and complex, can occur secondary to seizure activity. We present, to our knowledge, the first reported case of visual hallucinations and Capgras delusions for objects that developed secondary to new onset occipital lobe epilepsy. We then discuss the possible underlying neurologic mechanisms responsible for the symptomatology.

  2. Diagnostic and Therapeutic Management of a First Unprovoked Seizure in Children and Adolescents With a Focus on the Revised Diagnostic Criteria for Epilepsy.

    Science.gov (United States)

    Sansevere, Arnold J; Avalone, Jennifer; Strauss, Lauren Doyle; Patel, Archana A; Pinto, Anna; Ramachandran, Maya; Fernandez, Ivan Sanchez; Bergin, Ann M; Kimia, Amir; Pearl, Phillip L; Loddenkemper, Tobias

    2017-07-01

    By definition, unprovoked seizures are not precipitated by an identifiable factor, such as fever or trauma. A thorough history and physical examination are essential to caring for pediatric patients with a potential first unprovoked seizure. Differential diagnosis, EEG, neuroimaging, laboratory tests, and initiation of treatment will be reviewed. Treatment is typically initiated after 2 unprovoked seizures, or after 1 seizure in select patients with distinct epilepsy syndromes. Recent expansion of the definition of epilepsy by the ILAE allows for the diagnosis of epilepsy to be made after the first seizure if the clinical presentation and supporting diagnostic studies suggest a greater than 60% chance of a second seizure. This review summarizes the current literature on the diagnostic and therapeutic management of first unprovoked seizure in children and adolescents while taking into consideration the revised diagnostic criteria of epilepsy.

  3. Epidemiology of early stages of epilepsy: Risk of seizure recurrence after a first seizure.

    Science.gov (United States)

    Rizvi, Syed; Ladino, Lady Diana; Hernandez-Ronquillo, Lizbeth; Téllez-Zenteno, José F

    2017-07-01

    A single unprovoked seizure is a frequent phenomenon in the general population and the rate of seizure recurrence can vary widely. Individual risk prognostication is crucial in predicting patient outcomes and guiding treatment decisions. In this article, we review the most important risk factors associated with an increased likelihood of seizure recurrence after a single unprovoked seizure. In summary, the presence of focal seizure, nocturnal seizure, history of prior brain injury, family history of epilepsy, abnormal neurological exam, epileptiform discharges on electroencephalography and neuroimaging abnormalities, portend increased risk of seizure recurrence. Elucidation of these risk factors in patient assessment will augment clinical decision-making and may help determine the appropriateness of instituting anti-epilepsy treatment. We also discuss the Canadian model of single seizure clinics and the potential use to assess these patients. Copyright © 2017. Published by Elsevier Ltd.

  4. 11p Microdeletion including WT1 but not PAX6, presenting with cataract, mental retardation, genital abnormalities and seizures: a case report

    Directory of Open Access Journals (Sweden)

    Baekgaard Peter

    2009-02-01

    Full Text Available Abstract WAGR syndrome (Wilms' tumor, aniridia, genitourinary abnormalities and mental retardation and Potocki-Shaffer syndrome are rare contiguous gene deletion syndromes caused by deletions of the 11p14-p12 chromosome region. We present a patient with mental retardation, unilateral cataract, bilateral ptosis, genital abnormalities, seizures and a dysmorphic face. Cytogenetic analysis showed a deletion on 11p that was further characterized using FISH and MLPA analyses. The deletion (11p13-p12 located in the area between the deletions associated with the WAGR and Potocki-Shaffer syndromes had a maximum size of 8.5 Mb and encompasses 44 genes. Deletion of WT1 explains the genital abnormalities observed. As PAX6 was intact the cataract observed cannot be explained by a deletion of this gene. Seizures have been described in Potocki-Shaffer syndrome while mental retardation has been described in both WAGR and Potocki-Shaffer syndrome. Characterization of this patient contributes further to elucidate the function of the genes in the 11p14-p12 chromosome region.

  5. How do we diagnose and treat epilepsy with myoclonic-atonic seizures (Doose syndrome)? Results of the Pediatric Epilepsy Research Consortium survey.

    Science.gov (United States)

    Nickels, Katherine; Thibert, Ronald; Rau, Stephanie; Demarest, Scott; Wirrell, Elaine; Kossoff, Eric H; Joshi, Charuta; Nangia, Srishti; Shellhaas, Renee

    2018-04-25

    To obtain and assess opinions on EMAS diagnostic criteria, recommended investigations, and therapeutic options, from a large group of physicians who care for children with EMAS. The EMAS focus group of PERC created a survey to assess the opinions of pediatric neurologists who care for children with EMAS regarding diagnosis and treatment of this condition, which was sent to members of PERC, AES, and CNS. A Likert scale was used to assess the respondents' opinions on the importance of diagnostic and exclusion criteria (five point scale), investigations (four point scale), and treatment (six point scale) of EMAS. Inclusion/exclusion criteria were then classified as critical, strong, or modest. Investigations were classified as essential, recommended, or possible. Therapies were classified as first line, beneficial, indeterminate benefit, or contraindicated. Survey results from the 76 participants determined the following: EMAS inclusion criteria: history suggestive of MAS (critical), recorded or home video suggestive of MAS, generalized discharges on inter-ictal EEG, normal neuroimaging, normal development prior to seizure onset (strong). EMAS exclusionary criteria: epileptic spasms, abnormal neuroimaging, focal abnormal exam, seizure onset six years (strong). EEG and MRI (essential), amino acids, organic acids, fatty acid/acylcarnitine profile, microarray, genetic panel, lactate/pyruvate, CSF and serum glucose/lactate (strong). Valproic acid (first line), topiramate, zonisamide, levetiracetam, benzodiazepines, and dietary therapies (beneficial). To date, no similar surveys have been published, even though early syndrome identification and initiation of effective treatment have been associated with improved outcome in EMAS. Medications that exacerbate seizures in EMAS have also been identified. This survey identified critical and preferred diagnostic electro clinical features, investigations, and treatments for EMAS. It will guide future research and is a crucial

  6. Characteristics of the initial seizure in familial febrile seizures

    NARCIS (Netherlands)

    M. van Stuijvenberg (Margriet); E. van Beijeren; N.H. Wils; G. Derksen-Lubsen (Gerarda); C.M. van Duijn (Cornelia); H.A. Moll (Henriëtte)

    1999-01-01

    textabstractComplex seizure characteristics in patients with a positive family history were studied to define familial phenotype subgroups of febrile seizures. A total of 51 children with one or more affected first degree relatives and 177 without an affected first degree

  7. Usefulness of ketogenic diet in a girl with migrating partial seizures in infancy.

    Science.gov (United States)

    Mori, Tatsuo; Imai, Katsumi; Oboshi, Taikan; Fujiwara, Yuh; Takeshita, Saoko; Saitsu, Hirotomo; Matsumoto, Naomichi; Takahashi, Yukitoshi; Inoue, Yushi

    2016-06-01

    Migrating partial seizures in infancy (MPSI) are an age-specific epilepsy syndrome characterized by migrating focal seizures, which are intractable to various antiepileptic drugs and cause severe developmental delay. We report a case of MPSI with heterozygous missense mutation in KCNT1, which was successfully managed by ketogenic diet. At age 2months, the patient developed epilepsy initially manifesting focal seizures with eye deviation and apnea, then evolving to secondarily generalized clonic convulsion. Various antiepileptic drugs including phenytoin, valproic acid, zonisamide, clobazam, levetiracetam, vitamin B6, and carbamazepine were not effective, but high-dose phenobarbital allowed discontinuation of midazolam infusion. Ictal scalp electroencephalogram showed migrating focal seizures. MPSI was suspected and she was transferred to our hospital for further treatment. Potassium bromide (KBr) was partially effective, but the effect was transient. High-dose KBr caused severe adverse effects such as over-sedation and hypercapnia, with no further effects on the seizures. At age 9months, we started a ketogenic diet, which improved seizure frequency and severity without obvious adverse effects, allowing her to be discharged from hospital. Ketogenic diet should be tried in patients with MPSI unresponsive to antiepileptic drugs. In MPSI, the difference in treatment response in patients with and those without KCNT1 mutation remains unknown. Accumulation of case reports would contribute to establish effective treatment options for MPSI. Copyright © 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  8. A review of traditional and novel treatments for seizures in autism spectrum disorder: findings from a systematic review and expert panel.

    Science.gov (United States)

    Frye, Richard E; Rossignol, Daniel; Casanova, Manuel F; Brown, Gregory L; Martin, Victoria; Edelson, Stephen; Coben, Robert; Lewine, Jeffrey; Slattery, John C; Lau, Chrystal; Hardy, Paul; Fatemi, S Hossein; Folsom, Timothy D; Macfabe, Derrick; Adams, James B

    2013-09-13

    Despite the fact that seizures are commonly associated with autism spectrum disorder (ASD), the effectiveness of treatments for seizures has not been well studied in individuals with ASD. This manuscript reviews both traditional and novel treatments for seizures associated with ASD. Studies were selected by systematically searching major electronic databases and by a panel of experts that treat ASD individuals. Only a few anti-epileptic drugs (AEDs) have undergone carefully controlled trials in ASD, but these trials examined outcomes other than seizures. Several lines of evidence point to valproate, lamotrigine, and levetiracetam as the most effective and tolerable AEDs for individuals with ASD. Limited evidence supports the use of traditional non-AED treatments, such as the ketogenic and modified Atkins diet, multiple subpial transections, immunomodulation, and neurofeedback treatments. Although specific treatments may be more appropriate for specific genetic and metabolic syndromes associated with ASD and seizures, there are few studies which have documented the effectiveness of treatments for seizures for specific syndromes. Limited evidence supports l-carnitine, multivitamins, and N-acetyl-l-cysteine in mitochondrial disease and dysfunction, folinic acid in cerebral folate abnormalities and early treatment with vigabatrin in tuberous sclerosis complex. Finally, there is limited evidence for a number of novel treatments, particularly magnesium with pyridoxine, omega-3 fatty acids, the gluten-free casein-free diet, and low-frequency repetitive transcranial magnetic simulation. Zinc and l-carnosine are potential novel treatments supported by basic research but not clinical studies. This review demonstrates the wide variety of treatments used to treat seizures in individuals with ASD as well as the striking lack of clinical trials performed to support the use of these treatments. Additional studies concerning these treatments for controlling seizures in individuals

  9. A review of traditional and novel treatments for seizures in autism spectrum disorder: Findings from a systematic review and expert panel.

    Directory of Open Access Journals (Sweden)

    Richard Eugene Frye

    2013-09-01

    Full Text Available Despite the fact that seizures are commonly associated with autism spectrum disorder (ASD, the effectiveness of treatments for seizures has not been well studied in individuals with ASD. This manuscript reviews both traditional and novel treatments for seizures associated with ASD. Studies were selected by systematically searching major electronic databases and by a panel of experts that treat ASD individuals. Only a few anti-epileptic drugs (AEDs have undergone carefully controlled trials in ASD, but these trials examined outcomes other than seizures. Several lines of evidence point to valproate, lamotrigine and levetiracetam as the most effective and tolerable AEDs for individuals with ASD. Limited evidence supports the use of traditional non-AED treatments, such as the ketogenic and modified Atkins diet, multiple subpial transections and immunomodulation and neurofeedback treatments. Although specific treatments may be more appropriate for specific genetic and metabolic syndromes associated with ASD and seizures, there are few studies which have documented the effectiveness of treatments for seizures for specific syndromes. Limited evidence supports L-carnitine, multivitamins and N-acetyl-L-cysteine in mitochondrial disease and dysfunction, folinic acid in cerebral folate abnormalities and early treatment with vigabatrin in tuberous sclerosis complex. Finally, there is limited evidence for a number of novel treatments, particularly magnesium with pyridoxine, omega-3 fatty acids, the gluten-free casein-free diet and transcranial magnetic simulation. Zinc and L-carnosine are potential novel treatments supported by basic research but not clinical studies. This review demonstrates the wide variety of treatments used to treat seizures in individuals with ASD as well as the striking lack of clinical trials performed to support the use these treatments. Additional studies concerning these treatments for controlling seizures in individuals with ASD

  10. Generalized tonic-clonic seizure

    Science.gov (United States)

    ... tonic-clonic seizures have vision, taste, smell, or sensory changes, hallucinations, or dizziness before the seizure. This ... longer (called the post-ictal state) Loss of memory (amnesia) about the seizure episode Headache Weakness of ...

  11. Seizure detection, seizure prediction, and closed-loop warning systems in epilepsy.

    Science.gov (United States)

    Ramgopal, Sriram; Thome-Souza, Sigride; Jackson, Michele; Kadish, Navah Ester; Sánchez Fernández, Iván; Klehm, Jacquelyn; Bosl, William; Reinsberger, Claus; Schachter, Steven; Loddenkemper, Tobias

    2014-08-01

    Nearly one-third of patients with epilepsy continue to have seizures despite optimal medication management. Systems employed to detect seizures may have the potential to improve outcomes in these patients by allowing more tailored therapies and might, additionally, have a role in accident and SUDEP prevention. Automated seizure detection and prediction require algorithms which employ feature computation and subsequent classification. Over the last few decades, methods have been developed to detect seizures utilizing scalp and intracranial EEG, electrocardiography, accelerometry and motion sensors, electrodermal activity, and audio/video captures. To date, it is unclear which combination of detection technologies yields the best results, and approaches may ultimately need to be individualized. This review presents an overview of seizure detection and related prediction methods and discusses their potential uses in closed-loop warning systems in epilepsy. Copyright © 2014. Published by Elsevier Inc.

  12. HMPAO-SPECT in cerebral seizures

    International Nuclear Information System (INIS)

    Gruenwald, F.; Bockisch, A.; Reichmann, K.; Ammari, B.; Hotze, A.; Biersack, H.J.; Durwen, H.; Buelau, P.; Elger, C.E.; Rohde, A.; Penin, H.

    1988-01-01

    In nine patients with suspected psychogenic seizures and in three patients with proven epileptic seizures HMPAO-SPECT was performed prior to and during seizure. In the patients with lateron-proven psychogenic seizures no, or only slight, changes of regional cerebral blood flow were found. Patients with proven epilepsy revealed partly normal findings interictally but during seizure a markedly increased circumscript blood flow was found in all patients. Even though PET is superior to SPECT with respect to spatial resolution, in the diagnosis of seizures HMPAO-SPECT has the advantage of enabling injection of the tracer during the seizure and the performance of the SPECT study subsequently. (orig.) [de

  13. Seizure phenotypes, periodicity, and sleep-wake pattern of seizures in Kcna-1 null mice.

    Science.gov (United States)

    Wright, Samantha; Wallace, Eli; Hwang, Youngdeok; Maganti, Rama

    2016-02-01

    This study was undertaken to describe seizure phenotypes, natural progression, sleep-wake patterns, as well as periodicity of seizures in Kcna-1 null mutant mice. These mice were implanted with epidural electroencephalography (EEG) and electromyography (EMG) electrodes, and simultaneous video-EEG recordings were obtained while animals were individually housed under either diurnal (LD) condition or constant darkness (DD) over ten days of recording. The video-EEG data were analyzed to identify electrographic and behavioral phenotypes and natural progression and to examine the periodicity of seizures. Sleep-wake patterns were analyzed to understand the distribution and onset of seizures across the sleep-wake cycle. Four electrographically and behaviorally distinct seizure types were observed. Regardless of lighting condition that animals were housed in, Kcna-1 null mice initially expressed only a few of the most severe seizure types that progressively increased in frequency and decreased in seizure severity. In addition, a circadian periodicity was noted, with seizures peaking in the first 12h of the Zeitgeber time (ZT) cycle, regardless of lighting conditions. Interestingly, seizure onset differed between lighting conditions where more seizures arose out of sleep in LD conditions, whereas under DD conditions, the majority occurred out of the wakeful state. We suggest that this model be used to understand the circadian pattern of seizures as well as the pathophysiological implications of sleep and circadian disturbances in limbic epilepsies. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Uric acid is released in the brain during seizure activity and increases severity of seizures in a mouse model for acute limbic seizures.

    Science.gov (United States)

    Thyrion, Lisa; Raedt, Robrecht; Portelli, Jeanelle; Van Loo, Pieter; Wadman, Wytse J; Glorieux, Griet; Lambrecht, Bart N; Janssens, Sophie; Vonck, Kristl; Boon, Paul

    2016-03-01

    Recent evidence points at an important role of endogenous cell-damage induced pro-inflammatory molecules in the generation of epileptic seizures. Uric acid, under the form of monosodium urate crystals, has shown to have pro-inflammatory properties in the body, but less is known about its role in seizure generation. This study aimed to unravel the contribution of uric acid to seizure generation in a mouse model for acute limbic seizures. We measured extracellular levels of uric acid in the brain and modulated them using complementary pharmacological and genetic tools. Local extracellular uric acid levels increased three to four times during acute limbic seizures and peaked between 50 and 100 min after kainic acid infusion. Manipulating uric acid levels through administration of allopurinol or knock-out of urate oxidase significantly altered the number of generalized seizures, decreasing and increasing them by a twofold respectively. Taken together, our results consistently show that uric acid is released during limbic seizures and suggest that uric acid facilitates seizure generalization. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. Sequential fluctuating paraneoplastic ocular flutter-opsoclonus-myoclonus syndrome and Lambert-Eaton myasthenic syndrome in small-cell lung cancer.

    LENUS (Irish Health Repository)

    Simister, Robert J

    2011-03-01

    Paraneoplastic cerebellar degeneration may occur in association with Lambert-Eaton myasthenic syndrome (LEMS), but to our knowledge, the co-occurrence of paraneoplastic opsoclonus-myoclonus syndrome and LEMS has not been previously reported. A 67-year-old woman presented with a complex partial seizure and evolving ocular flutter, opsoclonus, myoclonus and \\'cerebellar\\' signs, all of which improved spontaneously within 6 weeks. Approximately 8 weeks after symptom onset, the patient became encephalopathic, she had a further complex partial seizure, and she became areflexic with potentiation of deep tendon reflexes. Radiological, bronchoscopic and histological investigations revealed small-cell lung cancer, and neurophysiological investigations confirmed a diagnosis of LEMS. High-titre anti-P\\/Q-type voltage-gated calcium-channel antibodies were identified in the serum, which increased as the signs of opsoclonus and myoclonus resolved. The encephalopathy and clinical features of LEMS responded dramatically to chemotherapy and radiotherapy. Spontaneous improvement of paraneoplastic opsoclonus-myoclonus syndrome may occur, and this syndrome may occur in association with LEMS. Antivoltage-gated calcium-channel antibodies are not implicated in the pathogenesis of paraneoplastic opsoclonus-myoclonus syndrome.

  16. Controlled-release oxycodone-induced seizures.

    Science.gov (United States)

    Klein, Moti; Rudich, Zvia; Gurevich, Boris; Lifshitz, Matityahu; Brill, Silviu; Lottan, Michael; Weksler, Natan

    2005-11-01

    The use of the opioid oxycodone hydrochloride in the management of chronic pain is gaining popularity principally because of its tolerability. However, opioid-related seizure in patients with epilepsy or other conditions that may decrease seizure threshold has been described in the literature; in particular, oxycodone has been associated with seizure in a patient with acute renal failure. The aim of this article was to report a patient with a history of seizures but normal renal and hepatic function who developed seizure on 2 occasions after oxycodone ingestion. A 54-year-old male patient presented with a history of tonic-clonic seizures that developed immediately after intracranial surgery. Long-term treatment with carbamazepine 400 mg QD was started, and the patient was free of convulsions for approximately 7 years. The patient presented to us with severe headache that was nonresponsive to an NSAID and the opiate agonist tramadol. Treatment with controlled-release (CR) oxycodone and tramadol drops (50 mg QID if necessary) was started, and tonic-clonic seizures developed 3 days later. Based on laboratory analysis, the patient had normal renal and hepatic function. On discontinuation of oxycodone treatment, the seizures resolved. However, due to effective pain relief with oxycodone, the patient decided to continue treatment, and seizures recurred. Carbamazepine was then administered 4 hours before oxycodone dosing, which allowed continuation of treatment without seizure. A patient with a history of seizures controlled with long-term carbamazepine therapy developed seizures when he started treatment with oxycodone CR at recommended doses. Oxycodone CR should be used with extreme caution in patients with epilepsy or other conditions that may decrease seizure threshold.

  17. Fibromyalgia and seizures.

    Science.gov (United States)

    Tatum, William O; Langston, Michael E; Acton, Emily K

    2016-06-01

    The purpose of this case-matched study was to determine how frequently fibromyalgia is associated with different paroxysmal neurological disorders and explore the utility of fibromyalgia as a predictor for the diagnosis of psychogenic non-epileptic seizures. The billing diagnosis codes of 1,730 new, non-selected patient encounters were reviewed over a three-year period for an epileptologist in a neurology clinic to identify all patients with historical diagnoses of fibromyalgia. The frequency with which epileptic seizures, psychogenic non-epileptic seizures, and physiological non-epileptic events were comorbid with fibromyalgia was assessed. Age and gender case-matched controls were used for a between-group comparison. Wilcoxon tests were used to analyse interval data, and Chi-square was used to analyse categorical data (pFibromyalgia was retrospectively identified in 95/1,730 (5.5%) patients in this cohort. Females represented 95% of the fibromyalgia sample (age: 53 years; 95% CI: 57, 51). Forty-three percent of those with fibromyalgia had a non-paroxysmal, neurological primary clinical diagnosis, most commonly chronic pain. Paroxysmal events were present in 57% of fibromyalgia patients and 54% of case-matched controls. Among patients with fibromyalgia and paroxysmal disorders, 11% had epileptic seizures, 74% had psychogenic non-epileptic seizures, and 15% had physiological non-epileptic events, compared to case-matched controls with 37% epileptic seizures, 51% psychogenic non-epileptic events, and 12% physiological non-epileptic events (p = 0.009). Fibromyalgia was shown to be a predictor for the diagnosis of psychogenic non-epileptic seizures in patients with undifferentiated paroxysmal spells. However, our results suggest that the specificity and sensitivity of fibromyalgia as a marker for psychogenic non-epileptic seizures in a mixed general neurological population of patients is less than previously described.

  18. Dyke Davidoff Masson Syndrome: A case report

    Directory of Open Access Journals (Sweden)

    More Sumeet S, Jadhav Aravinash L, Garkal Shailendra M, Tewari Suresh C

    2013-04-01

    Full Text Available Dyke-Davidoff-Masson syndrome (DDMS is characterized by seizures, facial asymmetry, contralateral hemiplegia and mental retardation. The characteristic radiologic features are cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. We report a case of DDMS in an 18years old girl who presented with a history of generalized tonic – clonic seizures, hemiparesis and hemiatrophy of right side.

  19. Epileptic seizures in Neuro-Behcet disease: why some patients develop seizure and others not?

    Science.gov (United States)

    Kutlu, Gulnihal; Semercioglu, Sencer; Ucler, Serap; Erdal, Abidin; Inan, Levent E

    2015-03-01

    Behcet disease (BD) is a chronic relapsing inflammatory disorder. Neuro BD (NBD) is seen in approximately 5% of all patients. The aim of this study is to investigate the frequency, type and prognosis of epileptic seizures in different forms of NBD. All files of 42 patients with NBD were evaluated between 2006 and 2012, retrospectively. The demographic data, the presentation of NBD, clinical findings including seizures, EEG and neuroimaging findings were reviewed. The mean age of patients was 35.02±8.43 years. Thirty (71.4%) patients were male; the remaining 12 of them were female. Twenty-four patients had brainstem lesions; 16 patients had cerebral venous thrombosis. Spinal cord involvement was seen in two patients. Seven patients had epileptic seizures (six partial onset seizures with or without secondary generalization). Six of them had cerebral sinus thrombosis (CVT). Four patients had a seizure as the first symptom of the thrombosis. One patient had late onset seizure due to chronic venous infarct. The other patient with seizure had brainstem involvement. The remaining was diagnosed as epilepsy before the determination of NBD. CVT seen in BD seems to be the main risk factor for epileptic seizures in patients with NBD. The prognosis is usually good especially in patients with CVT. Epileptic seizures in patients with brainstem involvement may be an indicator for poor prognosis. Superior sagittal thrombosis or cortical infarct would be predictor of seizures occurrence because of the high ratio in patients with seizures. Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  20. 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

  1. Do reflex seizures and spontaneous seizures form a continuum? - triggering factors and possible common mechanisms.

    Science.gov (United States)

    Irmen, Friederike; Wehner, Tim; Lemieux, Louis

    2015-02-01

    Recent changes in the understanding and classification of reflex seizures have fuelled a debate on triggering mechanisms of seizures and their conceptual organization. Previous studies and patient reports have listed extrinsic and intrinsic triggers, albeit their multifactorial and dynamic nature is poorly understood. This paper aims to review literature on extrinsic and intrinsic seizure triggers and to discuss common mechanisms among them. Among self-reported seizure triggers, emotional stress is most frequently named. Reflex seizures are typically associated with extrinsic sensory triggers; however, intrinsic cognitive or proprioceptive triggers have also been assessed. The identification of a trigger underlying a seizure may be more difficult if it is intrinsic and complex, and if triggering mechanisms are multifactorial. Therefore, since observability of triggers varies and triggers are also found in non-reflex seizures, the present concept of reflex seizures may be questioned. We suggest the possibility of a conceptual continuum between reflex and spontaneous seizures rather than a dichotomy and discuss evidence to the notion that to some extent most seizures might be triggered. Copyright © 2014 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  2. Morphine potentiates seizures induced by GABA antagonists and attenuates seizures induced by electroshock in the rat.

    Science.gov (United States)

    Foote, F; Gale, K

    1983-11-25

    In a naloxone-reversible, dose-dependent manner, morphine (10-50 mg/kg i.p.) protected against seizures induced by maximal electroshock and increased the incidence and severity of seizures induced by bicuculline, in rats. Morphine also potentiated seizures induced by isoniazid and by picrotoxin. Thus, opiate activity influences the expression of seizures in contrasting ways depending upon the mode of seizure induction. Since morphine consistently potentiated seizures induced by interference with GABA transmission, it appears that GABAergic systems may be of particular significance for the elucidation of the varied effects of morphine on seizure susceptibility.

  3. Multifractal detrended cross-correlation analysis for epileptic patient in seizure and seizure free status

    International Nuclear Information System (INIS)

    Ghosh, Dipak; Dutta, Srimonti; Chakraborty, Sayantan

    2014-01-01

    Highlights: • We analyze EEG of patients during seizure and in seizure free interval. • Data from different sections of the brain and seizure activity was analyzed. • Assessment of cross-correlation in seizure and seizure free interval using MF-DXA technique. - Abstract: This paper reports a study of EEG data of epileptic patients in terms of multifractal detrended cross-correlation analysis (MF-DXA). The EEG clinical data were obtained from the EEG Database available with the Clinic of Epileptology of the University Hospital of Bonn, Germany. The data sets (C, D, and E) were taken from five epileptic patients undergoing presurgical evaluations. The data sets consist of intracranial EEG recordings during seizure-free intervals (interictal periods) from within the epileptogenic zone (D) and from the hippocampal formation of the opposite hemisphere of the epileptic patients’ brain, respectively (C). The data set (E) was recorded during seizure activity (ictal periods). MF-DXA is a very rigorous and robust tool for assessment of cross-correlation among two nonlinear time series. The study reveals the degree of cross-correlation is more among seizure and seizure free interval in epileptogenic zone. These data are very significant for diagnosis, onset and prognosis of epileptic patients

  4. The effects of glycemic control on seizures and seizure-induced excitotoxic cell death

    Directory of Open Access Journals (Sweden)

    Schauwecker Paula

    2012-08-01

    Full Text Available Abstract Background Epilepsy is the most common neurological disorder after stroke, affecting more than 50 million persons worldwide. Metabolic disturbances are often associated with epileptic seizures, but the pathogenesis of this relationship is poorly understood. It is known that seizures result in altered glucose metabolism, the reduction of intracellular energy metabolites such as ATP, ADP and phosphocreatine and the accumulation of metabolic intermediates, such as lactate and adenosine. In particular, it has been suggested that the duration and extent of glucose dysregulation may be a predictor of the pathological outcome of status. However, little is known about neither the effects of glycemic control on brain metabolism nor the effects of managing systemic glucose concentrations in epilepsy. Results In this study, we examined glycemic modulation of kainate-induced seizure sensitivity and its neuropathological consequences. To investigate the relationship between glycemic modulation, seizure susceptibility and its neuropathological consequences, C57BL/6 mice (excitotoxin cell death resistant were subjected to hypoglycemia or hyperglycemia, followed by systemic administration of kainic acid to induce seizures. Glycemic modulation resulted in minimal consequences with regard to seizure severity but increased hippocampal pathology, irrespective of whether mice were hypoglycemic or hyperglycemic prior to kainate administration. Moreover, we found that exogenous administration of glucose following kainic acid seizures significantly reduced the extent of hippocampal pathology in FVB/N mice (excitotoxin cell death susceptible following systemic administration of kainic acid. Conclusion These findings demonstrate that modulation of the glycemic index can modify the outcome of brain injury in the kainate model of seizure induction. Moreover, modulation of the glycemic index through glucose rescue greatly diminishes the extent of seizure

  5. Seizures Induced by Music

    Directory of Open Access Journals (Sweden)

    A. O. Ogunyemi

    1993-01-01

    Full Text Available Musicogenic epilepsy is a rare disorder. Much remains to be learned about the electroclinical features. This report describes a patient who has been followed at our institution for 17 years, and was investigated with long-term telemetered simultaneous video-EEG recordings. She began to have seizures at the age of 10 years. She experienced complex partial seizures, often preceded by elementary auditory hallucination and complex auditory illusion. The seizures occurred in relation to singing, listening to music or thinking about music. She also had occasional generalized tonic clonic seizures during sleep. There was no significant antecedent history. The family history was negative for epilepsy. The physical examination was unremarkable. CT and MRI scans of the brain were normal. During long-term simultaneous video-EEG recordings, clinical and electrographic seizure activities were recorded in association with singing and listening to music. Mathematical calculation, copying or viewing geometric patterns and playing the game of chess failed to evoke seizures.

  6. SLC6A1 Mutation and Ketogenic Diet in Epilepsy With Myoclonic-Atonic Seizures.

    Science.gov (United States)

    Palmer, Samantha; Towne, Meghan C; Pearl, Phillip L; Pelletier, Renee C; Genetti, Casie A; Shi, Jiahai; Beggs, Alan H; Agrawal, Pankaj B; Brownstein, Catherine A

    2016-11-01

    Epilepsy with myoclonic-atonic seizures, also known as myoclonic-astatic epilepsy or Doose syndrome, has been recently linked to variants in the SLC6A1 gene. Epilepsy with myoclonic-atonic seizures is often refractory to antiepileptic drugs, and the ketogenic diet is known for treating medically intractable seizures, although the mechanism of action is largely unknown. We report a novel SLC6A1 variant in a patient with epilepsy with myoclonic-atonic seizures, analyze its effects, and suggest a mechanism of action for the ketogenic diet. We describe a ten-year-old girl with epilepsy with myoclonic-atonic seizures and a de novo SLC6A1 mutation who responded well to the ketogenic diet. She carried a c.491G>A mutation predicted to cause p.Cys164Tyr amino acid change, which was identified using whole exome sequencing and confirmed by Sanger sequencing. High-resolution structural modeling was used to analyze the likely effects of the mutation. The SLC6A1 gene encodes a transporter that removes gamma-aminobutyric acid from the synaptic cleft. Mutations in SLC6A1 are known to disrupt the gamma-aminobutyric acid transporter protein 1, affecting gamma-aminobutyric acid levels and causing seizures. The p.Cys164Tyr variant found in our study has not been previously reported, expanding on the variants linked to epilepsy with myoclonic-atonic seizures. A 10-year-old girl with a novel SLC6A1 mutation and epilepsy with myoclonic-atonic seizures had an excellent clinical response to the ketogenic diet. An effect of the diet on gamma-aminobutyric acid reuptake mediated by gamma-aminobutyric acid transporter protein 1 is suggested. A personalized approach to epilepsy with myoclonic-atonic seizures patients carrying SLC6A1 mutation and a relationship between epilepsy with myoclonic-atonic seizures due to SLC6A1 mutations, GABAergic drugs, and the ketogenic diet warrants further exploration. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Cerebrovascular Diseases and Early Seizure

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    Ayşegül Gündüz

    2006-08-01

    Full Text Available OBJECTIVE: Cerebrovascular disease is one of the important causes of seizures and epilepsy among the advanced age group. Seziures are found to be associated with lesion localization and size in previous studies. METHODS: Here, we aimed to detect prevelance of seizure, relation of seizure and lesion localization, and observed seizure types. RESULTS: Three hundred seventy eight patients with ischemic cerebrovascular disease or intraparenchymal hemorrhage who were followed in Cerrahpasa IVIedical School clinic were studied retrospectively and probability of seizure occurence within 1 month after stroke was evaluated. CONCLUSION: Among 378 patients hospitalized by acute stroke, 339 were diagnosed as ischemic cerebrovascular disease and 39 (10.3% had primary intraparenchymal hematoma. Seizures were observed in 16 patients (4.2%, 2 (%5.1 in intraparenchymal hematoma group and 14 (%4.1 in ischemic cerebrovascular disease. Early seizures were detected in 33% of patients with anterior cerebral artery, in 6.8% of posterior cerebral artery and in 3.3% of middle cerebral artery infarcts and in three patients out of 12 who were known to have epilepsy. Seizure types were secondarily generalised tonic-clonic seizure in nine cases (57%. Among whole group status epilepticus was observed in four patients (1.1%. Conclusion: Early seizure rates are found to be high among patients with anterior cerebral artery infarct and known epilepsy

  8. Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum

    Directory of Open Access Journals (Sweden)

    Prabhas Prasun Giri

    2011-01-01

    Full Text Available Drug rash with eosinophilia and systemic symptoms (DRESS syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS and spontaneous air leak syndrome (pnemothorax and pneumomediastinum. She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.

  9. Infantile ictal apneas in a child with williams-beuren syndrome.

    Science.gov (United States)

    Myers, Kenneth A; McLeod, D Ross; Bello-Espinosa, Luis

    2013-02-01

    Williams-Beuren syndrome is a genetic disorder rarely associated with seizures. The few described cases of Williams-Beuren syndrome and epilepsy have primarily involved infantile spasms and deletions extending beyond the common deletion region for this disorder. We present the case of a 5-week-old child with ictal apneas and typical Williams-Beuren syndrome deletion. Diagnosis was challenging, because the child had cardiac, respiratory, and gastrointestinal abnormalities typically associated with Williams-Beuren syndrome, which are also associated with cyanotic episodes. The results of interictal electroencephalography were normal, illustrating that prolonged electroencephalography is often essential in evaluation of suspected ictal apneas. Seizure freedom was achieved with carbamazepine. Sudden death is seen in Williams-Beuren syndrome, and this case raises the question whether some of these cases may be related to ictal apneas and could potentially be preventable with appropriate pharmaceutical intervention. Copyright © 2013 Elsevier Inc. All rights reserved.

  10. Benign epilepsy with centrotemporal spikes: Relationship between type of seizures and response to medication in a Greek population

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    Anastasia Gkampeta

    2015-01-01

    Full Text Available Purpose: Benign epilepsy with centrotemporal spikes (BECTS is considered to be the most common childhood epileptic syndrome. We studied the relationship between the type of seizures and response to medication in a Greek population. Materials and Methods: We studied 60 neurodevelopmentally normal children diagnosed with BECTS. Children were subdivided into three groups, based on type of seizures: Group A comprised 32 children with generalized tonic-clonic seizures, Group B 19 children with focal seizures and Group C 9 children with focal seizures with secondary generalization. All patients in the present study were started on an antiepileptic medication after the third seizure (sodium valproate, carbamazepine, and oxcarbazepine, and we studied the response to medication. Results: 10 from 13 (76.92% of patients in Group A, 13 from 15 (86.66% patients in Group B, and all 6 patients (100% in Group C started carbamazepine or oxcarbazepine had a favorable respond. Similarly, 16 from 19 (84.2% of patients in Group A, 3 from 4 patients (75% in Group B, and 1 from 3 patients (33.3% in Group C, started sodium valproate responded well to medication. Conclusions: The majority of children responded well to the first antiepileptic treatment and had a favorable outcome, regardless of type of seizures. 88.3% of children became seizure free by 1 or 2 years after seizure onset. These findings are indicative that the type of seizures has no major effect neither in response to antiepileptic treatment or in the final outcome. Further research in a larger number of children is needed.

  11. Epilepsy after Febrile Seizures

    DEFF Research Database (Denmark)

    Seinfeld, S. A.; Pellock, J M; Kjeldsen, Lone Marianne Juel

    2016-01-01

    to evaluate genetic associations of different febrile seizure subtypes. Results Histories of febrile seizures were validated in 1051 twins in 900 pairs. The febrile seizure type was classified as simple, complex, or febrile status epilepticus. There were 61% simple, 12% complex, and 7% febrile status...... epilepticus. There were 78 twins who developed epilepsy. The highest rate of epilepsy (22.2%) occurred in the febrile status epilepticus group. Concordance was highest in simple group. Conclusion A twin with febrile status epilepticus is at the highest risk of developing epilepsy, but simple febrile seizures...

  12. Phenobarbital reduces EEG amplitude and propagation of neonatal seizures but does not alter performance of automated seizure detection.

    Science.gov (United States)

    Mathieson, Sean R; Livingstone, Vicki; Low, Evonne; Pressler, Ronit; Rennie, Janet M; Boylan, Geraldine B

    2016-10-01

    Phenobarbital increases electroclinical uncoupling and our preliminary observations suggest it may also affect electrographic seizure morphology. This may alter the performance of a novel seizure detection algorithm (SDA) developed by our group. The objectives of this study were to compare the morphology of seizures before and after phenobarbital administration in neonates and to determine the effect of any changes on automated seizure detection rates. The EEGs of 18 term neonates with seizures both pre- and post-phenobarbital (524 seizures) administration were studied. Ten features of seizures were manually quantified and summary measures for each neonate were statistically compared between pre- and post-phenobarbital seizures. SDA seizure detection rates were also compared. Post-phenobarbital seizures showed significantly lower amplitude (pphenobarbital reduces both the amplitude and propagation of seizures which may help to explain electroclinical uncoupling of seizures. The seizure detection rate of the algorithm was unaffected by these changes. The results suggest that users should not need to adjust the SDA sensitivity threshold after phenobarbital administration. Copyright © 2016 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.

  13. Grand Mal Seizure

    Science.gov (United States)

    ... grand mal seizures include: A family history of seizure disorders Any injury to the brain from trauma, a ... the risk of birth defects. If you have epilepsy and plan to become pregnant, work with your ...

  14. Frontal Lobe Seizures

    Science.gov (United States)

    ... cause of frontal lobe epilepsy remains unknown. Complications Status epilepticus. Frontal lobe seizures tend to occur in clusters and may provoke a dangerous condition called status epilepticus — in which seizure activity lasts much longer than ...

  15. “Capgras” Delusions Involving Belongings, Not People, and Evolving Visual Hallucinations Associated with Occipital Lobe Seizures

    Directory of Open Access Journals (Sweden)

    Brandon Lilly

    2018-01-01

    Full Text Available Capgras syndrome is characterized by the delusional belief that a familiar person has been replaced by a visually similar imposter or replica. Rarely, the delusional focus may be objects rather than people. Numerous etiologies have been described for Capgras to include seizures. Similarly, visual hallucinations, both simple and complex, can occur secondary to seizure activity. We present, to our knowledge, the first reported case of visual hallucinations and Capgras delusions for objects that developed secondary to new onset occipital lobe epilepsy. We then discuss the possible underlying neurologic mechanisms responsible for the symptomatology.

  16. The prevalence of thyrotoxicosis-related seizures.

    Science.gov (United States)

    Song, Tae-Jin; Kim, Sun-Jung; Kim, Gyu Sik; Choi, Young-Chul; Kim, Won-Joo

    2010-09-01

    Central nervous system dysfunction, such as hyperexcitation, irritability, and disturbance of consciousness, may occur in patients with thyrotoxicosis. There are also a few case reports of seizures attributed to thyrotoxicosis. The objective of the present study was to determine the prevalence of seizures that appeared to be related to the thyrotoxic state in patients with thyrotoxicosis. We retrospectively determined the prevalence and clinical features of seizures in 3382 patients with hyperthyroidism. Among patients with seizures, we excluded those with other causes of seizures or a history of epilepsy. We did not exclude two patients in whom later work-up showed an abnormal magnetic resonance imaging, as their seizures resolved after they became euthyroid. Among the 3382 patients with hyperthyroidism, there were seven patients (0.2%) with seizures who met our criteria. Primary generalized tonic-clonic seizures occurred in four patients (57%), complex partial seizures with secondary generalized tonic-clonic seizures occurred in two patients (29%), and one patient had a focal seizure (14%). The initial electroencephalography (EEG) was normal in two patients (29%), had generalized slow activity in four patients (57%), and had diffuse generalized beta activity in one patient (14%). On magnetic resonance imaging, one patient had diffuse brain atrophy, and one had an old basal ganglia infarct. After the patients became euthyroid, the EEG was repeated and was normal in all patients. During follow-up periods ranging from 18 to 24 months, none of the patients had seizures. Hyperthyroidism is the precipitating cause of seizures in a small percentage of these patients. In these patients, the prognosis is good if they become euthyroid. The prevalence of thyrotoxicosis-related seizures reported here can be used in conjunction with the prevalence of thyrotoxicosis in the population to estimate the prevalence of thyrotoxicosis-related seizures in populations.

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

    Science.gov (United States)

    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.

  18. Proteus syndrome: craniofacial and cerebral MRI

    International Nuclear Information System (INIS)

    DeLone, D.R.; Brown, W.D.; Gentry, L.R.

    1999-01-01

    The Proteus syndrome is a rare hamartoneoplastic syndrome that may affect the brain, skull, and extracranial head and neck. We present a case with severe, characteristic findings. Brain abnormalities are not common in Proteus syndrome; when present, hemimegalencephaly and migrational disorders are typically seen, commonly with an associated seizure disorder. Maxillary and mandibular dysmorphism may occur, including unilateral condylar hyperplasia. Subcutaneous fatty, fibrous, lymphangiomatous masses commonly seen in this syndrome may involve the neck and face, leading to disfigurement and potential airway compromise. (orig.)

  19. Seizure Prediction and its Applications

    Science.gov (United States)

    Iasemidis, Leon D.

    2011-01-01

    Epilepsy is characterized by intermittent, paroxysmal, hypersynchronous electrical activity, that may remain localized and/or spread and severely disrupt the brain’s normal multi-task and multi-processing function. Epileptic seizures are the hallmarks of such activity and had been considered unpredictable. It is only recently that research on the dynamics of seizure generation by analysis of the brain’s electrographic activity (EEG) has shed ample light on the predictability of seizures, and illuminated the way to automatic, prospective, long-term prediction of seizures. The ability to issue warnings in real time of impending seizures (e.g., tens of minutes prior to seizure occurrence in the case of focal epilepsy), may lead to novel diagnostic tools and treatments for epilepsy. Applications may range from a simple warning to the patient, in order to avert seizure-associated injuries, to intervention by automatic timely administration of an appropriate stimulus, for example of a chemical nature like an anti-epileptic drug (AED), electromagnetic nature like vagus nerve stimulation (VNS), deep brain stimulation (DBS), transcranial direct current (TDC) or transcranial magnetic stimulation (TMS), and/or of another nature (e.g., ultrasonic, cryogenic, biofeedback operant conditioning). It is thus expected that seizure prediction could readily become an integral part of the treatment of epilepsy through neuromodulation, especially in the new generation of closed-loop seizure control systems. PMID:21939848

  20. Improving staff response to seizures on the epilepsy monitoring unit with online EEG seizure detection algorithms.

    Science.gov (United States)

    Rommens, Nicole; Geertsema, Evelien; Jansen Holleboom, Lisanne; Cox, Fieke; Visser, Gerhard

    2018-05-11

    User safety and the quality of diagnostics on the epilepsy monitoring unit (EMU) depend on reaction to seizures. Online seizure detection might improve this. While good sensitivity and specificity is reported, the added value above staff response is unclear. We ascertained the added value of two electroencephalograph (EEG) seizure detection algorithms in terms of additional detected seizures or faster detection time. EEG-video seizure recordings of people admitted to an EMU over one year were included, with a maximum of two seizures per subject. All recordings were retrospectively analyzed using Encevis EpiScan and BESA Epilepsy. Detection sensitivity and latency of the algorithms were compared to staff responses. False positive rates were estimated on 30 uninterrupted recordings (roughly 24 h per subject) of consecutive subjects admitted to the EMU. EEG-video recordings used included 188 seizures. The response rate of staff was 67%, of Encevis 67%, and of BESA Epilepsy 65%. Of the 62 seizures missed by staff, 66% were recognized by Encevis and 39% by BESA Epilepsy. The median latency was 31 s (staff), 10 s (Encevis), and 14 s (BESA Epilepsy). After correcting for walking time from the observation room to the subject, both algorithms detected faster than staff in 65% of detected seizures. The full recordings included 617 h of EEG. Encevis had a median false positive rate of 4.9 per 24 h and BESA Epilepsy of 2.1 per 24 h. EEG-video seizure detection algorithms may improve reaction to seizures by improving the total number of seizures detected and the speed of detection. The false positive rate is feasible for use in a clinical situation. Implementation of these algorithms might result in faster diagnostic testing and better observation during seizures. Copyright © 2018. Published by Elsevier Inc.

  1. Randomized open-label trial of dextromethorphan in Rett syndrome.

    Science.gov (United States)

    Smith-Hicks, Constance L; Gupta, Siddharth; Ewen, Joshua B; Hong, Manisha; Kratz, Lisa; Kelley, Richard; Tierney, Elaine; Vaurio, Rebecca; Bibat, Genila; Sanyal, Abanti; Yenokyan, Gayane; Brereton, Nga; Johnston, Michael V; Naidu, Sakkubai

    2017-10-17

    To determine safety and perform a preliminary assessment of dose-dependent efficacy of dextromethorphan in normalizing electrographic spikes, clinical seizures, and behavioral and cognitive functions in girls with Rett syndrome. We used a prospective randomized, open-label trial in fast metabolizers of dextromethorphan to examine the effect of dextromethorphan on core clinical features of Rett syndrome. Interictal spike activity and clinical seizures were determined using EEG and parent reporting. Cognitive data were obtained using the Mullen Scales of Early Learning and Vineland Adaptive Behavior Scales, while behavioral data were obtained from parent-completed checklists, the Aberrant Behavior Checklist-Community Version, and the Screen for Social Interaction. Anthropometric data were obtained according to the National Health and Nutrition Examination Survey. The Rett Syndrome Severity Scale provided a clinical global impression of the effect of dextromethorphan on clinical severity. Dextromethorphan is safe for use in 3- to 15-year-old girls with Rett syndrome. Thirty-five girls were treated with 1 of 3 doses of dextromethorphan over a period of 6 months. Statistically significant dose-dependent improvements were seen in clinical seizures, receptive language, and behavioral hyperactivity. There was no significant improvement in global clinical severity as measured by the Rett Syndrome Severity Scale. Dextromethorphan is a potent noncompetitive antagonist of the NMDA receptor channel that is safe for use in young girls with Rett syndrome. Preliminary evidence suggests that dextromethorphan may improve some core features of Rett syndrome. This study provides Class IV evidence that dextromethorphan at various doses does not change EEG spike counts over 6 months, though precision was limited to exclude an important effect. © 2017 American Academy of Neurology.

  2. Role of biomarkers in differentiating new-onset seizures from psychogenic nonepileptic seizures

    Directory of Open Access Journals (Sweden)

    Mahendra Javali

    2017-01-01

    Full Text Available Introduction: Review of literature revealed very limited studies considering a combination of serum prolactin (PRL and serum creatine kinase (CK as markers for differentiating epileptic and psychogenic nonepileptic seizures (PNES. Therefore, in the present study, we analyzed the role of serum PRL and serum CK, individually and in combination. Methodology: This prospective study was conducted in a tertiary care medical teaching hospital over a period of 18 months. Patients aged over 15 years suspected to have new-onset seizures presenting within 5 h of ictus were included in this study. CK, serum PRL was measured at 0–1, 1–3, and 3–5 h after seizures. Results: Hundred subjects were studied for the role of serum PRL and serum CK in differentiating epileptic and PNES. The mean age was 42.24 years with a male:female ratio of 1.27:1. All patients of generalized tonic–clonic seizures (GTCS, who presented within 1 h, had elevated PRL, whereas 75% of patients with partial seizures had elevated PRL within 1 h of presentation. Nearly 91.66% of patients with GTCS who presented within 1 h had elevated CPK, whereas 70% of patients with partial seizures had elevated CPK. None of the patients diagnosed with PNES showed rise in either of the markers. Conclusion: In the present study, none of the patients with PNES showed raise in either serum PRL or CK. However, there was no correlation between the types of seizure and PRL or serum CK levels.

  3. Early and late postoperative seizure outcome in 97 patients with supratentorial meningioma and preoperative seizures: a retrospective study.

    Science.gov (United States)

    Zheng, Zhe; Chen, Peng; Fu, Weiming; Zhu, Junming; Zhang, Hong; Shi, Jian; Zhang, Jianmin

    2013-08-01

    We identified factors associated with early and late postoperative seizure control in patients with supratentorial meningioma plus preoperative seizures. In this retrospective study, univariate analysis and multivariate logistic regression analysis compared 24 clinical variables according to the occurrence of early (≤1 week) or late (>1 week) postoperative seizures. Sixty-two of 97 patients (63.9 %) were seizure free for the entire postoperative follow-up period (29.5 ± 11.8 months), while 13 patients (13.4 %) still had frequent seizures at the end of follow-up. Fourteen of 97 patients (14.4 %) experienced early postoperative seizures, and emergence of new postoperative neurological deficits was the only significant risk factor (odds ratio = 7.377). Thirty-three patients (34.0 %) experienced late postoperative seizures at some time during follow-up, including 12 of 14 patients with early postoperative seizures. Associated risk factors for late postoperative seizures included tumor progression (odds ratio = 7.012) and new permanent postoperative neurological deficits (odds ratio = 4.327). Occurrence of postoperative seizures in patients with supratentorial meningioma and preoperative seizure was associated with new postoperative neurological deficits. Reduced cerebral or vascular injury during surgery may lead to fewer postoperative neurological deficits and better seizure outcome.

  4. Seizure-associated aphasia has good lateralizing but poor localizing significance.

    Science.gov (United States)

    Loesch, Anna Mira; Steger, Hannah; Losher, Claudia; Hartl, Elisabeth; Rémi, Jan; Vollmar, Christian; Noachtar, Soheyl

    2017-09-01

    To investigate the occurrence of ictal and postictal aphasia in different focal epilepsy syndromes. We retrospectively analyzed the video-electroencephalographic monitoring data of 1,118 patients with focal epilepsy for seizure-associated aphasia (SAA). Statistical analysis included chi-square analysis and Fisher's exact test. We identified 102 of 1,118 patients (9.1%) in whom ictal or postictal aphasia (SAA) was part of their recorded seizures (n = 59 of 102; 57.8%) or who reported aphasia by history (n = 43; 42.2% only reported aphasia by history). Postictal aphasia was present in 18 patients (30.5%). Six of the 59 patients had both ictal and postictal aphasia (10.2%). SAA occurred either with left hemisphere seizure onset or with seizures spreading from the right to the left hemisphere. SAA was most common in patients with parieto-occipital epilepsy (10.9%; five of 46 patients), followed by patients with temporal (6.7%; 28 of 420 patients), focal (not further localized; 4.8%; 22 of 462 patients), and frontal epilepsy (2.1%; four of 190 patients; p = 0.04). SAA was more common in parieto-occipital epilepsy than in frontal epilepsy (p = 0.02). In contrast, there was no significant difference in SAA between temporal and parieto-occipital epilepsy (p = 0.36). SAA has a high lateralizing but limited localizing value, as it often reflects spread of epileptic activity into speech-harboring brain regions. Wiley Periodicals, Inc. © 2017 International League Against Epilepsy.

  5. Hyponatraemia and seizures after ecstasy use

    Science.gov (United States)

    Holmes, S.; Banerjee, A.; Alexander, W.

    1999-01-01

    A patient presented to our unit with seizures and profound hyponatraemia after ingestion of a single tablet of ecstasy. The seizures proved resistant to therapy and ventilation on the intensive care unit was required. Resolution of the seizures occurred on correction of the metabolic abnormalities. The pathogenesis of seizures and hyponatraemia after ecstasy use is discussed. Ecstasy use should be considered in any young patient presenting with unexplained seizures and attention should be directed towards electrolyte levels, particularly sodium.


Keywords: ecstasy; seizures; hyponatraemia PMID:10396584

  6. The genetic basis of DOORS syndrome : an exome-sequencing study

    NARCIS (Netherlands)

    Campeau, Philippe M.; Kasperaviciute, Dalia; Lu, James T.; Burrage, Lindsay C.; Kim, Choel; Hori, Mutsuki; Powell, Berkley R.; Stewart, Fiona; Felix, Temis Maria; van den Ende, Jenneke; Wisniewska, Marzena; Kayserili, Huelya; Rump, Patrick; Nampoothiri, Sheela; Aftimos, Salim; Mey, Antje; Nair, Lal D. V.; Begleiter, Michael L.; De Bie, Isabelle; Meenakshi, Girish; Murray, Mitzi L.; Repetto, Gabriela M.; Golabi, Mahin; Blair, Edward; Male, Alison; Giuliano, Fabienne; Kariminejad, Ariana; Newman, William G.; Bhaskar, Sanjeev S.; Dickerson, Jonathan E.; Kerr, Bronwyn; Banka, Siddharth; Giltay, Jacques C.; Wieczorek, Dagmar; Tostevin, Anna; Wiszniewska, Joanna; Cheung, Sau Wai; Hennekam, Raoul C.; Gibbs, Richard A.; Lee, Brendan H.; Sisodiya, Sanjay M.

    Background Deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures (DOORS) syndrome is a rare autosomal recessive disorder of unknown cause. We aimed to identify the genetic basis of this syndrome by sequencing most coding exons in affected individuals. Methods Through a search

  7. Plasticity-modulated seizure dynamics for seizure termination in realistic neuronal models

    NARCIS (Netherlands)

    Koppert, M.M.J.; Kalitzin, S.; Lopes da Silva, F.H.; Viergever, M.A.

    2011-01-01

    In previous studies we showed that autonomous absence seizure generation and termination can be explained by realistic neuronal models eliciting bi-stable dynamics. In these models epileptic seizures are triggered either by external stimuli (reflex epilepsies) or by internal fluctuations. This

  8. Envenomation Seizures.

    Science.gov (United States)

    Kharal, Ghulam Abbas; Darby, Richard Ryan; Cohen, Adam B

    2018-01-01

    Insect sting-related envenomation rarely produces seizures. We present a patient with confusion and seizures that began 24 hours after a yellow jacket (wasp) sting. Given the rapid onset and resolution of symptoms, as well as accompanying dermatological and orbital features, and the lack of any infectious or structural abnormalities identified, the toxic effect of the wasp venom (and related anaphylaxis reaction) was believed to be the cause of his presentation.

  9. Genetics Home Reference: Kabuki syndrome

    Science.gov (United States)

    ... also have seizures, an unusually small head size ( microcephaly ), or weak muscle tone (hypotonia). Some have eye ... Syndrome ClinicalTrials.gov (1 link) ClinicalTrials.gov Scientific Articles on PubMed (1 link) PubMed OMIM (2 links) ...

  10. Characteristics of seizure-induced signal changes on MRI in patients with first seizures.

    Science.gov (United States)

    Kim, Si Eun; Lee, Byung In; Shin, Kyong Jin; Ha, Sam Yeol; Park, JinSe; Park, Kang Min; Kim, Hyung Chan; Lee, Joonwon; Bae, Soo-Young; Lee, Dongah; Kim, Sung Eun

    2017-05-01

    The aim of this study was to investigate the predictive factors and identify the characteristics of the seizure-induced signal changes on MRI (SCM) in patients with first seizures. We conducted a retrospective study of patients with first seizures from March 2010 to August 2014. The inclusion criteria for this study were patients with 1) first seizures, and 2) MRI and EEG performed within 24h of the first seizures. The definition of SCM was hyper-intensities in the brain not applying to cerebral arterial territories. Multivariate logistic regression was performed with or without SCM as a dependent variable. Of 431 patients with seizures visiting the ER, 69 patients met the inclusion criteria. Of 69 patients, 11 patients (15.9%) had SCM. Epileptiform discharge on EEG (OR 29.7, 95% CI 1.79-493.37, p=0.018) was an independently significant variable predicting the presence of SCM in patients with first seizures. In addition, the topography of SCM was as follows; i) ipsilateral hippocampus, thalamus and cerebral cortex (5/11), ii) unilateral cortex (4/11), iii) ipsilateral thalamus and cerebral cortex (1/11), iv) bilateral hippocampus (1/11). Moreover, 6 out of 7 patients who underwent both perfusion CT and MRI exhibited unilateral cortical hyperperfusion with ipsilateral thalamic involvement reflecting unrestricted vascular territories. There is an association between epileptiform discharges and SCM. Additionally, the involvement of the unilateral cortex and ipsilateral thalamus in SCM and its hyperperfusion state could be helpful in differentiating the consequences of epileptic seizures from other pathologies. Copyright © 2017 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  11. Electroencephalography after a single unprovoked seizure.

    Science.gov (United States)

    Debicki, Derek B

    2017-07-01

    Electroencephalography (EEG) is an essential diagnostic tool in the evaluation of seizure disorders. In particular, EEG is used as an additional investigation for a single unprovoked seizure. Epileptiform abnormalities are related to seizure disorders and have been shown to predict recurrent unprovoked seizures (i.e., a clinical definition of epilepsy). Thus, the identification of epileptiform abnormalities after a single unprovoked seizure can inform treatment options. The current review addresses the relationship between EEG abnormalities and seizure recurrence. This review also addresses factors that are found to improve the yield of recording epileptiform abnormalities including timing of EEG relative to the new-onset seizure, use of repeat studies, use of sleep deprivation and prolonged recordings. Copyright © 2017 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  12. Influence of vigilance state on physiological consequences of seizures and seizure-induced death in mice.

    Science.gov (United States)

    Hajek, Michael A; Buchanan, Gordon F

    2016-05-01

    Sudden unexpected death in epilepsy (SUDEP) is the leading cause of death in patients with refractory epilepsy. SUDEP occurs more commonly during nighttime sleep. The details of why SUDEP occurs at night are not well understood. Understanding why SUDEP occurs at night during sleep might help to better understand why SUDEP occurs at all and hasten development of preventive strategies. Here we aimed to understand circumstances causing seizures that occur during sleep to result in death. Groups of 12 adult male mice were instrumented for EEG, EMG, and EKG recording and subjected to seizure induction via maximal electroshock (MES) during wakefulness, nonrapid eye movement (NREM) sleep, and rapid eye movement (REM) sleep. Seizure inductions were performed with concomitant EEG, EMG, and EKG recording and breathing assessment via whole body plethysmography. Seizures induced via MES during sleep were associated with more profound respiratory suppression and were more likely to result in death. Despite REM sleep being a time when seizures do not typically occur spontaneously, when seizures were forced to occur during REM sleep, they were invariably fatal in this model. An examination of baseline breathing revealed that mice that died following a seizure had increased baseline respiratory rate variability compared with those that did not die. These data demonstrate that sleep, especially REM sleep, can be a dangerous time for a seizure to occur. These data also demonstrate that there may be baseline respiratory abnormalities that can predict which individuals have higher risk for seizure-induced death.

  13. Upregulation of Haploinsufficient Gene Expression in the Brain by Targeting a Long Non-coding RNA Improves Seizure Phenotype in a Model of Dravet Syndrome.

    Science.gov (United States)

    Hsiao, J; Yuan, T Y; Tsai, M S; Lu, C Y; Lin, Y C; Lee, M L; Lin, S W; Chang, F C; Liu Pimentel, H; Olive, C; Coito, C; Shen, G; Young, M; Thorne, T; Lawrence, M; Magistri, M; Faghihi, M A; Khorkova, O; Wahlestedt, C

    2016-07-01

    Dravet syndrome is a devastating genetic brain disorder caused by heterozygous loss-of-function mutation in the voltage-gated sodium channel gene SCN1A. There are currently no treatments, but the upregulation of SCN1A healthy allele represents an appealing therapeutic strategy. In this study we identified a novel, evolutionary conserved mechanism controlling the expression of SCN1A that is mediated by an antisense non-coding RNA (SCN1ANAT). Using oligonucleotide-based compounds (AntagoNATs) targeting SCN1ANAT we were able to induce specific upregulation of SCN1A both in vitro and in vivo, in the brain of Dravet knock-in mouse model and a non-human primate. AntagoNAT-mediated upregulation of Scn1a in postnatal Dravet mice led to significant improvements in seizure phenotype and excitability of hippocampal interneurons. These results further elucidate the pathophysiology of Dravet syndrome and outline a possible new approach for the treatment of this and other genetic disorders with similar etiology. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

  14. Upregulation of Haploinsufficient Gene Expression in the Brain by Targeting a Long Non-coding RNA Improves Seizure Phenotype in a Model of Dravet Syndrome

    Directory of Open Access Journals (Sweden)

    J. Hsiao

    2016-07-01

    Full Text Available Dravet syndrome is a devastating genetic brain disorder caused by heterozygous loss-of-function mutation in the voltage-gated sodium channel gene SCN1A. There are currently no treatments, but the upregulation of SCN1A healthy allele represents an appealing therapeutic strategy. In this study we identified a novel, evolutionary conserved mechanism controlling the expression of SCN1A that is mediated by an antisense non-coding RNA (SCN1ANAT. Using oligonucleotide-based compounds (AntagoNATs targeting SCN1ANAT we were able to induce specific upregulation of SCN1A both in vitro and in vivo, in the brain of Dravet knock-in mouse model and a non-human primate. AntagoNAT-mediated upregulation of Scn1a in postnatal Dravet mice led to significant improvements in seizure phenotype and excitability of hippocampal interneurons. These results further elucidate the pathophysiology of Dravet syndrome and outline a possible new approach for the treatment of this and other genetic disorders with similar etiology.

  15. Predicting epileptic seizures in advance.

    Directory of Open Access Journals (Sweden)

    Negin Moghim

    Full Text Available Epilepsy is the second most common neurological disorder, affecting 0.6-0.8% of the world's population. In this neurological disorder, abnormal activity of the brain causes seizures, the nature of which tend to be sudden. Antiepileptic Drugs (AEDs are used as long-term therapeutic solutions that control the condition. Of those treated with AEDs, 35% become resistant to medication. The unpredictable nature of seizures poses risks for the individual with epilepsy. It is clearly desirable to find more effective ways of preventing seizures for such patients. The automatic detection of oncoming seizures, before their actual onset, can facilitate timely intervention and hence minimize these risks. In addition, advance prediction of seizures can enrich our understanding of the epileptic brain. In this study, drawing on the body of work behind automatic seizure detection and prediction from digitised Invasive Electroencephalography (EEG data, a prediction algorithm, ASPPR (Advance Seizure Prediction via Pre-ictal Relabeling, is described. ASPPR facilitates the learning of predictive models targeted at recognizing patterns in EEG activity that are in a specific time window in advance of a seizure. It then exploits advanced machine learning coupled with the design and selection of appropriate features from EEG signals. Results, from evaluating ASPPR independently on 21 different patients, suggest that seizures for many patients can be predicted up to 20 minutes in advance of their onset. Compared to benchmark performance represented by a mean S1-Score (harmonic mean of Sensitivity and Specificity of 90.6% for predicting seizure onset between 0 and 5 minutes in advance, ASPPR achieves mean S1-Scores of: 96.30% for prediction between 1 and 6 minutes in advance, 96.13% for prediction between 8 and 13 minutes in advance, 94.5% for prediction between 14 and 19 minutes in advance, and 94.2% for prediction between 20 and 25 minutes in advance.

  16. The genetic basis of DOORS syndrome: an exome-sequencing study

    NARCIS (Netherlands)

    Campeau, Philippe M.; Kasperaviciute, Dalia; Lu, James T.; Burrage, Lindsay C.; Kim, Choel; Hori, Mutsuki; Powell, Berkley R.; Stewart, Fiona; Félix, Têmis Maria; van den Ende, Jenneke; Wisniewska, Marzena; Kayserili, Hülya; Rump, Patrick; Nampoothiri, Sheela; Aftimos, Salim; Mey, Antje; Nair, Lal D. V.; Begleiter, Michael L.; de Bie, Isabelle; Meenakshi, Girish; Murray, Mitzi L.; Repetto, Gabriela M.; Golabi, Mahin; Blair, Edward; Male, Alison; Giuliano, Fabienne; Kariminejad, Ariana; Newman, William G.; Bhaskar, Sanjeev S.; Dickerson, Jonathan E.; Kerr, Bronwyn; Banka, Siddharth; Giltay, Jacques C.; Wieczorek, Dagmar; Tostevin, Anna; Wiszniewska, Joanna; Cheung, Sau Wai; Hennekam, Raoul C.; Gibbs, Richard A.; Lee, Brendan H.; Sisodiya, Sanjay M.

    2014-01-01

    Deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures (DOORS) syndrome is a rare autosomal recessive disorder of unknown cause. We aimed to identify the genetic basis of this syndrome by sequencing most coding exons in affected individuals. Through a search of available case

  17. Seizures and Teens: Surgery for Seizures--What's It All About?

    Science.gov (United States)

    Duchowny, Michael S.; Dean, Patricia

    2006-01-01

    Nearly 1 out of 2 children and teens with seizures may need to take medications throughout their lives. At least 25% will develop a condition called refractory epilepsy--meaning that their seizures do not respond to medical therapy. For these children and teens, non-drug therapies such as brain surgery are available that may offer a chance to…

  18. Seizure clusters: characteristics and treatment.

    Science.gov (United States)

    Haut, Sheryl R

    2015-04-01

    Many patients with epilepsy experience 'clusters' or flurries of seizures, also termed acute repetitive seizures (ARS). Seizure clustering has a significant impact on health and quality of life. This review summarizes recent advances in the definition and neurophysiologic understanding of clustering, the epidemiology and risk factors for clustering and both inpatient and outpatient clinical implications. New treatments for seizure clustering/ARS are perhaps the area of greatest recent progress. Efforts have focused on creating a uniform definition of a seizure cluster. In neurophysiologic studies of refractory epilepsy, seizures within a cluster appear to be self-triggering. Clinical progress has been achieved towards a more precise prevalence of clustering, and consensus guidelines for epilepsy monitoring unit safety. The greatest recent advances are in the study of nonintravenous route of benzodiazepines as rescue medications for seizure clusters/ARS. Rectal benzodiazepines have been very effective but barriers to use exist. New data on buccal, intramuscular and intranasal preparations are anticipated to lead to a greater number of approved treatments. Progesterone may be effective for women who experience catamenial clusters. Seizure clustering is common, particularly in the setting of medically refractory epilepsy. Clustering worsens health and quality of life, and the field requires greater focus on clarifying of definition and clinical implications. Progress towards the development of nonintravenous routes of benzodiazepines has the potential to improve care in this area.

  19. Risk Factors for Preoperative Seizures and Loss of Seizure Control in Patients Undergoing Surgery for Metastatic Brain Tumors.

    Science.gov (United States)

    Wu, Adela; Weingart, Jon D; Gallia, Gary L; Lim, Michael; Brem, Henry; Bettegowda, Chetan; Chaichana, Kaisorn L

    2017-08-01

    Metastatic brain tumors are the most common brain tumors in adults. Patients with metastatic brain tumors have poor prognoses with median survival of 6-12 months. Seizures are a major presenting symptom and cause of morbidity and mortality. In this article, risk factors for the onset of preoperative seizures and postoperative seizure control are examined. Adult patients who underwent resection of one or more brain metastases at a single institution between 1998 and 2011 were reviewed retrospectively. Of 565 patients, 114 (20.2%) patients presented with seizures. Factors independently associated with preoperative seizures were preoperative headaches (P = 0.044), cognitive deficits (P = 0.031), more than 2 intracranial metastatic tumors (P = 0.013), temporal lobe location (P = 0.031), occipital lobe location (P = 0.010), and bone involvement by tumor (P = 0.029). Factors independently associated with loss of seizure control after surgical resection were preoperative seizures (P = 0.001), temporal lobe location (P = 0.037), lack of postoperative chemotherapy (P = 0.010), subtotal resection of tumor (P = 0.022), and local recurrence (P = 0.027). At last follow-up, the majority of patients (93.8%) were seizure-free. Thirty patients (5.30%) in total had loss of seizure control, and only 8 patients (1.41%) who did not have preoperative seizures presented with new-onset seizures after surgical resection of their metastases. The brain is a common site for metastases from numerous primary cancers, such as breast and lung. The identification of factors associated with onset of preoperative seizures as well as seizure control postoperatively could aid management strategies for patients with metastatic brain tumors. Patients with preoperative seizures who underwent resection tended to have good seizure control after surgery. Copyright © 2017 Elsevier Inc. All rights reserved.

  20. Sanjad-Sakati Syndrome and Its Association with Superior Mesenteric Artery Syndrome

    Directory of Open Access Journals (Sweden)

    Osamah Abdullah AlAyed

    2014-01-01

    Full Text Available Sanjad-Sakati syndrome (SSS is an autosomal recessive disorder found exclusively in people of Arabian origin. It was first reported in the Kingdom of Saudi Arabia in 1988 and confirmed by a definitive report in 1991. The syndrome comprises of congenital hypoparathyroidism, seizures, severe growth and developmental retardation, low IQ, and atypical facial features. Supportive treatment in the form of vitamin D and growth hormone supplementation is often offered to patients suffering from SSS. This case study focuses on the steps taken to help a patient who was found to have very unusual symptoms and was later found to have superior mesenteric artery syndrome.

  1. Neonatal Seizure Models to Study Epileptogenesis

    Directory of Open Access Journals (Sweden)

    Yuka Kasahara

    2018-04-01

    Full Text Available Current therapeutic strategies for epilepsy include anti-epileptic drugs and surgical treatments that are mainly focused on the suppression of existing seizures rather than the occurrence of the first spontaneous seizure. These symptomatic treatments help a certain proportion of patients, but these strategies are not intended to clarify the cellular and molecular mechanisms underlying the primary process of epilepsy development, i.e., epileptogenesis. Epileptogenic changes include reorganization of neural and glial circuits, resulting in the formation of an epileptogenic focus. To achieve the goal of developing “anti-epileptogenic” drugs, we need to clarify the step-by-step mechanisms underlying epileptogenesis for patients whose seizures are not controllable with existing “anti-epileptic” drugs. Epileptogenesis has been studied using animal models of neonatal seizures because such models are useful for studying the latent period before the occurrence of spontaneous seizures and the lowering of the seizure threshold. Further, neonatal seizure models are generally easy to handle and can be applied for in vitro studies because cells in the neonatal brain are suitable for culture. Here, we review two animal models of neonatal seizures for studying epileptogenesis and discuss their features, specifically focusing on hypoxia-ischemia (HI-induced seizures and febrile seizures (FSs. Studying these models will contribute to identifying the potential therapeutic targets and biomarkers of epileptogenesis.

  2. IB-12LUNG MASS AND IMMUNOSUPPRESSANT RESPONSIVE SEIZURES: VGKC AUTOIMMUNITY MASQUERADING AS ETIOLOGY OR ACTING AS REACTIVE MARKER?

    Science.gov (United States)

    Umemura, Yoshie; Bujarski, Krzysztof; Ronan, Lara

    2014-01-01

    Voltage gated potassium channel complex antibody (VGKC Ab) has been associated with many neurological illnesses including seizures. VGKC Ab related seizures are less responsive to antiepileptic drugs alone, and often require immunosuppression to achieve seizure freedom. Recently, specific antigenic targets such as LGI1 and CASPR2 within VGKC have been found and have been associated with specific syndromes. It has also been noted that the term "VGKC Ab" itself is problematic because it groups LGI1 and CASPR2 mediated disorders with other illnesses with unknown antigenic specificity. We describe a case of faciobrachial dystonia seizures refractory to multiple antiepileptic agents. Video EEG monitoring revealed mesial frontal epileptic discharges and serum test was positive for VGKC Ab. Patient's seizures were controlled after intravenous corticosteroid then oral mycophenolate maintenance, however, seizures returned with immunotherapy taper and cessation. When the seizures recurred, serum VGKC Ab, CSF CASPR 2 and LGI1 Abs were negative. Interestingly, she was subsequently found to have a lung mass consistent with sarcoidosis. Seizure control was achieved again with restarting of mycophenolate. Most VGKC Ab related illnesses are autoimmune even though it is a part of readily available commercial paraneoplastic testing panels. There has been no previously reported VGKC Ab related seizures associated with sarcoidosis. Although sarcoidosis is also an autoimmune entity, it is of T-cell mediated condition. It has been proposed that VGKC Ab may be a part of normal autoantibodies found in otherwise healthy individuals, which can rise to detectable levels under some circumstances. This patient suffered recurrent seizures responsive to immunotherapy even though VGKC, LGI1, and CASPR2 Abs were negative, suggesting another underlying autoimmune pathogenesis and VGKC Ab may have been a mere reactive marker of such underlying process. This raises the question of clinical

  3. Pretreatment seizure semiology in childhood absence epilepsy.

    Science.gov (United States)

    Kessler, Sudha Kilaru; Shinnar, Shlomo; Cnaan, Avital; Dlugos, Dennis; Conry, Joan; Hirtz, Deborah G; Hu, Fengming; Liu, Chunyan; Mizrahi, Eli M; Moshé, Solomon L; Clark, Peggy; Glauser, Tracy A

    2017-08-15

    To determine seizure semiology in children with newly diagnosed childhood absence epilepsy and to evaluate associations with short-term treatment outcomes. For participants enrolled in a multicenter, randomized, double-blind, comparative-effectiveness trial, semiologic features of pretreatment seizures were analyzed as predictors of treatment outcome at the week 16 to 20 visit. Video of 1,932 electrographic absence seizures from 416 participants was evaluated. Median seizure duration was 10.2 seconds; median time between electrographic seizure onset and clinical manifestation onset was 1.5 seconds. For individual seizures and by participant, the most common semiology features were pause/stare (seizure 95.5%, participant 99.3%), motor automatisms (60.6%, 86.1%), and eye involvement (54.9%, 76.5%). The interrater agreement for motor automatisms and eye involvement was good (72%-84%). Variability of semiology features between seizures even within participants was high. Clustering analyses revealed 4 patterns (involving the presence/absence of eye involvement and motor automatisms superimposed on the nearly ubiquitous pause/stare). Most participants experienced more than one seizure cluster pattern. No individual semiologic feature was individually predictive of short-term outcome. Seizure freedom was half as likely in participants with one or more seizure having the pattern of eye involvement without motor automatisms than in participants without this pattern. Almost all absence seizures are characterized by a pause in activity or staring, but rarely is this the only feature. Semiologic features tend to cluster, resulting in identifiable absence seizure subtypes with significant intraparticipant seizure phenomenologic heterogeneity. One seizure subtype, pause/stare and eye involvement but no motor automatisms, is specifically associated with a worse treatment outcome. © 2017 American Academy of Neurology.

  4. 19 CFR 162.22 - Seizure of conveyances.

    Science.gov (United States)

    2010-04-01

    ... 19 Customs Duties 2 2010-04-01 2010-04-01 false Seizure of conveyances. 162.22 Section 162.22... TREASURY (CONTINUED) INSPECTION, SEARCH, AND SEIZURE Seizures § 162.22 Seizure of conveyances. (a) General applicability. If it shall appear to any officer authorized to board conveyances and make seizures that there...

  5. A STUDY OF THE VARIED AETIOLOGY AND CLINICAL PRESENTATION OF NEW ONSET SEIZURES IN POSTPARTUM PERIOD

    Directory of Open Access Journals (Sweden)

    Ravindra Kumar Sudarsi

    2016-08-01

    Full Text Available BACKGROUND Seizures in the postpartum period are the major cause of mortality and morbidity. A variety of neurological disorders causing seizures may be encountered during pregnancy and puerperium. These disorders may be unrelated to pregnancy (e.g. Meningitis or peculiar to pregnancy (e.g. Eclampsia or. Pregnancy may affect the course of the pre-existing neurological disorders such as epilepsy. So, keeping in mind the varied aetiology of postpartum seizures and its response to appropriate treatment, our study has been conducted to find out the most common causes in our setup to guide the optimal therapy. AIM OF THE STUDY To study the varied aetiology and clinical presentation of new onset seizures in the postpartum period over a period of two years. MATERIALS AND METHODS 50 patients who developed first episode of seizures in postpartum period (that is after delivery to 6 weeks admitted to Osmania General Hospital on random selection. All women complaining of new onset seizure with in postpartum period up to 6 weeks. All those women who are known epileptics and who had seizures in the antenatal period were excluded from the study. The selected patients were studied in detail with history and physical examination. RESULTS Different causes for first episode of seizures in postpartum period are enlisted here. Cavernous sinus thrombosis in 17 patients, late postpartum eclampsia accounted for 13 patients, intracranial haemorrhage in 6 patients, ischaemic stroke in 5 patients, posterior reversible encephalopathy syndrome in 4 patients, CNS infections in 6 patients, glioma in 1 patient, viral hepatitis in one patient, idiopathic in 3 patients. CONCLUSIONS Cavernous sinus thrombosis and late postpartum eclampsia constitute the major aetiology of first onset of seizes occurring in the postpartum period.

  6. Neuropathological mechanisms of seizures in autism spectrum disorder

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    Richard Eugene Frye

    2016-05-01

    Full Text Available This manuscript reviews biological abnormalities shared by autism spectrum disorder (ASD and epilepsy. Two neuropathological findings are shared by ASD and epilepsy: abnormalities in minicolumn architecture and -aminobutyric acid (GABA. The peripheral neuropil, which is the region that contains the inhibition circuits of the minicolumns, has been found to be decreased in the post-mortem ASD brain. ASD and epilepsy are associated with inhibitory GABA neurotransmission abnormalities including reduced GABAA and GABAB subunit expression. These abnormalities can elevate the excitation-to-inhibition balance, resulting in hyperexcitablity of the cortex and, in turn, increases the risk of seizures. Medical abnormalities associated with both epilepsy and ASD are discussed. These include specific genetic syndromes, specific metabolic disorders including disorders of energy metabolism and GABA and glutamate neurotransmission, mineral and vitamin deficiencies, heavy metal exposures and immune dysfunction. Many of these medical abnormalities can result in an elevation of the excitatory-inhibitory balance. Fragile X is linked to dysfunction of the mGluR5 receptor and Fragile X, Angelman and Rett syndromes are linked to a reduction in GABAA receptor expression. Defects in energy metabolism can reduce GABA interneuron function. Both pyridoxine dependent seizures and succinic semialdehyde dehydrogenase deficiency cause GABA deficiencies while urea cycle defects and phenylketonuria cause abnormalities in glutamate neurotransmission. Mineral deficiencies can cause glutamate and GABA neurotransmission abnormalities and heavy metals can cause mitochondrial dysfunction which disrupts GABA metabolism. Thus, both ASD and epilepsy are associated with similar abnormalities that may alter the excitatory-to-inhibitory balance of the cortex. These parallels may explain the high prevalence of epilepsy in ASD and the elevated prevalence of ASD features in individuals with

  7. Using trend templates in a neonatal seizure algorithm improves detection of short seizures in a foetal ovine model.

    Science.gov (United States)

    Zwanenburg, Alex; Andriessen, Peter; Jellema, Reint K; Niemarkt, Hendrik J; Wolfs, Tim G A M; Kramer, Boris W; Delhaas, Tammo

    2015-03-01

    Seizures below one minute in duration are difficult to assess correctly using seizure detection algorithms. We aimed to improve neonatal detection algorithm performance for short seizures through the use of trend templates for seizure onset and end. Bipolar EEG were recorded within a transiently asphyxiated ovine model at 0.7 gestational age, a common experimental model for studying brain development in humans of 30-34 weeks of gestation. Transient asphyxia led to electrographic seizures within 6-8 h. A total of 3159 seizures, 2386 shorter than one minute, were annotated in 1976 h-long EEG recordings from 17 foetal lambs. To capture EEG characteristics, five features, sensitive to seizures, were calculated and used to derive trend information. Feature values and trend information were used as input for support vector machine classification and subsequently post-processed. Performance metrics, calculated after post-processing, were compared between analyses with and without employing trend information. Detector performance was assessed after five-fold cross-validation conducted ten times with random splits. The use of trend templates for seizure onset and end in a neonatal seizure detection algorithm significantly improves the correct detection of short seizures using two-channel EEG recordings from 54.3% (52.6-56.1) to 59.5% (58.5-59.9) at FDR 2.0 (median (range); p seizures by EEG monitoring at the NICU.

  8. Modulation of autonomic activity in neurological conditions: Epilepsy and Tourette syndrome

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    Yoko eNagai

    2015-09-01

    Full Text Available This manuscript considers the central but neglected role of the autonomic nervous system in the expression and control of seizures in Epilepsy and tics in Tourette Syndrome (TS. In epilepsy, consideration of autonomic involvement is typically confined to differential diagnoses (e.g. syncope, or in relation to Sudden Unexpected Death in Epilepsy (SUDEP. Investigation is more limited in Tourette Syndrome. The role of the autonomic nervous system in the generation and prevention of epileptic seizures is largely overlooked. Emotional stimuli such as anxiety and stress are potent causes of seizures and tic activity in TS, respectively. This manuscript will describe a possible neural mechanism by which afferent autonomic projections linked to cognition and behaviour influence central nervous system thalamo-cortical regulation, which appears to be an important means for controlling both seizure and tic activity. It also summarizes the link between the integrity of the default mode network and autonomic regulation in patients with epilepsy as well as the link between impaired motor control and autonomic regulation in patients with TS. Two neurological conditions; epilepsy and TS were chosen, as seizures and tics represent parameters that can be easily measured to investigate influences of autonomic functions. The EDA biofeedback approach is anticipated

  9. Seizures and Sleep in the Thalamus: Focal Limbic Seizures Show Divergent Activity Patterns in Different Thalamic Nuclei.

    Science.gov (United States)

    Feng, Li; Motelow, Joshua E; Ma, Chanthia; Biche, William; McCafferty, Cian; Smith, Nicholas; Liu, Mengran; Zhan, Qiong; Jia, Ruonan; Xiao, Bo; Duque, Alvaro; Blumenfeld, Hal

    2017-11-22

    The thalamus plays diverse roles in cortical-subcortical brain activity patterns. Recent work suggests that focal temporal lobe seizures depress subcortical arousal systems and convert cortical activity into a pattern resembling slow-wave sleep. The potential simultaneous and paradoxical role of the thalamus in both limbic seizure propagation, and in sleep-like cortical rhythms has not been investigated. We recorded neuronal activity from the central lateral (CL), anterior (ANT), and ventral posteromedial (VPM) nuclei of the thalamus in an established female rat model of focal limbic seizures. We found that population firing of neurons in CL decreased during seizures while the cortex exhibited slow waves. In contrast, ANT showed a trend toward increased neuronal firing compatible with polyspike seizure discharges seen in the hippocampus. Meanwhile, VPM exhibited a remarkable increase in sleep spindles during focal seizures. Single-unit juxtacellular recordings from CL demonstrated reduced overall firing rates, but a switch in firing pattern from single spikes to burst firing during seizures. These findings suggest that different thalamic nuclei play very different roles in focal limbic seizures. While limbic nuclei, such as ANT, appear to participate directly in seizure propagation, arousal nuclei, such as CL, may contribute to depressed cortical function, whereas sleep spindles in relay nuclei, such as VPM, may interrupt thalamocortical information flow. These combined effects could be critical for controlling both seizure severity and impairment of consciousness. Further understanding of differential effects of seizures on different thalamocortical networks may lead to improved treatments directly targeting these modes of impaired function. SIGNIFICANCE STATEMENT Temporal lobe epilepsy has a major negative impact on quality of life. Previous work suggests that the thalamus plays a critical role in thalamocortical network modulation and subcortical arousal

  10. Seizure Outcomes in Occipital Lobe and Posterior Quadrant Epilepsy Surgery: A Systematic Review and Meta-Analysis.

    Science.gov (United States)

    Harward, Stephen C; Chen, William C; Rolston, John D; Haglund, Michael M; Englot, Dario J

    2018-03-01

    Occipital lobe epilepsy (OLE) is an uncommon but debilitating focal epilepsy syndrome with seizures often refractory to medical management. While surgical resection has proven a viable treatment, previous studies examining postoperative seizure freedom rates are limited by small sample size and patient heterogeneity, thus exhibiting significant variability in their results. To review the medical literature on OLE so as to investigate rates and predictors of both seizure freedom and visual outcomes following surgery. We reviewed manuscripts exploring surgical resection for drug-resistant OLE published between January 1990 and June 2015 on PubMed. Seizure freedom rates were analyzed and potential predictors were evaluated with separate meta-analyses. Postoperative visual outcomes were also examined. We identified 27 case series comprising 584 patients with greater than 1 yr of follow-up. Postoperative seizure freedom (Engel class I outcome) was observed in 65% of patients, and was significantly predicted by age less than 18 yr (odds ratio [OR] 1.54, 95% confidence interval [CI] 1.13-2.18), focal lesion on pathological analysis (OR 2.08, 95% CI 1.58-2.89), and abnormal preoperative magnetic resonance imaging (OR 3.24, 95% 2.03-6.55). Of these patients, 175 also had visual outcomes reported with 57% demonstrating some degree of visual decline following surgery. We did not find any relationship between postoperative visual and seizure outcomes. Surgical resection for OLE is associated with favorable outcomes with nearly two-thirds of patients achieving postoperative seizure freedom. However, patients must be counseled regarding the risk of visual decline following surgery. Copyright © 2017 by the Congress of Neurological Surgeons

  11. Seizure prognosis of patients with low-grade tumors.

    Science.gov (United States)

    Kahlenberg, Cynthia A; Fadul, Camilo E; Roberts, David W; Thadani, Vijay M; Bujarski, Krzysztof A; Scott, Rod C; Jobst, Barbara C

    2012-09-01

    Seizures frequently impact the quality of life of patients with low grade tumors. Management is often based on best clinical judgment. We examined factors that correlate with seizure outcome to optimize seizure management. Patients with supratentorial low-grade tumors evaluated at a single institution were retrospectively reviewed. Using multiple regression analysis the patient characteristics and treatments were correlated with seizure outcome using Engel's classification. Of the 73 patients with low grade tumors and median follow up of 3.8 years (range 1-20 years), 54 (74%) patients had a seizure ever and 46 (63%) had at least one seizure before tumor surgery. The only factor significantly associated with pre-surgical seizures was tumor histology. Of the 54 patients with seizures ever, 25 (46.3%) had a class I outcome at last follow up. There was no difference in seizure outcome between grade II gliomas (astrocytoma grade II, oligodendroglioma grade II, mixed oligo-astrocytoma grade II) and other pathologies (pilocytic astrocytoma, ependymomas, DNET, gangliocytoma and ganglioglioma). Once seizures were established seizure prognosis was similar between different pathologies. Chemotherapy (p=0.03) and radiation therapy (p=0.02) had a positive effect on seizure outcome. No other parameter including significant tumor growth during the follow up period predicted seizure outcome. Only three patients developed new-onset seizures after tumor surgery that were non-perioperative. Anticonvulsant medication was tapered in 14 patients with seizures and 10 had no further seizures. Five patients underwent additional epilepsy surgery with a class I outcome in four. Two patients received a vagal nerve stimulator with >50% seizure reduction. Seizures at presentation are the most important factor associated with continued seizures after tumor surgery. Pathology does not influence seizure outcome. Use of long term prophylactic anticonvulsants is unwarranted. Chemotherapy and

  12. 15 CFR 904.501 - Notice of seizure.

    Science.gov (United States)

    2010-01-01

    ... 15 Commerce and Foreign Trade 3 2010-01-01 2010-01-01 false Notice of seizure. 904.501 Section 904... Seizure and Forfeiture Procedures § 904.501 Notice of seizure. Within 60 days from the date of the seizure, NOAA will serve the Notice of Seizure as provided in § 904.3 to the owner or consignee, if known or...

  13. Aborting Seizures by Painful Stimulation

    Directory of Open Access Journals (Sweden)

    R. L. Carasso

    1992-01-01

    Full Text Available It has been well established that serious consequences may result from allowing seizures to continue. The opportunities for early interruption of seizures by medication is often restricted to medical personnel, leaving non-trained bystanders unable to intervene. We were able to interrupt seizures (including status epilepticus by application of painful dorsiflexion. The mode of action that enables pain to elevate the seizure threshold remains to be elucidated, although the phenomenon is consistent with earlier laboratory studies in experimental epilepsy. The technique may be recommended as an effective and easily learned procedure that may have wide applicability.

  14. Post-partum posterior reversible encephalopathy syndrome

    OpenAIRE

    B. V. Triveni; Salman Mohammed Sheikh; Deepak Shedde

    2014-01-01

    Posterior Reversible Encephalopathy Syndrome (PRES) is a clinicopathological syndrome associated with various clinical conditions presenting with headache, encephalopathy, seizure and cortical visual disturbances. Radiological findings in PRES are thought to be due to vasogenic edema predominantly in posterior cerebral hemispheres and are reversible with appropriate management. We present a case of post partum PRES,A 29 year old primigravida of 33 weeks 3 days period of gestation who prese...

  15. Pediatric intracerebral hemorrhage: acute symptomatic seizures and epilepsy.

    Science.gov (United States)

    Beslow, Lauren A; Abend, Nicholas S; Gindville, Melissa C; Bastian, Rachel A; Licht, Daniel J; Smith, Sabrina E; Hillis, Argye E; Ichord, Rebecca N; Jordan, Lori C

    2013-04-01

    Seizures are believed to be common presenting symptoms in neonates and children with spontaneous intracerebral hemorrhage (ICH). However, few data are available on the epidemiology of acute symptomatic seizures or the risk for later epilepsy. To define the incidence of and explore risk factors for seizures and epilepsy in children with spontaneous ICH. Our a priori hypotheses were that younger age at presentation, cortical involvement of ICH, acute symptomatic seizures after presentation, ICH due to vascular malformation, and elevated intracranial pressure requiring urgent intervention would predict remote symptomatic seizures and epilepsy. Prospective cohort study conducted between March 1, 2007, and January 1, 2012. Three tertiary care pediatric hospitals. Seventy-three pediatric subjects with spontaneous ICH including 20 perinatal (≥37 weeks' gestation to 28 days) and 53 childhood subjects (>28 days to Acute symptomatic seizures (clinically evident and electrographic-only seizures within 7 days), remote symptomatic seizures, and epilepsy. Acute symptomatic seizures occurred in 35 subjects (48%). Acute symptomatic seizures as a presenting symptom of ICH occurred in 12 perinatal (60%) and 19 childhood (36%) subjects (P = .07). Acute symptomatic seizures after presentation occurred in 7 children. Electrographic-only seizures were present in 9 of 32 subjects (28%) with continuous electroencephalogram monitoring. One-year and 2-year remote symptomatic seizure-free survival rates were 82% (95% CI, 68-90) and 67% (95% CI, 46-82), respectively. One-year and 2-year epilepsy-free survival rates were 96% (95% CI, 83-99) and 87% (95% CI, 65-95), respectively. Elevated intracranial pressure requiring acute intervention was a risk factor for seizures after presentation (P = .01; Fisher exact test), remote symptomatic seizures, and epilepsy (P = .03, and P = .04, respectively; log-rank test). Presenting seizures are common in perinatal and childhood ICH. Continuous

  16. Children with new onset seizures: A prospective study of parent variables, child behavior problems, and seizure occurrence.

    Science.gov (United States)

    Austin, Joan K; Haber, Linda C; Dunn, David W; Shore, Cheryl P; Johnson, Cynthia S; Perkins, Susan M

    2015-12-01

    Parent variables (stigma, mood, unmet needs for information and support, and worry) are associated with behavioral difficulties in children with seizures; however, it is not known how this relationship is influenced by additional seizures. This study followed children (ages 4-14 years) and their parents over a 24-month period (with data collected at baseline and 6, 12, and 24 months) and investigated the effect of an additional seizure on the relationship between parenting variables and child behavior difficulties. The sample was parents of 196 children (104 girls and 92 boys) with a first seizure within the past 6 weeks. Child mean age at baseline was 8 years, 3 months (SD 3 years). Data were analyzed using t-tests, chi-square tests, and repeated measures analyses of covariance. Relationships between parent variables, additional seizures, and child behavior problems were consistent across time. Several associations between parent variables and child behavior problems were stronger in the additional seizure group than in the no additional seizure group. Findings suggest that interventions that assist families to respond constructively to the reactions of others regarding their child's seizure condition and to address their needs for information and support could help families of children with continuing seizures to have an improved quality of life. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. Sleep disturbances in voltage-gated potassium channel antibody syndrome.

    Science.gov (United States)

    Barone, Daniel A; Krieger, Ana C

    2016-05-01

    Voltage-gated potassium channels (VGKCs) are a family of membrane proteins responsible for controlling cell membrane potential. The presence of antibodies (Ab) against neuronal VGKC complexes aids in the diagnosis of idiopathic and paraneoplastic autoimmune neurologic disorders. The diagnosis of VGKC Ab-associated encephalopathy (VCKC Ab syndrome) should be suspected in patients with subacute onset of disorientation, confusion, and memory loss in the presence of seizures or a movement disorder. VGKC Ab syndrome may present with sleep-related symptoms, and the purpose of this communication is to alert sleep and neurology clinicians of this still-under-recognized condition. In this case, we are presenting the VGKC Ab syndrome which improved after treatment with solumedrol. The prompt recognition and treatment of this condition may prevent the morbidity associated with cerebral atrophy and the mortality associated with intractable seizures and electrolyte disturbances. Copyright © 2016. Published by Elsevier B.V.

  18. Nodding syndrome in Tanzania may not be associated with ...

    African Journals Online (AJOL)

    2014-06-02

    Jun 2, 2014 ... Department of Psychiatry, University of British Columbia, Vancouver, Canada. 5. ... primary generalized seizures and community controls were not significantly different. However ..... Nodding Syndrome meeting, researchers.

  19. Channel selection for automatic seizure detection

    DEFF Research Database (Denmark)

    Duun-Henriksen, Jonas; Kjaer, Troels Wesenberg; Madsen, Rasmus Elsborg

    2012-01-01

    Objective: To investigate the performance of epileptic seizure detection using only a few of the recorded EEG channels and the ability of software to select these channels compared with a neurophysiologist. Methods: Fifty-nine seizures and 1419 h of interictal EEG are used for training and testing...... of an automatic channel selection method. The characteristics of the seizures are extracted by the use of a wavelet analysis and classified by a support vector machine. The best channel selection method is based upon maximum variance during the seizure. Results: Using only three channels, a seizure detection...... sensitivity of 96% and a false detection rate of 0.14/h were obtained. This corresponds to the performance obtained when channels are selected through visual inspection by a clinical neurophysiologist, and constitutes a 4% improvement in sensitivity compared to seizure detection using channels recorded...

  20. Neuroimaging observations in a cohort of elderly manifesting with new onset seizures: Experience from a university hospital

    Directory of Open Access Journals (Sweden)

    Sanjib Sinha

    2012-01-01

    Full Text Available Background: The occurrence of epilepsy is higher among elderly patients. The clinical manifestations of seizures, causes of epilepsy, and choice of anti-epileptic drugs (AEDs are different in elderly people with epilepsy compared to the young. Aim: To evaluate the imaging (CT/MRI observations in elderly patients manifesting with new-onset seizures. Materials and Methods: Two hundred and one elderly patients with new onset seizures, >60 years (age: 68.0 ± 7.5 years; M:F = 1.8:1 from Jan′ 07 to Jan′ 09, were prospectively recruited. Observations of cranial CT scan (n = 201 and MR imaging (n = 43 were analyzed. Results: The type of seizures included: Simple partial (42%, generalized tonic-clonic (30.3%, and complex partial (27.4%. The pattern of epilepsy syndromes were acute symptomatic (42.3%, remote symptomatic (18.4%, cryptogenic (37.8%, and idiopathic (1.5%. Seizures were controlled with monotherapy in 85%. The CT scan (n = 201 revealed cerebral atrophy (139, mild (79, moderate (43, and severe (18; focal lesions (98, infarcts (45, hemorrhages (18, granuloma (16, tumor (15 and gliosis (4, and hemispheric atrophy (1, white matter changes (75 and diffuse edema (21. An MRI (n = 43 showed variable degree of cerebral atrophy (31; white matter changes (20; focal cerebral lesions (24; - infarct (7; intracranial hemorrhage (6; granuloma (5; tumor (6; gliosis (1; hemispheric atrophy (1; and prominent Virchow-Robin spaces (7; and UBOs (12. Patients with focal lesions in neuroimaging more often had partial seizures, symptomatic epilepsy, past stroke, focal deficit, absence of diffuse atrophy, focal EEG slowing, abnormal CSF, seizure recurrence at follow-up (P < 0.05. Conclusions: Brain imaging observations in elderly patients with new-onset seizures revealed underlying symptomatic nature, hence the etiology and thereby assisted in deciding the specific therapy.

  1. Genetics Home Reference: ring chromosome 20 syndrome

    Science.gov (United States)

    ... drugs. Prolonged seizure episodes known as non-convulsive status epilepticus also appear to be characteristic of ring chromosome ... K, Takahashi Y. Ring chromosome 20 and nonconvulsive status epilepticus. A new epileptic syndrome. Brain. 1997 Jun;120 ( ...

  2. Aminophylline increases seizure length during electroconvulsive therapy.

    Science.gov (United States)

    Stern, L; Dannon, P N; Hirschmann, S; Schriber, S; Amytal, D; Dolberg, O T; Grunhaus, L

    1999-12-01

    Electroconvulsive therapy (ECT) is considered to be one of the most effective treatments for patients with major depression and persistent psychosis. Seizure characteristics probably determine the therapeutic effect of ECT; as a consequence, short seizures are accepted as one of the factors of poor outcome. During most ECT courses seizure threshold increases and seizure duration decreases. Methylxanthine preparations, caffeine, and theophylline have been used to prolong seizure duration. The use of aminophylline, more readily available than caffeine, has not been well documented. The objective of this study was to test the effects of aminophylline on seizure length. Fourteen drug-free patients with diagnoses of affective disorder or psychotic episode receiving ECT participated in this study. Seizure length was assessed clinically and per EEG. Statistical comparisons were done using paired t tests. A significant increase (p < 0.04) in seizure length was achieved and maintained on three subsequent treatments with aminophylline. No adverse events were noted from the addition of aminophylline.

  3. Automatic Seizure Detection in Rats Using Laplacian EEG and Verification with Human Seizure Signals

    Science.gov (United States)

    Feltane, Amal; Boudreaux-Bartels, G. Faye; Besio, Walter

    2012-01-01

    Automated detection of seizures is still a challenging problem. This study presents an approach to detect seizure segments in Laplacian electroencephalography (tEEG) recorded from rats using the tripolar concentric ring electrode (TCRE) configuration. Three features, namely, median absolute deviation, approximate entropy, and maximum singular value were calculated and used as inputs into two different classifiers: support vector machines and adaptive boosting. The relative performance of the extracted features on TCRE tEEG was examined. Results are obtained with an overall accuracy between 84.81 and 96.51%. In addition to using TCRE tEEG data, the seizure detection algorithm was also applied to the recorded EEG signals from Andrzejak et al. database to show the efficiency of the proposed method for seizure detection. PMID:23073989

  4. 27 CFR 478.152 - Seizure and forfeiture.

    Science.gov (United States)

    2010-04-01

    ... 27 Alcohol, Tobacco Products and Firearms 3 2010-04-01 2010-04-01 false Seizure and forfeiture... Exemptions, Seizures, and Forfeitures § 478.152 Seizure and forfeiture. (a) Any firearm or ammunition... demonstrated by clear and convincing evidence, shall be subject to seizure and forfeiture, and all provisions...

  5. Seizure semiology identifies patients with bilateral temporal lobe epilepsy.

    Science.gov (United States)

    Loesch, Anna Mira; Feddersen, Berend; Tezer, F Irsel; Hartl, Elisabeth; Rémi, Jan; Vollmar, Christian; Noachtar, Soheyl

    2015-01-01

    Laterality in temporal lobe epilepsy is usually defined by EEG and imaging results. We investigated whether the analysis of seizure semiology including lateralizing seizure phenomena identifies bilateral independent temporal lobe seizure onset. We investigated the seizure semiology in 17 patients in whom invasive EEG-video-monitoring documented bilateral temporal seizure onset. The results were compared to 20 left and 20 right consecutive temporal lobe epilepsy (TLE) patients who were seizure free after anterior temporal lobe resection. The seizure semiology was analyzed using the semiological seizure classification with particular emphasis on the sequence of seizure phenomena over time and lateralizing seizure phenomena. Statistical analysis included chi-square test or Fisher's exact test. Bitemporal lobe epilepsy patients had more frequently different seizure semiology (100% vs. 40%; p<0.001) and significantly more often lateralizing seizure phenomena pointing to bilateral seizure onset compared to patients with unilateral TLE (67% vs. 11%; p<0.001). The sensitivity of identical vs. different seizure semiology for the identification of bilateral TLE was high (100%) with a specificity of 60%. Lateralizing seizure phenomena had a low sensitivity (59%) but a high specificity (89%). The combination of lateralizing seizure phenomena and different seizure semiology showed a high specificity (94%) but a low sensitivity (59%). The analysis of seizure semiology including lateralizing seizure phenomena adds important clinical information to identify patients with bilateral TLE. Copyright © 2014 Elsevier B.V. All rights reserved.

  6. Epilepsy in patients with Angelman syndrome

    Directory of Open Access Journals (Sweden)

    Fiumara Agata

    2010-04-01

    Full Text Available Abstract Angelman syndrome (AS is a neuro-behavioural, genetically determined condition, characterized by ataxic jerky movements, happy sociable disposition and unprovoked bouts of laughter in association with seizures, learning disabilities and language impairment. Most of the cases are hardly diagnosed during infancy as jerky movements, the cardinal sign, appear later in childhood. AS is caused by a variety of genetic mechanisms involving the 15q 11-13 chromosome. About 70% of cases are due to a "de novo" interstitial deletion in the long arm region, arising on the maternally inherited chromosome. The diagnosis is confirmed by methylation test or by mutation analysis of UBE3A gene. The deletion phenotype is generally linked to a more severe clinical picture in that 95% of patients manifest more severe seizures, severe mental and motor retardation, dysmorphic features and microcephaly. The pathogenesis of epilepsy in AS is still not fully understood. The presence in the commonly deleted region of a cluster of genes coding for 3 subunits of the GABAa receptor complex has lead to the hypothesis that GABA neurotransmission is involved. Epilepsy, often severe and hard to control, is present in 85% of patients within the first three years of life, although less than 25% develop seizures during the first year. It was observed that febrile seizures often precede the diagnosis. Most frequent types are atypical absences, generalized tonic-clonic, atonic or myoclonic seizures, with multiple seizure types occurring in 50% of deleted patients. There is still some doubt about the association with West syndrome. The EEG abnormalities are not themselves pathognomonic of AS and both background activity and epileptic discharges vary even in the same patient with time. Nevertheless, the existence of some suggestive patterns should facilitate the early diagnosis allowing the correct genetic counselling for the family. Some drugs seems to act better than others

  7. Posterior reversible encephalopathy syndrome in patient

    African Journals Online (AJOL)

    abp

    2015-05-26

    May 26, 2015 ... reversible Posterior leukoencephalopathy syndrome [1, 2]. The. PRES is a clinical and radiological entity associating varying degrees, headaches, impaired consciousness, seizures and visual disturbances to neurological and radiological abnormalities of the parietal-occipital white matter [3]. PRES has a ...

  8. Optimizing the Diagnosis and Management of Dravet Syndrome: Recommendations From a North American Consensus Panel.

    Science.gov (United States)

    Wirrell, Elaine C; Laux, Linda; Donner, Elizabeth; Jette, Nathalie; Knupp, Kelly; Meskis, Mary Anne; Miller, Ian; Sullivan, Joseph; Welborn, Michelle; Berg, Anne T

    2017-03-01

    To establish standards for early, cost-effective, and accurate diagnosis; optimal therapies for seizures; and recommendations for evaluation and management of comorbidities for children and adults with Dravet syndrome, using a modified Delphi process. An expert panel was convened comprising epileptologists with nationally recognized expertise in Dravet syndrome and parents of children with Dravet syndrome, whose experience and understanding was enhanced by their active roles in Dravet syndrome associations. Panelists were asked to base their responses to questions both on their clinical expertise and results of a literature review that was forwarded to each panelist. Three rounds of online questionnaires were conducted to identify areas of consensus and strength of that consensus, as well as areas of contention. The panel consisted of 13 physicians and five family members. Strong consensus was reached regarding typical clinical presentation of Dravet syndrome, range of electroencephalography and magnetic resonance imaging findings, need for genetic testing, critical information that should be conveyed to families at diagnosis, priorities for seizure control and typical degree of control, seizure triggers and recommendations for avoidance, first- and second-line therapies for seizures, requirement and indications for rescue therapy, specific recommendations for comorbidity screening, and need for family support. Consensus was not as strong regarding later therapies, including vagus nerve stimulation and callosotomy, and for specific therapies of associated comorbidities. Beyond the initial treatment with benzodiazepines and use of valproate, there was no consensus on the optimal in-hospital management of convulsive status epilepticus. We were able to identify areas where there was strong consensus that we hope will (1) inform health care providers on optimal diagnosis and management of patients with Dravet syndrome, (2) support reimbursement from insurance companies

  9. 50 CFR 12.11 - Notification of seizure.

    Science.gov (United States)

    2010-10-01

    ... 50 Wildlife and Fisheries 1 2010-10-01 2010-10-01 false Notification of seizure. 12.11 Section 12... SEIZURE AND FORFEITURE PROCEDURES Preliminary Requirements § 12.11 Notification of seizure. Except where the owner or consignee is personally notified or seizure is made pursuant to a search warrant, the...

  10. EEG analysis of seizure patterns using visibility graphs for detection of generalized seizures

    NARCIS (Netherlands)

    Wang, Lei; Long, Xi; Arends, J.B.A.M.; Aarts, R.M.

    2017-01-01

    Background The traditional EEG features in the time and frequency domain show limited seizure detection performance in the epileptic population with intellectual disability (ID). In addition, the influence of EEG seizure patterns on detection performance was less studied. New method A single-channel

  11. Opiate v CNS depressant therapy in neonatal drug abstinence syndrome.

    Science.gov (United States)

    Kandall, S R; Doberczak, T M; Mauer, K R; Strashun, R H; Korts, D C

    1983-04-01

    Paregoric and phenobarbital, administered randomly in 153 passively addicted neonates, initially appeared to control neonatal abstinence signs equally well. However, seven of the 62 phenobarbital-treated newborns had abstinence-associated seizures within the first month of life, while none of 49 paregoric-treated neonates had seizures. Forty-two neonates initially requiring no specific pharmacotherapy for abstinence signs were born to mothers taking less methadone hydrochloride just before delivery. Five of those 42 neonates, however, had seizures within the first 14 days of life. Seizure occurrence could not be predicted from analysis of early abstinence patterns. We consider paregoric to be the treatment of choice for the neonatal abstinence syndrome. Phenobarbital use should be monitored with serum drug levels and modification of recommended dosage regimens considered.

  12. Early-onset seizures due to mosaic exonic deletions of CDKL5 in a male and two females.

    Science.gov (United States)

    Bartnik, Magdalena; Derwińska, Katarzyna; Gos, Monika; Obersztyn, Ewa; Kołodziejska, Katarzyna E; Erez, Ayelet; Szpecht-Potocka, Agnieszka; Fang, Ping; Terczyńska, Iwona; Mierzewska, Hanna; Lohr, Naomi J; Bellus, Gary A; Reimschisel, Tyler; Bocian, Ewa; Mazurczak, Tadeusz; Cheung, Sau Wai; Stankiewicz, Paweł

    2011-05-01

    Mutations in the CDKL5 gene have been associated with an X-linked dominant early infantile epileptic encephalopathy-2. The clinical presentation is usually of severe encephalopathy with refractory seizures and Rett syndrome (RTT)-like phenotype. We attempted to assess the role of mosaic intragenic copy number variation in CDKL5. We have used comparative genomic hybridization with a custom-designed clinical oligonucleotide array targeting exons of selected disease and candidate genes, including CDKL5. We have identified mosaic exonic deletions of CDKL5 in one male and two females with developmental delay and medically intractable seizures. These three mosaic changes represent 60% of all deletions detected in 12,000 patients analyzed by array comparative genomic hybridization and involving the exonic portion of CDKL5. We report the first case of an exonic deletion of CDKL5 in a male and emphasize the importance of underappreciated mosaic exonic copy number variation in patients with early-onset seizures and RTT-like features of both genders.

  13. Epilepsy in a child with Wolf-Hirschhorn syndrome

    Directory of Open Access Journals (Sweden)

    Radlović Nedeljko

    2011-01-01

    Full Text Available Introduction. Wolf-Hirschhorn syndrome (WHS is a rare chromosomal disorder characterized by facial dismorphy, multiple congenital anomalies, delayed psychomotor development and pharmaco-resistant epilepsy. Case Outline. We present a 5-year-old girl with severe delay in growth and development, microcephaly, mild facial dismorphy and epilepsy. The pregnancy was complicated by intrauterine growth retardation. Generalized muscle hypotonia was observed at birth. First seizures started at age of 9 months as unilateral convulsive status epilepticus (SE, sometimes with bilateral generalization. Seizures were often triggered by fever and were resistant to antiepileptic treatment. Introduction of lamotrigine and valproate therapy led to complete seizure control at the age of 33 months. Electroencephalographic (EEG finding was typical at the beginning. After transitory improvement between age four and five years, epileptiform EEG activity appeared again at the age of five years, without observed clinical seizures. Magnetic resonance imaging showed diffuse brain atrophy and delay in myelination. Using Multiplex ligation-dependent probe amplification (MLPA method, we disclosed heterozygote microdeletation of the distal part of the short arm of chromosome 4 (4p16. Conclusion. We present a clinical course of epilepsy in a patient with Wolf-Hirschhorn syndrome. The diagnosis was verified by modern molecular technique. This is the first molecular characterization of a patient with WHS performed in our country.

  14. Benign focal epilepsy of childhood with centrotemporal spikes (BECTS: clinical characteristics of seizures according to age at first seizure

    Directory of Open Access Journals (Sweden)

    Miziara Carmen Silvia M.G.

    2002-01-01

    Full Text Available BECTS is characterized by the presence of simple partial motor seizures in the face and/or oropharynx, with or without sensory symptoms and often with secondary generalization. These seizures tend to occur more often during sleep or drowsiness. According to some authors, generalized seizures prevail over other types particularly among children aged five or less. The purpose of this study is to determine the characteristics of the first epileptic episode among children with BECTS, grouped by age as of their first epileptic seizure, as well as to analyze how such seizures change over the course of clinical evolution. A total of 61 children were examined, 16 of whom below the age of 5 and 45 above. With regard to the first and recurrent epileptic episodes, our final assessment showed that partial seizures occurred more frequently than generalized tonic-clonic seizures in both groups. Although no conclusive relation could be established between the type of partial seizure (i.e. simple versus complex and the children's age as of their first epileptic episode, it was possible to correlate the type of epileptic seizure with their clinical evolution, in which case simple partial seizures proved to be more frequent than complex partial seizures. It should be noted that the number of children under the age of five was relatively small, which evinces the need for further studies. It should also be borne in mind that the reported frequency of generalized seizures in these children's first epileptic episode may be due to their parents' lack of attention and familiarity with this pathology and their attendant difficulty in characterizing its clinical symptoms.

  15. Seizures in multiple sclerosis

    NARCIS (Netherlands)

    Koch, Marcus; Uyttenboogaart, Maarten; Polman, Susan; De Keyser, Jacques

    Seizures have long been recognized to be part of the disease spectrum of multiple sclerosis (MS). While they occur in only a minority of patients with MS, epileptic seizures can have serious consequences. The treatment of MS can be epileptogenic, and antiepileptic treatment can conversely worsen the

  16. Fahr's Syndrome and Secondary Hypoparathyroidism.

    Science.gov (United States)

    Dos Santos, Vitorino Modesta; Da Mata, Ana Medeiros De Farias; Ribeiro, Kelle Regina Alves; Calvo, Isadora Cartaxo De Sousa

    2016-01-01

    A typical case of Fahr's syndrome is described in a 76-year-old Brazilian female who underwent a total thyroidectomy three decades ago. Six years before the current admission, she started with generalized tonic-clonic seizures. Associated disorders involved extra-pyramidal, cognitive, nocturnal terror and mood changes. With suspicion of hypocalcemia due to secondary hypoparathyroidism, laboratory determinations confirmed the diagnoses. Furthermore, imaging studies of the central nervous system detected multiple calcifications, with characteristic distribution of Fahr's syndrome. Clinical management was successful.

  17. 19 CFR 162.92 - Notice of seizure.

    Science.gov (United States)

    2010-04-01

    ... 19 Customs Duties 2 2010-04-01 2010-04-01 false Notice of seizure. 162.92 Section 162.92 Customs... (CONTINUED) INSPECTION, SEARCH, AND SEIZURE Civil Asset Forfeiture Reform Act § 162.92 Notice of seizure. (a) Generally. Customs will send written notice of seizure as provided in this section to all known interested...

  18. Multi-modal intelligent seizure acquisition (MISA) system--a new approach towards seizure detection based on full body motion measures.

    Science.gov (United States)

    Conradsen, Isa; Beniczky, Sandor; Wolf, Peter; Terney, Daniella; Sams, Thomas; Sorensen, Helge B D

    2009-01-01

    Many epilepsy patients cannot call for help during a seizure, because they are unconscious or because of the affection of their motor system or speech function. This can lead to injuries, medical complications and at worst death. An alarm system setting off at seizure onset could help to avoid hazards. Today no reliable alarm systems are available. A Multi-modal Intelligent Seizure Acquisition (MISA) system based on full body motion data seems as a good approach towards detection of epileptic seizures. The system is the first to provide a full body description for epilepsy applications. Three test subjects were used for this pilot project. Each subject simulated 15 seizures and in addition performed some predefined normal activities, during a 4-hour monitoring with electromyography (EMG), accelerometer, magnetometer and gyroscope (AMG), electrocardiography (ECG), electroencephalography (EEG) and audio and video recording. The results showed that a non-subject specific MISA system developed on data from the modalities: accelerometer (ACM), gyroscope and EMG is able to detect 98% of the simulated seizures and at the same time mistakes only 4 of the normal movements for seizures. If the system is individualized (subject specific) it is able to detect all simulated seizures with a maximum of 1 false positive. Based on the results from the simulated seizures and normal movements the MISA system seems to be a promising approach to seizure detection.

  19. 8 CFR 1280.21 - Seizure of aircraft.

    Science.gov (United States)

    2010-01-01

    ... 8 Aliens and Nationality 1 2010-01-01 2010-01-01 false Seizure of aircraft. 1280.21 Section 1280... REGULATIONS IMPOSITION AND COLLECTION OF FINES § 1280.21 Seizure of aircraft. Seizure of an aircraft under the... that its value is less than the amount of the fine which may be imposed. If seizure of an aircraft for...

  20. 8 CFR 280.21 - Seizure of aircraft.

    Science.gov (United States)

    2010-01-01

    ... 8 Aliens and Nationality 1 2010-01-01 2010-01-01 false Seizure of aircraft. 280.21 Section 280.21... OF FINES § 280.21 Seizure of aircraft. Seizure of an aircraft under the authority of section 239 of... than the amount of the fine which may be imposed. If seizure of an aircraft for violation of section...

  1. Clustering of spontaneous recurrent seizures separated by long seizure-free periods: An extended video-EEG monitoring study of a pilocarpine mouse model.

    Science.gov (United States)

    Lim, Jung-Ah; Moon, Jangsup; Kim, Tae-Joon; Jun, Jin-Sun; Park, Byeongsu; Byun, Jung-Ick; Sunwoo, Jun-Sang; Park, Kyung-Il; Lee, Soon-Tae; Jung, Keun-Hwa; Jung, Ki-Young; Kim, Manho; Jeon, Daejong; Chu, Kon; Lee, Sang Kun

    2018-01-01

    Seizure clustering is a common and significant phenomenon in patients with epilepsy. The clustering of spontaneous recurrent seizures (SRSs) in animal models of epilepsy, including mouse pilocarpine models, has been reported. However, most studies have analyzed seizures for a short duration after the induction of status epilepticus (SE). In this study, we investigated the detailed characteristics of seizure clustering in the chronic stage of a mouse pilocarpine-induced epilepsy model for an extended duration by continuous 24/7 video-EEG monitoring. A seizure cluster was defined as the occurrence of one or more seizures per day for at least three consecutive days and at least five seizures during the cluster period. We analyzed the cluster duration, seizure-free period, cluster interval, and numbers of seizures within and outside the seizure clusters. The video-EEG monitoring began 84.5±33.7 days after the induction of SE and continued for 53.7±20.4 days. Every mouse displayed seizure clusters, and 97.0% of the seizures occurred within a cluster period. The seizure clusters were followed by long seizure-free periods of 16.3±6.8 days, showing a cyclic pattern. The SRSs also occurred in a grouped pattern within a day. We demonstrate that almost all seizures occur in clusters with a cyclic pattern in the chronic stage of a mouse pilocarpine-induced epilepsy model. The seizure-free periods between clusters were long. These findings should be considered when performing in vivo studies using this animal model. Furthermore, this model might be appropriate for studying the unrevealed mechanism of ictogenesis.

  2. Blockade of T-type calcium channels prevents tonic-clonic seizures in a maximal electroshock seizure model.

    Science.gov (United States)

    Sakkaki, Sophie; Gangarossa, Giuseppe; Lerat, Benoit; Françon, Dominique; Forichon, Luc; Chemin, Jean; Valjent, Emmanuel; Lerner-Natoli, Mireille; Lory, Philippe

    2016-02-01

    T-type (Cav3) calcium channels play important roles in neuronal excitability, both in normal and pathological activities of the brain. In particular, they contribute to hyper-excitability disorders such as epilepsy. Here we have characterized the anticonvulsant properties of TTA-A2, a selective T-type channel blocker, in mouse. Using the maximal electroshock seizure (MES) as a model of tonic-clonic generalized seizures, we report that mice treated with TTA-A2 (0.3 mg/kg and higher doses) were significantly protected against tonic seizures. Although no major change in Local Field Potential (LFP) pattern was observed during the MES seizure, analysis of the late post-ictal period revealed a significant increase in the delta frequency power in animals treated with TTA-A2. Similar results were obtained for Cav3.1-/- mice, which were less prone to develop tonic seizures in the MES test, but not for Cav3.2-/- mice. Analysis of extracellular signal-regulated kinase 1/2 (ERK) phosphorylation and c-Fos expression revealed a rapid and elevated neuronal activation in the hippocampus following MES clonic seizures, which was unchanged in TTA-A2 treated animals. Overall, our data indicate that TTA-A2 is a potent anticonvulsant and that the Cav3.1 isoform plays a prominent role in mediating TTA-A2 tonic seizure protection. Copyright © 2015. Published by Elsevier Ltd.

  3. Dissociation in patients with dissociative seizures: relationships with trauma and seizure symptoms.

    Science.gov (United States)

    Pick, S; Mellers, J D C; Goldstein, L H

    2017-05-01

    This study aimed to extend the current understanding of dissociative symptoms experienced by patients with dissociative (psychogenic, non-epileptic) seizures (DS), including psychological and somatoform types of symptomatology. An additional aim was to assess possible relationships between dissociation, traumatic experiences, post-traumatic symptoms and seizure manifestations in this group. A total of 40 patients with DS were compared with a healthy control group (n = 43), matched on relevant demographic characteristics. Participants completed several self-report questionnaires, including the Multiscale Dissociation Inventory (MDI), Somatoform Dissociation Questionnaire-20, Traumatic Experiences Checklist and the Post-Traumatic Diagnostic Scale. Measures of seizure symptoms and current emotional distress (Hospital Anxiety and Depression Scale) were also administered. The clinical group reported significantly more psychological and somatoform dissociative symptoms, trauma, perceived impact of trauma, and post-traumatic symptoms than controls. Some dissociative symptoms (i.e. MDI disengagement, MDI depersonalization, MDI derealization, MDI memory disturbance, and somatoform dissociation scores) were elevated even after controlling for emotional distress; MDI depersonalization scores correlated positively with trauma scores while seizure symptoms correlated with MDI depersonalization, derealization and identity dissociation scores. Exploratory analyses indicated that somatoform dissociation specifically mediated the relationship between reported sexual abuse and DS diagnosis, along with depressive symptoms. A range of psychological and somatoform dissociative symptoms, traumatic experiences and post-traumatic symptoms are elevated in patients with DS relative to healthy controls, and seem related to seizure manifestations. Further studies are needed to explore peri-ictal dissociative experiences in more detail.

  4. Masks of Schwartz-Bartter Syndrome clinical pictures: a case report

    Directory of Open Access Journals (Sweden)

    E A Pigarova

    2008-06-01

    Full Text Available Schwartz-Bartter Syndrome (synonyms: syndrome of inappropriate secretion of antidiuretic hormone syndrome of inappropriate secretion of vasopressin, anti-diabetes insipidus - this is a rare disease characterized by excessive secretion of antidiuretic hormone (ADH from the posterior pituitary, or other source, resulting in developing hyponatremia and water intoxication. Hyponatremia dilutions at Schwartz-Bartter Syndrome is usually non-specific symptoms, such as headache, nausea, vomiting and confusion. Marked reduction in blood sodium levels may lead to seizures or even coma.

  5. A systematic review of suggestive seizure induction for the diagnosis of psychogenic nonepileptic seizures.

    Science.gov (United States)

    Popkirov, Stoyan; Grönheit, Wenke; Wellmer, Jörg

    2015-09-01

    Suggestive seizure induction is a widely used method for diagnosing psychogenic nonepileptic seizures (PNES). Despite seven decades of multidisciplinary research, however, there is still no unified protocol, no definitive agreement on the ethical framework and no consensus on diagnostic utility. This systematic review surveys the evidence at hand and addresses clinically relevant aspects of suggestive seizure induction. In addition to its use for facilitating the diagnostic process, its mechanism of action and utility in elucidating the psychopathology of PNES will be discussed. Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  6. Recessive loss-of-function mutations in AP4S1 cause mild fever-sensitive seizures, developmental delay and spastic paraplegia through loss of AP-4 complex assembly

    Science.gov (United States)

    Hardies, Katia; May, Patrick; Djémié, Tania; Tarta-Arsene, Oana; Deconinck, Tine; Craiu, Dana; Helbig, Ingo; Suls, Arvid; Balling, Rudy; Weckhuysen, Sarah; De Jonghe, Peter; Hirst, Jennifer; Afawi, Zaid; Barisic, Nina; Baulac, Stéphanie; Caglayan, Hande; Depienne, Christel; De Kovel, Carolien G.F.; Dimova, Petia; Guerrero-López, Rosa; Guerrini, Renzo; Hjalgrim, Helle; Hoffman-Zacharska, Dorota; Jahn, Johanna; Klein, Karl Martin; Koeleman, Bobby P.C.; Leguern, Eric; Lehesjoki, Anna-Elina; Lemke, Johannes; Lerche, Holger; Marini, Carla; Muhle, Hiltrud; Rosenow, Felix; Serratosa, Jose M.; Møller, Rikke S.; Stephani, Ulrich; Striano, Pasquale; Talvik, Tiina; Von Spiczak, Sarah; Weber, Yvonne; Zara, Federico

    2015-01-01

    We report two siblings with infantile onset seizures, severe developmental delay and spastic paraplegia, in whom whole-genome sequencing revealed compound heterozygous mutations in the AP4S1 gene, encoding the σ subunit of the adaptor protein complex 4 (AP-4). The effect of the predicted loss-of-function variants (p.Gln46Profs*9 and p.Arg97*) was further investigated in a patient's fibroblast cell line. We show that the premature stop mutations in AP4S1 result in a reduction of all AP-4 subunits and loss of AP-4 complex assembly. Recruitment of the AP-4 accessory protein tepsin, to the membrane was also abolished. In retrospect, the clinical phenotype in the family is consistent with previous reports of the AP-4 deficiency syndrome. Our study reports the second family with mutations in AP4S1 and describes the first two patients with loss of AP4S1 and seizures. We further discuss seizure phenotypes in reported patients, highlighting that seizures are part of the clinical manifestation of the AP-4 deficiency syndrome. We also hypothesize that endosomal trafficking is a common theme between heritable spastic paraplegia and some inherited epilepsies. PMID:25552650

  7. Unsupervised EEG analysis for automated epileptic seizure detection

    Science.gov (United States)

    Birjandtalab, Javad; Pouyan, Maziyar Baran; Nourani, Mehrdad

    2016-07-01

    Epilepsy is a neurological disorder which can, if not controlled, potentially cause unexpected death. It is extremely crucial to have accurate automatic pattern recognition and data mining techniques to detect the onset of seizures and inform care-givers to help the patients. EEG signals are the preferred biosignals for diagnosis of epileptic patients. Most of the existing pattern recognition techniques used in EEG analysis leverage the notion of supervised machine learning algorithms. Since seizure data are heavily under-represented, such techniques are not always practical particularly when the labeled data is not sufficiently available or when disease progression is rapid and the corresponding EEG footprint pattern will not be robust. Furthermore, EEG pattern change is highly individual dependent and requires experienced specialists to annotate the seizure and non-seizure events. In this work, we present an unsupervised technique to discriminate seizures and non-seizures events. We employ power spectral density of EEG signals in different frequency bands that are informative features to accurately cluster seizure and non-seizure events. The experimental results tried so far indicate achieving more than 90% accuracy in clustering seizure and non-seizure events without having any prior knowledge on patient's history.

  8. Predictability of uncontrollable multifocal seizures - towards new treatment options

    Science.gov (United States)

    Lehnertz, Klaus; Dickten, Henning; Porz, Stephan; Helmstaedter, Christoph; Elger, Christian E.

    2016-04-01

    Drug-resistant, multifocal, non-resectable epilepsies are among the most difficult epileptic disorders to manage. An approach to control previously uncontrollable seizures in epilepsy patients would consist of identifying seizure precursors in critical brain areas combined with delivering a counteracting influence to prevent seizure generation. Predictability of seizures with acceptable levels of sensitivity and specificity, even in an ambulatory setting, has been repeatedly shown, however, in patients with a single seizure focus only. We did a study to assess feasibility of state-of-the-art, electroencephalogram-based seizure-prediction techniques in patients with uncontrollable multifocal seizures. We obtained significant predictive information about upcoming seizures in more than two thirds of patients. Unexpectedly, the emergence of seizure precursors was confined to non-affected brain areas. Our findings clearly indicate that epileptic networks, spanning lobes and hemispheres, underlie generation of seizures. Our proof-of-concept study is an important milestone towards new therapeutic strategies based on seizure-prediction techniques for clinical practice.

  9. Neuroimaging abnormalities and seizure recurrence in a prospective cohort study of Zambians with human immunodeficiency virus and first seizure

    Directory of Open Access Journals (Sweden)

    Michael J. Potchen

    2014-10-01

    Full Text Available In HIV-positive individuals with first seizure, we describe neuroimaging findings, detail clinical and demographic risk factors for imaging abnormalities, and evaluate the relationship between imaging abnormalities and seizure recurrence to determine if imaging abnormalities predict recurrent seizures. Among 43 participants (mean 37.4 years, 56% were male, 16 (37% were on antiretroviral drugs, 32 (79% had advanced HIV disease, and (28 66% had multiple seizures and/or status epilepticus at enrollment. Among those with cerebrospinal fluid studies, 14/31 (44% had opportunistic infections (OIs. During follow-up, 9 (21% died and 15 (35% experienced recurrent seizures. Edema was associated with OIs (odds ratio: 8.79; confidence interval: 1.03-236 and subcortical atrophy with poorer scores on the International HIV Dementia Scale (5.2 vs. 9.3; P=0.002. Imaging abnormalities were not associated with seizure recurrence or death (P>0.05. Seizure recurrence occurred in at least a third and over 20% died during follow-up. Imaging was not predictive of recurrent seizure or death, but imaging abnormalities may offer additional diagnostic insights in terms of OI risk and cognitive impairment.

  10. Profile of seizures in adult falciparum malaria and the clinical efficacy of phenytoin sodium for control of seizures

    Directory of Open Access Journals (Sweden)

    Manoj Ku Mohapatra

    2012-10-01

    Full Text Available Objective: To study the profile of convulsion in adult severe falciparum malaria and efficacy of phenytoin sodium for its control. Methods: It comprised of two sub studies. Study-1 evaluated the pattern and risk factors of seizure in severe malaria and Study-2 investigated the efficacy of phenytoin sodium to control seizure in an open label trial. Patients of severe malaria were diagnosed as per WHO guideline. Clinical type and duration of convulsion were determined. Biochemical and haematological investigations including EEG and CT scan of brain were performed in all cases. All patients were treated with injection artesunate along with other supportive measures and patients with convulsions were treated with injection phenytoin sodium. Results: Out of 408 patients of severe malaria 118 (28.9% patients had seizure. Generalized tonic clonic seizure, partial seizure with secondary generalization, and status epilepticus was present in 89(75.4%, 25(21.2%, and 4(3.4% cases respectively. CT scan was abnormal in 16 (13.6% cases. EEG was abnormal in 108 (91.5% cases showing generalized seizure activity. Patients with convulsion (n=118 were treated with phenytoin sodium injection and convulsion was controlled within 12 hours [mean (6.2依2.1 hours] of treatment in 107 (90.6% patients. Recurrence of seizure occurred in 2 (1.7% patients and 11 (9.3% patients did not respond. The mortality and sequelae were more among patients with than without convulsion. Conclusions: Seizure is common in adult falciparum malaria and phenytoin is an effective drug for seizure control.

  11. Febrile Infection-Related Epilepsy Syndrome (FIRES): An Overview of Treatment and Recent Patents.

    Science.gov (United States)

    Hon, Kam Lun E Lun; Leung, Alexander K C; Torres, Alcy R

    2018-05-08

    New-onset refractory status epilepticus (NORSE) refers to a clinical presentation in a patient without active epilepsy or other existing relevant neurological disorder, with new onset of refractory status epilepticus in the absence of a clear acute or active structural, metabolic, or toxic cause. Febrile infection-related epilepsy syndrome (FIRES) is a subset of NORSE that requires a febrile infection between 24 hours and 2 weeks prior to the onset of refractory status epilepticus, with or without fever at the onset of status epilepticus, and with no restriction to the age of the patient. The literature on FIRES is scarce. This article reviews the pathophysiology, clinical features, and various treatment modalities in the treatment of FIRES. A Medline/Pubmed search was conducted using Clinical Queries with the key terms "febrile infection-related epilepsy syndrome", "FIRES", "new-onset refractory status epilepticus" and "NORSE". The search strategy included meta-analyses, randomized controlled trials, clinical trials, reviews and pertinent references. Patents were searched using the key term "FIRES", "NORSE" and "febrile epilepsy syndrome" from www.google.com/patents, www.uspto.gov, and www.freepatentsonline.com. FIRES almost invariably begins with a mild nonspecific febrile illness in an otherwise healthy individual. Twenty four hours to two weeks later, seizures begin and quickly become very frequent and worsen, becoming status epilepticus. Seizures can be simple motor, complex partial or secondary generalized. The exact etiology is no known. It is possible that the syndrome is caused by an inflammatory or autoimmune mechanism. Seizures in FIRES are notoriously very difficult to treat. Treatment modalities include, among others, various antiepileptic drugs, ketogenic diet, intravenous corticosteroids, intravenous immunoglobulin, and burst-suppression coma. Outcome is poor; most children are left with significant cognitive disability and refractory epilepsy

  12. Anti-Seizure Medications: Relief from Nerve Pain

    Science.gov (United States)

    Anti-seizure medications: Relief from nerve pain Anti-seizure drugs often are used to help control the type of ... by damaged nerves. By Mayo Clinic Staff Anti-seizure medications were originally designed to treat people with ...

  13. Types of Seizures Affecting Individuals with TSC

    Science.gov (United States)

    ... Policy Sitemap Learn Engage Donate About TSC Epilepsy/Seizure Disorders Seizures remain one of the most common neurological ... TSC Brain and Neurological Function Brain Abnormalities Epilepsy/Seizure Disorders Infantile Spasms Epilepsy in Adults with TSC Epilepsy ...

  14. Febrile seizures and risk of schizophrenia

    DEFF Research Database (Denmark)

    Vestergaard, Mogens; Pedersen, Carsten Bøcker; Christensen, Jakob

    2005-01-01

    BACKGROUND: Febrile seizure is a benign condition for most children, but experiments in animals and neuroimaging studies in humans suggest that some febrile seizures may damage the hippocampus, a brain area of possible importance in schizophrenia. METHODS: A population-based cohort of all children...... with schizophrenia. A history of febrile seizures was associated with a 44% increased risk of schizophrenia [relative risk (RR)=1.44; 95% confidence interval (CI), 1.07-1.95] after adjusting for confounding factors. The association between febrile seizures and schizophrenia remained virtually unchanged when...... restricting the analyses to people with no history of epilepsy. A history of both febrile seizures and epilepsy was associated with a 204% increased risk of schizophrenia (RR=3.04; 95% CI, 1.36-6.79) as compared with people with no such history. CONCLUSIONS: We found a slightly increased risk of schizophrenia...

  15. A new model to study sleep deprivation-induced seizure.

    Science.gov (United States)

    Lucey, Brendan P; Leahy, Averi; Rosas, Regine; Shaw, Paul J

    2015-05-01

    A relationship between sleep and seizures is well-described in both humans and rodent animal models; however, the mechanism underlying this relationship is unknown. Using Drosophila melanogaster mutants with seizure phenotypes, we demonstrate that seizure activity can be modified by sleep deprivation. Seizure activity was evaluated in an adult bang-sensitive seizure mutant, stress sensitive B (sesB(9ed4)), and in an adult temperature sensitive seizure mutant seizure (sei(ts1)) under baseline and following 12 h of sleep deprivation. The long-term effect of sleep deprivation on young, immature sesB(9ed4) flies was also assessed. Laboratory. Drosophila melanogaster. Sleep deprivation. Sleep deprivation increased seizure susceptibility in adult sesB(9ed4)/+ and sei(ts1) mutant flies. Sleep deprivation also increased seizure susceptibility when sesB was disrupted using RNAi. The effect of sleep deprivation on seizure activity was reduced when sesB(9ed4)/+ flies were given the anti-seizure drug, valproic acid. In contrast to adult flies, sleep deprivation during early fly development resulted in chronic seizure susceptibility when sesB(9ed4)/+ became adults. These findings show that Drosophila is a model organism for investigating the relationship between sleep and seizure activity. © 2015 Associated Professional Sleep Societies, LLC.

  16. Microarray and FISH-based genotype-phenotype analysis of 22 Japanese patients with Wolf-Hirschhorn syndrome.

    Science.gov (United States)

    Shimizu, Kenji; Wakui, Keiko; Kosho, Tomoki; Okamoto, Nobuhiko; Mizuno, Seiji; Itomi, Kazuya; Hattori, Shigeto; Nishio, Kimio; Samura, Osamu; Kobayashi, Yoshiyuki; Kako, Yuko; Arai, Takashi; Tsutomu, Oh-ishi; Kawame, Hiroshi; Narumi, Yoko; Ohashi, Hirofumi; Fukushima, Yoshimitsu

    2014-03-01

    Wolf-Hirschhorn syndrome (WHS) is a contiguous gene deletion syndrome of the distal 4p chromosome, characterized by craniofacial features, growth impairment, intellectual disability, and seizures. Although genotype-phenotype correlation studies have previously been published, several important issues remain to be elucidated including seizure severity. We present detailed clinical and molecular-cytogenetic findings from a microarray and fluorescence in situ hybridization (FISH)-based genotype-phenotype analysis of 22 Japanese WHS patients, the first large non-Western series. 4p deletions were terminal in 20 patients and interstitial in two, with deletion sizes ranging from 2.06 to 29.42 Mb. The new Wolf-Hirschhorn syndrome critical region (WHSCR2) was deleted in all cases, and duplication of other chromosomal regions occurred in four. Complex mosaicism was identified in two cases: two different 4p terminal deletions; a simple 4p terminal deletion and an unbalanced translocation with the same 4p breakpoint. Seizures began in infancy in 33% (2/6) of cases with small (6 Mb). Status epilepticus occurred in 17% (1/6) with small deletions and in 87% (13/15) with larger deletions. Renal hypoplasia or dysplasia and structural ocular anomalies were more prevalent in those with larger deletions. A new susceptible region for seizure occurrence is suggested between 0.76 and 1.3 Mb from 4 pter, encompassing CTBP1 and CPLX1, and distal to the previously-supposed candidate gene LETM1. The usefulness of bromide therapy for seizures and additional clinical features including hypercholesterolemia are also described. © 2013 Wiley Periodicals, Inc.

  17. Incidence and outcome of epilepsy syndromes with onset in the first year of life: A retrospective population-based study.

    Science.gov (United States)

    Gaily, Eija; Lommi, Markus; Lapatto, Risto; Lehesjoki, Anna-Elina

    2016-10-01

    Population-based studies on infantile epilepsy syndromes are scarce. Our aim was to provide syndrome-specific data on the incidence and outcome of epilepsy in a population-based cohort of infants with epilepsy onset in the first year. Included were all infants born in 1997 through 2006 whose epileptic seizures started before 12 months of age and who were residents of the Helsinki University Hospital district at the time of seizure onset. Patients were ascertained from hospital statistics, and all patient charts were reviewed. A reevaluation of the epilepsy syndromes, age at onset, etiology, and outcome at 24 months of age was based on data abstracted from the patient files. Inclusion criteria were fulfilled by 158 infants, of whom 92% were followed until age 24 months or death. The incidence of epilepsy in the first year was 124 of 100,000. An epilepsy syndrome recognized by the revised organization of epilepsies by ILAE was identified in 58% of the patients. The most common syndromes were West syndrome (41/100,000) and benign familial or nonfamilial infantile epilepsy (22/100,000). Etiology was structural-metabolic in 35%, genetic in 17%, and unknown in 48%. Early age at onset was associated with structural-metabolic etiology. Seven infants (4.4%) died before age 2 years. One infant with an SCN2A mutation died of sudden unexplained death in epilepsy (SUDEP). At 24 months, 58% of all children included in the cohort were seizure-free, and 46% had both seizure freedom and age-appropriate cognitive development. Age at onset was not associated with outcome when etiology was controlled for. Benign familial and nonfamilial infantile epilepsy appears to be more common than previously suggested, second only to West syndrome. Early age at onset is not an independent risk factor for poor outcome. Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.

  18. Change in illness perception is associated with short-term seizure burden outcome following video-EEG confirmation of psychogenic nonepileptic seizures.

    Science.gov (United States)

    Chen, David K; Majmudar, Shirine; Ram, Aarthi; Rutherford, Holly C; Fadipe, Melissa; Dunn, Callie B; Collins, Robert L

    2018-04-27

    We aimed to evaluate whether potential changes in the patient's illness perception can significantly influence short-term seizure burden following video-electroencephalography (EEG) confirmation/explanation of psychogenic nonepileptic seizures (PNES). Patients with PNES were dichotomized to two groups based on a five-point Symptom Attribution Scale: (a) those who prior to diagnosis perceived their seizures to be solely ("5") or mainly ("4") physical in origin (physical group) and (b) the remainder of patients with PNES (psychological group). The physical group (n=32), psychological group (n=40), and group with epilepsy (n=26) also completed the Brief Illness Perception Questionnaire (BIPQ) prior to diagnosis, and were followed up at 3months as well as at 6months postdiagnosis. At 3months postdiagnosis, the physical group experienced significantly greater improvement in seizure intensity (p=0.002) and seizure frequency (p=0.016) when compared with the psychological group. The physical group was significantly more likely to have modified their symptom attribution toward a greater psychological role to their seizures (p=0.002), and their endorsement on the BIPQ item addressing "consequences" (How much do your seizures affect your life?) was significantly less severe (p'=0.014) when compared with that of the psychological group and the group with epilepsy. At 6months postdiagnosis, the physical group continued to experience significantly greater improvement in seizure intensity (p=0.007) while their seizure frequency no longer reached significant difference (p=0.078) when compared with the psychological group. The physical group continued to be significantly more likely to have modified their symptom attribution toward a greater psychological role to their seizures (p=0.005), and their endorsement on the BIPQ item addressing "consequences" remained significantly less severe (p'=0.037) when compared with the psychological group and the group with epilepsy. Among

  19. Ketogenic diet: Predictors of seizure control.

    Science.gov (United States)

    Agarwal, Nitin; Arkilo, Dimitrios; Farooq, Osman; Gillogly, Cynthia; Kavak, Katelyn S; Weinstock, Arie

    2017-01-01

    The ketogenic diet is an effective non-pharmacologic treatment for medically resistant epilepsy. The aim of this study was to identify any predictors that may influence the response of ketogenic diet. A retrospective chart review for all patients with medically resistant epilepsy was performed at a tertiary care epilepsy center from 1996 to 2012. Patient- and diet-related variables were evaluated with respect to seizure reduction at 1, 3, 6, 9 and 12-month intervals and divided into four possible outcome classes. Sixty-three patients met inclusion. Thirty-seven (59%) reported >50% seizure reduction at 3 months with 44% and 37% patients benefiting at 6-month and 12-month follow up, respectively. A trend toward significant seizure improvement was noted in 48% patients with seizure onset >1 year at 12-month (p = 0.09) interval and in 62% patients with >10 seizure/day at 6-month interval (p = 0.054). An ordinal logistic regression showed later age of seizure to have higher odds of favorable response at 1-month (p = 0.005) and 3-month (p = 0.013) follow up. Patients with non-fasting diet induction were more likely to have a favorable outcome at 6 months (p = 0.008) as do females (p = 0.037) and those treated with higher fat ratio diet (p = 0.034). Our study reports the effectiveness of ketogenic diet in children with medically resistant epilepsy. Later age of seizure onset, female gender, higher ketogenic diet ratio and non-fasting induction were associated with better odds of improved seizure outcome. A larger cohort is required to confirm these findings.

  20. Modulation of autonomic activity in neurological conditions: Epilepsy and Tourette Syndrome.

    Science.gov (United States)

    Nagai, Yoko

    2015-01-01

    This manuscript considers the central but neglected role of the autonomic nervous system in the expression and control of seizures in epilepsy (small) and tics in Tourette Syndrome (TS). In epilepsy, consideration of autonomic involvement is typically confined to differential diagnoses (e.g., syncope), or in relation to Sudden Unexpected Death in Epilepsy (SUDEP). Investigation is more limited in Tourette Syndrome. The role of the autonomic nervous system in the generation and prevention of epileptic seizures is largely overlooked. Emotional stimuli such as anxiety and stress are potent causes of seizures and tic activity in epilepsy and TS, respectively. This manuscript will describe a possible neural mechanism by which afferent autonomic projections linked to cognition and behavior influence central thalamo-cortical regulation, which appears to be an important means for controlling both seizure and tic activity. It also summarizes the link between the integrity of the default mode network and autonomic regulation in patients with epilepsy as well as the link between impaired motor control and autonomic regulation in patients with TS. Two neurological conditions; epilepsy and TS were chosen, as seizures and tics represent parameters that can be easily measured to investigate influences of autonomic functions. The EDA biofeedback approach is anticipated to gain a strong position within the next generation of treatment for epilepsy, as a non-invasive technique with minimal side effects. This approach also takes advantage of the current practical opportunity to utilize growing digital health technology.

  1. A case of Wolf-Hirschhorn syndrome and hypoplastic left heart syndrome.

    Science.gov (United States)

    von Elten, Kelley; Sawyer, Taylor; Lentz-Kapua, Sarah; Kanis, Adam; Studer, Matthew

    2013-06-01

    Wolf-Hirschhorn Syndrome (WHS) is a genetic syndrome that includes a typical facial appearance, mental retardation, growth delay, seizures, and congenital cardiac defects. A deletion of the terminal band of the short arm of chromosome 4, with a breakpoint at the 4p15 to 4p16 region, is the most common genetic mutation causing WHS. Congenital heart disease associated with WHS typically includes atrial and ventricular septal defects, though there are a few case reports of associated complex congenital heart disease. Here we report a case of an infant with a large 4p deletion, with a breakpoint at the 4p12 region, and hypoplasic left heart syndrome. We discuss a possible link between the size of the chromosomal deletion in WHS and the severity of the cardiac defect.

  2. Psychogenic non-epileptic seizures: our video-EEG experience.

    Science.gov (United States)

    Nežádal, Tomáš; Hovorka, Jiří; Herman, Erik; Němcová, Iveta; Bajaček, Michal; Stichová, Eva

    2011-09-01

    The aim of our study was to assess the number of psychogenic non-epileptic seizures (PNES) in our patients with a refractory seizure disorder, to determine the 'typical' PNES semiology using video-EEG monitoring and describe other PNES parameters. We evaluated prospectively 596 patients with pharmacoresistant seizures. All these patients underwent continuous video-EEG monitoring. In consenting patients, we used suggestive seizure provocation. We assessed seizure semiology, interictal EEG, brain MRI, psychiatric co-morbidities, personality profiles, and seizure outcome. In the sample of 596 monitored patients, we detected 111 (19.3%) patients with PNES. Of the 111 patients with PNES, 86.5% had spontaneous and 76.5% had provoked seizures. The five most typical symptoms were: initially closed eyelids (67.6%), rapid tremor (47.7%), asynchronous limb movement (37.8%), preictal pseudosleep (33.3%), and side-to-side head movement (32.4%). Interictal EEG was rated as abnormal in 46.2% and with epileptiform abnormality in 9%. Brain MRI was abnormal in 32 (28.8%) patients. Personality disorders (46.8%), anxiety (39.6%), and depression (12.6%) were the most frequent additional psychiatric co-morbidities. PNES outcome after at least 2 years is reported; 22.5% patients was seizure-free; one-third had markedly reduced seizure frequency. We have not seen any negative impact of the provocative testing on the seizure outcome. Video-EEG monitoring with suggestive seizure provocation supported by clinical psychiatric and psychological evaluation significantly contributes to the correct PNES diagnosis, while interictal EEG and brain MRI are frequently abnormal. Symptoms typical for PNES, as opposed to epileptic seizures, could be distinguished.

  3. Automatic multi-modal intelligent seizure acquisition (MISA) system for detection of motor seizures from electromyographic data and motion data

    DEFF Research Database (Denmark)

    Conradsen, Isa; Beniczky, Sándor; Wolf, Peter

    2012-01-01

    measures of reconstructed sub-bands from the discrete wavelet transformation (DWT) and the wavelet packet transformation (WPT). Based on the extracted features all data segments were classified using a support vector machine (SVM) algorithm as simulated seizure or normal activity. A case study...... of the seizure from the patient showed that the simulated seizures were visually similar to the epileptic one. The multi-modal intelligent seizure acquisition (MISA) system showed high sensitivity, short detection latency and low false detection rate. The results showed superiority of the multi- modal detection...... system compared to the uni-modal one. The presented system has a promising potential for seizure detection based on multi-modal data....

  4. The alcohol withdrawal syndrome.

    LENUS (Irish Health Repository)

    McKeon, A

    2008-08-01

    The alcohol withdrawal syndrome (AWS) is a common management problem in hospital practice for neurologists, psychiatrists and general physicians alike. Although some patients have mild symptoms and may even be managed in the outpatient setting, others have more severe symptoms or a history of adverse outcomes that requires close inpatient supervision and benzodiazepine therapy. Many patients with AWS have multiple management issues (withdrawal symptoms, delirium tremens, the Wernicke-Korsakoff syndrome, seizures, depression, polysubstance abuse, electrolyte disturbances and liver disease), which requires a coordinated, multidisciplinary approach. Although AWS may be complex, careful evaluation and available treatments should ensure safe detoxification for most patients.

  5. Tranexamic acid–associated seizures: Causes and treatment

    Science.gov (United States)

    Lecker, Irene; Wang, Dian‐Shi; Whissell, Paul D.; Avramescu, Sinziana; Mazer, C. David

    2015-01-01

    Antifibrinolytic drugs are routinely used worldwide to reduce the bleeding that results from a wide range of hemorrhagic conditions. The most commonly used antifibrinolytic drug, tranexamic acid, is associated with an increased incidence of postoperative seizures. The reported increase in the frequency of seizures is alarming, as these events are associated with adverse neurological outcomes, longer hospital stays, and increased in‐hospital mortality. However, many clinicians are unaware that tranexamic acid causes seizures. The goal of this review is to summarize the incidence, risk factors, and clinical features of these seizures. This review also highlights several clinical and preclinical studies that offer mechanistic insights into the potential causes of and treatments for tranexamic acid–associated seizures. This review will aid the medical community by increasing awareness about tranexamic acid–associated seizures and by translating scientific findings into therapeutic interventions for patients. ANN NEUROL 2016;79:18–26 PMID:26580862

  6. Acute and chronic pain syndromes in multiple sclerosis

    DEFF Research Database (Denmark)

    Stenager, E; Knudsen, L; Jensen, K

    1991-01-01

    A representative sample of 117 patients with definite multiple sclerosis (MS) was interviewed on pain syndromes. Chronic syndromes lasting more than one month included dysaestesthesia, low back pain, spasms, tonic seizures, tightening and painful sensations in the extremities. Acute syndromes...... with pain at the time of the examination increased with age and duration of disease. Patients with pain were significantly more often spastic and significantly more often sought alternative treatment forms. No difference was found for mean age, sex, physical impairment, duration of disease from onset of MS...

  7. Minimum Electric Field Exposure for Seizure Induction with Electroconvulsive Therapy and Magnetic Seizure Therapy.

    Science.gov (United States)

    Lee, Won H; Lisanby, Sarah H; Laine, Andrew F; Peterchev, Angel V

    2017-05-01

    Lowering and individualizing the current amplitude in electroconvulsive therapy (ECT) has been proposed as a means to produce stimulation closer to the neural activation threshold and more focal seizure induction, which could potentially reduce cognitive side effects. However, the effect of current amplitude on the electric field (E-field) in the brain has not been previously linked to the current amplitude threshold for seizure induction. We coupled MRI-based E-field models with amplitude titrations of motor threshold (MT) and seizure threshold (ST) in four nonhuman primates (NHPs) to determine the strength, distribution, and focality of stimulation in the brain for four ECT electrode configurations (bilateral, bifrontal, right-unilateral, and frontomedial) and magnetic seizure therapy (MST) with cap coil on vertex. At the amplitude-titrated ST, the stimulated brain subvolume (23-63%) was significantly less than for conventional ECT with high, fixed current (94-99%). The focality of amplitude-titrated right-unilateral ECT (25%) was comparable to cap coil MST (23%), demonstrating that ECT with a low current amplitude and focal electrode placement can induce seizures with E-field as focal as MST, although these electrode and coil configurations affect differently specific brain regions. Individualizing the current amplitude reduced interindividual variation in the stimulation focality by 40-53% for ECT and 26% for MST, supporting amplitude individualization as a means of dosing especially for ECT. There was an overall significant correlation between the measured amplitude-titrated ST and the prediction of the E-field models, supporting a potential role of these models in dosing of ECT and MST. These findings may guide the development of seizure therapy dosing paradigms with improved risk/benefit ratio.

  8. Predictors of acute symptomatic seizures after intracranial hemorrhage in infants.

    Science.gov (United States)

    Bansal, Seema; Kebede, Tewodros; Dean, Nathan P; Carpenter, Jessica L

    2014-10-01

    To determine the prevalence of acute symptomatic seizures in infants with supratentorial intracranial hemorrhage, to identify potential risk factors, and to determine the effect of acute seizures on long-term morbidity and mortality. Children less than 24 months with intracranial hemorrhage were identified from a neurocritical care database. All patients who received seizure prophylaxis beginning at admission were included in the study. Risk factors studied were gender, etiology, location of hemorrhage, seizure(s) on presentation, and the presence of parenchymal injury. Acute clinical and electrographic seizures were identified from hospital medical records. Subsequent development of late seizures was determined based on clinical information from patients' latest follow-up. Patients with idiopathic neonatal intracranial hemorrhage, premature infants, and those with prior history of seizures were excluded from analysis. Seventy-two infants met inclusion criteria. None. Forty percent of infants had acute symptomatic seizures. The prevalence was similar regardless of whether etiology of hemorrhage was traumatic or nontraumatic. Seizures on presentation and parenchymal injury were independent risk factors of acute seizures (p = 0.001 and p = 0.006, respectively). Younger children and women were also at higher risk (p Acute seizures were not predictive of mortality, but nearly twice as many patients with acute seizures developed late seizures when compared with those without. Electrographic seizures and parenchymal injury were also predictive of development of late seizures (p hemorrhage are at high risk for acute symptomatic seizures. This is regardless of the etiology of hemorrhage. Younger patients, women, patients with parenchymal injury, and patients presenting with seizure are most likely to develop acute seizures. Although the benefits of seizure prophylaxis have not been studied in this specific population, these results suggest that it is an important component

  9. Epilepsy classification and additional definitions in occipital lobe epilepsy.

    Science.gov (United States)

    Yilmaz, Kutluhan; Karatoprak, Elif Yüksel

    2015-09-01

    To evaluate epileptic children with occipital lobe epilepsy (OLE) in the light of the characteristics of Panayiotopoulos syndrome and late-onset occipital lobe epilepsy of Gastaut (OLE-G). Patients were categorized into six groups: primary OLE with autonomic symptoms (Panayiotopoulos syndrome), primary OLE with visual symptoms (OLE-G), secondary OLE with autonomic symptoms (P-type sOLE), secondary OLE with visual symptoms (G-type sOLE), and non-categorized primary OLE and non-categorized secondary OLE according to characteristic ictal symptoms of both Panayiotopoulos syndrome and OLE-G, as well as aetiology (primary or secondary). Patients were compared with regards to seizure symptoms, aetiology, cranial imaging, EEG, treatment and outcome. Of 108 patients with OLE (6.4±3.9 years of age), 60 patients constituted primary groups (32 with Panayiotopoulos syndrome, 11 with OLE-G, and 17 with non-categorized primary OLE); the other 48 patients constituted secondary groups (eight with P-type sOLE, three with G-type sOLE, and 37 with non-categorized sOLE). Epileptiform activity was restricted to the occipital area in half of the patients. Generalized epileptiform activity was observed in three patients, including a patient with Panayiotopoulos syndrome (PS). Only one patient had refractory epilepsy in the primary groups while such patients made up 29% in the secondary groups. In OLE, typical autonomic or visual ictal symptoms of Panayiotopoulos syndrome and OLE-G do not necessarily indicate primary (i.e. genetic or idiopathic) aetiology. Moreover, primary OLE may not present with these symptoms. Since there are many patients with OLE who do not exhibit the characteristics of Panayiotopoulos syndrome or OLE-G, additional definitions and terminology appear to be necessary to differentiate between such patients in both clinical practice and studies.

  10. Characteristics of people with self-reported stress-precipitated seizures.

    Science.gov (United States)

    Privitera, Michael; Walters, Michael; Lee, Ikjae; Polak, Emily; Fleck, Adrienne; Schwieterman, Donna; Haut, Sheryl R

    2014-12-01

    Stress is the most common patient-reported seizure precipitant. We aimed to determine mood and epilepsy characteristics of people who report stress-precipitated seizures. Sequential patients at a tertiary epilepsy center were surveyed about stress as a seizure precipitant. We asked whether acute (lasting minutes-hours) or chronic (lasting days-months) stress was a seizure precipitant, whether stress reduction had been tried, and what effect stress reduction had on seizure frequency. We collected information on antiepileptic drugs, history of depression and anxiety disorder, prior or current treatment for depression or anxiety, and scores on the Neurological Disorders Depression Inventory (NDDI-E) and Generalized Anxiety Disorders-7 (GAD-7) instruments, which are administered at every visit in our Epilepsy Center. We also asked whether respondents thought that they could predict their seizures to determine if stress as a seizure precipitant was correlated with seizure self-prediction. Two hundred sixty-six subjects were included: 219 endorsed stress as a seizure precipitant [STRESS (+)] and 47 did not [STRESS (-)]. Among STRESS (+) subjects, 85% endorsed chronic stress as a seizure precipitant, and 68% endorsed acute stress as a seizure precipitant. In STRESS (+) subjects, 57% had used some type of relaxation or stress reduction method (most commonly yoga, exercise and meditation), and, of those who tried, 88% thought that these methods improved seizures. Among STRESS (-) subjects, 25% had tried relaxation or stress reduction, and 71% thought that seizures improved. Although univariate analysis showed multiple associations with stress as a seizure precipitant, in the multivariable logistic regression, only the GAD-7 score was associated with STRESS (+) (OR = 1.18 [1.03-1.35], p = 0.017). Subjects who reported stress as a seizure precipitant were more likely to report an ability to self-predict seizures (p < 0.001). Stress-precipitated seizures are commonly reported

  11. Specific features of early post-stroke seizures

    Directory of Open Access Journals (Sweden)

    Tatyana Valeryevna Danilova

    2012-01-01

    Full Text Available The paper presents the results of examining 101 patients (68 men and 33 women aged 48 to 89 years with seizures in the first 7 days of stroke. A control group comprised 97 patients who had experienced ischemic stroke without seizures. Early seizures more frequently occurred in the cardioembolic subtype of stroke as simple partial seizures. The neuroimaging features of ischemic foci were revealed and the cerebrovascular responsiveness was evaluated in different vascular basins in these patients.

  12. 50 CFR 12.5 - Seizure by other agencies.

    Science.gov (United States)

    2010-10-01

    ... 50 Wildlife and Fisheries 1 2010-10-01 2010-10-01 false Seizure by other agencies. 12.5 Section 12... SEIZURE AND FORFEITURE PROCEDURES General Provisions § 12.5 Seizure by other agencies. Any authorized... the laws listed in § 12.2 will, if so requested, deliver such seizure to the appropriate Special Agent...

  13. Seizure characteristics in multiple sclerosis patients

    Directory of Open Access Journals (Sweden)

    Vahid Shaygannejad

    2013-01-01

    Full Text Available Background: To evaluate seizure characteristic among multiple sclerosis patients with coexistent seizure activity compared to control group. Materials and Methods : This study is a cross-sectional study which was conducted by reviewing the clinical records of patients with definite diagnosis of MS according to McDonald′s criteria from March 2007 to June 2011, who referred to the MS clinic of the university. Results : A total of 920 patients with a diagnosis of MS were identified, among whom 29 patients (3.15% with seizure activity (case due to MS with the mean age of 32.6 ± 6.23 years were analyzed. Also, fifty MS patients without any seizure occurrence with the mean age of 33.7 ± 7.4 years were used as our control group. In case group, seizure was general tonic clonic in 23 patients (79.3%, complex partial in four (13.8%, and simple partial in two (5.9%. The 26 available interictal EEGs in MS patients showed abnormal EEG pattern in 22 (84.6% of them, including focal epileptic form discharge or focal slowing in 10 (38.5%, generalized discharge (spike-wave, polyspike, or general paroxysmal fast activity in 10 (38.5%, and general slowing activity in 10 record (38.5%. MRI reviews of the 26 available brain MRIs showed subcortical white mater lesions in 22 (84.6% of patients with seizure. All MRIs were performed within one month after the first seizure episode. Amongst 48 available MRIs in our control group, 91.7% (44 cases showed periventricular lesions and in 8.3% (4 cases subcortical white matter lesions were reported. Conclusion : The result of this study demonstrated the higher rate of subcortical whit matter lesion in MS patients with seizure occurrence compared to control group.

  14. Seizures in dominantly inherited Alzheimer disease.

    Science.gov (United States)

    Zarea, Aline; Charbonnier, Camille; Rovelet-Lecrux, Anne; Nicolas, Gaël; Rousseau, Stéphane; Borden, Alaina; Pariente, Jeremie; Le Ber, Isabelle; Pasquier, Florence; Formaglio, Maite; Martinaud, Olivier; Rollin-Sillaire, Adeline; Sarazin, Marie; Croisile, Bernard; Boutoleau-Bretonnière, Claire; Ceccaldi, Mathieu; Gabelle, Audrey; Chamard, Ludivine; Blanc, Frédéric; Sellal, François; Paquet, Claire; Campion, Dominique; Hannequin, Didier; Wallon, David

    2016-08-30

    To assess seizure frequency in a large French cohort of autosomal dominant early-onset Alzheimer disease (ADEOAD) and to determine possible correlations with causative mutations. A national multicentric study was performed in patients with ADEOAD harboring a pathogenic mutation within PSEN1, PSEN2, APP, or a duplication of APP, and a minimal follow-up of 5 years. Clinical, EEG, and imaging data were systematically recorded. We included 132 patients from 77 families: 94 PSEN1 mutation carriers (MCs), 16 APP duplication carriers, 15 APP MCs, and 7 PSEN2 MCs. Seizure frequency was 47.7% after a mean follow-up of 8.4 years (range 5-25). After 5-year follow-up and using a Cox model analysis, the percentages of patients with seizures were respectively 19.1% (10.8%-26.7%) for PSEN1, 28.6% (0%-55.3%) for PSEN2, 31.2% (4.3%-50.6%) for APP duplications, and no patient for APP mutation. APP duplication carriers showed a significantly increased seizure risk compared to both APP MCs (hazard ratio [HR] = 5.55 [95% confidence interval 1.87-16.44]) and PSEN1 MCs (HR = 4.46 [2.11-9.44]). Among all PSEN1 mutations, those within the domains of protein hydrophilic I, transmembrane II (TM-II), TM-III, TM-IV, and TM-VII were associated with a significant increase in seizure frequency compared to other domains (HR = 4.53 [1.93-10.65], p = 0.0005). Seizures are a common feature of ADEOAD. In this population, risk was significantly higher in the APP duplication group than in all other groups. Within PSEN1, 5 specific domains were associated with a higher seizure risk indicating specific correlations between causative mutation and seizures. © 2016 American Academy of Neurology.

  15. Evaluation of Seizure Attacks in Patients with Cerebrovascular Accident

    Directory of Open Access Journals (Sweden)

    Ebrahim Koochaki

    2013-04-01

    Full Text Available Background: The most common reason for seizure in elderly duration is the stroke. This study was conducted aiming to assess the frequency of seizure attack occurrence in those patients. Materials and Methods: This investigation was carried out through a cross-sectional method for one year on 330 patients admitted to the neurology ward as diagnosed with stroke. The required data was collected through the researcher-made questionnaire from the patients suffering from stoke which was diagnosed based on clinical findings, CT-Scan and MRI as required. Results: Among 330 patient suffering from stroke (162 men and 168 women, 48 cases (14.5% were suffering from seizure. Six percent of the patients had early seizure and another 8.5% had late seizure. Among 162 men suffering from the stroke, 32 ones were without seizures and 30 men were suffering the seizure. A number of 150 women out of total 168 ones suffering from the stroke, had no seizure and 18 others had seizures; frequency of seizure occurrence was more in male samples (p=0.044. In the people under 60 year, there were mostly early types of seizure (45% and in the age range above 60 year, it was mostly late type (89.3%. A 68.5% of the patients suffering from the seizure had experienced ischemic stroke. However, the frequency of seizure occurrence in the patients with hemorrhagic stroke was statistically greater (p=0.003. Conclusion: This examination showed that occurrence of seizure attacks in the people with stroke is 14.5% and it is seen more in the hemorrhagic strokes than ischemic ones. The frontoparietal area is the most common location involved and tonic clonic was the most common seizure in the patients suffering from it who have experienced the stroke

  16. 27 CFR 447.63 - Seizure and forfeiture.

    Science.gov (United States)

    2010-04-01

    ... 27 Alcohol, Tobacco Products and Firearms 3 2010-04-01 2010-04-01 false Seizure and forfeiture. 447.63 Section 447.63 Alcohol, Tobacco Products, and Firearms BUREAU OF ALCOHOL, TOBACCO, FIREARMS... IMPLEMENTS OF WAR Penalties, Seizures and Forfeitures § 447.63 Seizure and forfeiture. Whoever knowingly...

  17. 9 CFR 118.4 - Seizure and condemnation.

    Science.gov (United States)

    2010-01-01

    ... 9 Animals and Animal Products 1 2010-01-01 2010-01-01 false Seizure and condemnation. 118.4... AGRICULTURE VIRUSES, SERUMS, TOXINS, AND ANALOGOUS PRODUCTS; ORGANISMS AND VECTORS DETENTION; SEIZURE AND CONDEMNATION § 118.4 Seizure and condemnation. Any biological product which is prepared, sold, bartered...

  18. Mozart K.448 listening decreased seizure recurrence and epileptiform discharges in children with first unprovoked seizures: a randomized controlled study.

    Science.gov (United States)

    Lin, Lung-Chang; Lee, Mei-Wen; Wei, Ruey-Chang; Mok, Hin-Kiu; Yang, Rei-Cheng

    2014-01-13

    Increasing numbers of reports show the beneficial effects of listening to Mozart music in decreasing epileptiform discharges as well as seizure frequency in epileptic children. There has been no effective method to reduce seizure recurrence after the first unprovoked seizure until now. In this study, we investigated the effect of listening to Mozart K.448 in reducing the seizure recurrence rate in children with first unprovoked seizures. Forty-eight children who experienced their first unprovoked seizure with epileptiform discharges were included in the study. They were randomly placed into treatment (n = 24) and control (n = 24) groups. Children in the treatment group listened to Mozart K.448 daily before bedtime for at least six months. Two patients in the treatment group were excluded from analysis due to discontinuation intervention. Finally, forty-six patients were analyzed. Most of these patients (89.1%) were idiopathic in etiology. Seizure recurrence rates and reduction of epileptiform discharges were compared. The average follow-up durations in the treatment and control groups were 18.6 ± 6.6 and 20.1 ± 5.1 months, respectively. The seizure recurrence rate was estimated to be significantly lower in the treatment group than the control group over 24 months (37.2% vs. 76.8%, p = 0.0109). Significant decreases in epileptiform discharges were also observed after 1, 2, and 6 months of listening to Mozart K.448 when compared with EEGs before listening to music. There were no significant differences in gender, mentality, seizure type, and etiology between the recurrence and non-recurrence groups. Although the case number was limited and control music was not performed in this study, the study revealed that listening to Mozart K.448 reduced the seizure recurrence rate and epileptiform discharges in children with first unprovoked seizures, especially of idiopathic etiology. We believe that Mozart K.448 could be a promising alternative treatment in patients with

  19. Tranexamic acid-associated seizures: Causes and treatment.

    Science.gov (United States)

    Lecker, Irene; Wang, Dian-Shi; Whissell, Paul D; Avramescu, Sinziana; Mazer, C David; Orser, Beverley A

    2016-01-01

    Antifibrinolytic drugs are routinely used worldwide to reduce the bleeding that results from a wide range of hemorrhagic conditions. The most commonly used antifibrinolytic drug, tranexamic acid, is associated with an increased incidence of postoperative seizures. The reported increase in the frequency of seizures is alarming, as these events are associated with adverse neurological outcomes, longer hospital stays, and increased in-hospital mortality. However, many clinicians are unaware that tranexamic acid causes seizures. The goal of this review is to summarize the incidence, risk factors, and clinical features of these seizures. This review also highlights several clinical and preclinical studies that offer mechanistic insights into the potential causes of and treatments for tranexamic acid-associated seizures. This review will aid the medical community by increasing awareness about tranexamic acid-associated seizures and by translating scientific findings into therapeutic interventions for patients. © 2015 The Authors Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association.

  20. Posterior Reversible Encephalopathy Syndrome (PRES Associated with Eclampsia: A Case Study

    Directory of Open Access Journals (Sweden)

    Maasoumeh Mirzamoradi

    2017-03-01

    Full Text Available The occurrence of posterior reversible encephalopathy syndrome (PRES in patients with eclampsia is a rare condition. PRES is a reversible syndrome characterized by headache, seizure, altered mentation and loss of vision associated with white matter changes on imaging. The lesions in PRES are thought to be due to vasogenic oedema, predominantly in the posterior cerebral hemispheres. This study reports a 16-year-old pregnant woman who presented with blindness and seizure. The MRI of her brain showed abnormal signal intensity in the white matter of the occipital and frontal lobes. She was treated successfully with pregnancy termination, anti-hypertensives, anticonvulsants, and supportive care. It is concluded that early diagnosis is important to prevent permanent neurologic damage and mortality.

  1. Cerebrospinal fluid findings after epileptic seizures.

    Science.gov (United States)

    Chatzikonstantinou, Anastasios; Ebert, Anne D; Hennerici, Michael G

    2015-12-01

    We aimed to evaluate ictally-induced CSF parameter changes after seizures in adult patients without acute inflammatory diseases or infectious diseases associated with the central nervous system. In total, 151 patients were included in the study. All patients were admitted to our department of neurology following acute seizures and received an extensive work-up including EEG, cerebral imaging, and CSF examinations. CSF protein elevation was found in most patients (92; 60.9%) and was significantly associated with older age, male sex, and generalized seizures. Abnormal CSF-to-serum glucose ratio was found in only nine patients (5.9%) and did not show any significant associations. CSF lactate was elevated in 34 patients (22.5%) and showed a significant association with focal seizures with impaired consciousness, status epilepticus, the presence of EEG abnormalities in general and epileptiform potentials in particular, as well as epileptogenic lesions on cerebral imaging. Our results indicate that non-inflammatory CSF elevation of protein and lactate after epileptic seizures is relatively common, in contrast to changes in CSF-to-serum glucose ratio, and further suggest that these changes are caused by ictal activity and are related to seizure type and intensity. We found no indication that these changes may have further-reaching pathological implications besides their postictal character.

  2. 27 CFR 555.166 - Seizure or forfeiture.

    Science.gov (United States)

    2010-04-01

    ... 27 Alcohol, Tobacco Products and Firearms 3 2010-04-01 2010-04-01 false Seizure or forfeiture. 555... EXPLOSIVES, DEPARTMENT OF JUSTICE EXPLOSIVES COMMERCE IN EXPLOSIVES Unlawful Acts, Penalties, Seizures and Forfeitures § 555.166 Seizure or forfeiture. Any explosive materials involved or used or intended to be used...

  3. Acute postoperative seizures and long-term seizure outcome after surgery for hippocampal sclerosis.

    Science.gov (United States)

    Di Gennaro, Giancarlo; Casciato, Sara; Quarato, Pier Paolo; Mascia, Addolorata; D'Aniello, Alfredo; Grammaldo, Liliana G; De Risi, Marco; Meldolesi, Giulio N; Romigi, Andrea; Esposito, Vincenzo; Picardi, Angelo

    2015-01-01

    To assess the incidence and the prognostic value of acute postoperative seizures (APOS) in patients surgically treated for drug-resistant temporal lobe epilepsy due to hippocampal sclerosis (TLE-HS). We studied 139 consecutive patients with TLE-HS who underwent epilepsy surgery and were followed up for at least 5 years (mean duration of follow-up 9.1 years, range 5-15). Medical charts were reviewed to identify APOS, defined as ictal events with the exception of auras occurring within the first 7 days after surgery. Seizure outcome was determined at annual intervals. Patients who were in Engel Class Ia at the last contact were classified as having a favorable outcome. Seizure outcome was favorable in 99 patients (71%). Six patients (4%) experienced APOS and in all cases their clinical manifestations were similar to the habitual preoperative seizures. All patients with APOS had unfavorable long-term outcome, as compared with 35 (26%) of 133 in whom APOS did not occur (pseizure outcome. Given some study limitations, our findings should be regarded as preliminary and need confirmation from future larger, prospective, multicenter studies. Copyright © 2014 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  4. Fahr’s Syndrome and Secondary Hypoparathyroidism

    Directory of Open Access Journals (Sweden)

    Santos Vitorino Modesto dos

    2016-03-01

    Full Text Available A typical case of Fahr’s syndrome is described in a 76-year-old Brazilian female who underwent a total thyroidectomy three decades ago. Six years before the current admission, she started with generalized tonic-clonic seizures. Associated disorders involved extra-pyramidal, cognitive, nocturnal terror and mood changes. With suspicion of hypocalcemia due to secondary hypoparathyroidism, laboratory determinations confirmed the diagnoses. Furthermore, imaging studies of the central nervous system detected multiple calcifications, with characteristic distribution of Fahr’s syndrome. Clinical management was successful.

  5. Source and sink nodes in absence seizures.

    Science.gov (United States)

    Rodrigues, Abner C; Machado, Birajara S; Caboclo, Luis Otavio S F; Fujita, Andre; Baccala, Luiz A; Sameshima, Koichi

    2016-08-01

    As opposed to focal epilepsy, absence seizures do not exhibit a clear seizure onset zone or focus since its ictal activity rapidly engages both brain hemispheres. Yet recent graph theoretical analysis applied to absence seizures EEG suggests the cortical focal presence, an unexpected feature for this type of epilepsy. In this study, we explore the characteristics of absence seizure by classifying the nodes as to their source/sink natures via weighted directed graph analysis based on connectivity direction and strength estimation using information partial directed coherence (iPDC). By segmenting the EEG signals into relatively short 5-sec-long time windows we studied the evolution of coupling strengths from both sink and source nodes, and the network dynamics of absence seizures in eight patients.

  6. Temporal seizure focus and status epilepticus are associated with high-sensitive troponin I elevation after epileptic seizures.

    Science.gov (United States)

    Chatzikonstantinou, Anastasios; Ebert, Anne D; Hennerici, Michael G

    2015-09-01

    Postictal elevation of high-sensitive troponin I (TNI), a highly specific biomarker for myocardial ischemia, has been reported. We aimed at evaluating its association of high-sensitive troponin I (TNI) with seizure type and focus, as well as vascular risk factors. TNI was measured in 247 patients admitted to our clinic via the emergency room with an acute epileptic seizure. TNI control measurements were performed in 61.5% of cases. All patients underwent electroencephalography and cerebral imaging. Seizure focus - when possible - was determined using results from these examinations as well as clinical data. Of 247 patients, 133 (53.8%) were men, the mean age was 59 ± 18 years. 70 (28.3%) patients had focal and 177 (71.7%) generalized seizures. Status epilepticus was present in 38 cases (15.4%). Mean TNI was 0.05 ± 0.17. TNI was elevated in 27 patients (10.9%). Higher age, status epilepticus and temporal seizure focus were significantly associated with TNI elevation in multivariate analysis. In 21 (13.8%) of the patients with TNI control measurement, TNI was continuously elevated. Higher age and temporal seizure focus were significantly associated with continuously high TNI. Coronary heart disease and vascular risk factors were significantly associated with high TNI only in univariate analysis. No patient had a symptomatic myocardial ischemia. Postictal TNI elevation is relatively common in older patients with status epilepticus or temporal seizure focus. These data support the concept of relevant and possibly dangerous ictal effects on cardiac function especially in temporal lobe seizures. Although the risk of manifest postictal myocardial infarction seems to be very low, selected patients could profit from closer monitoring. Copyright © 2015 Elsevier B.V. All rights reserved.

  7. Contributions of fMRI towards our understanding of the response to psychosocial stress in epilepsy and psychogenic nonepileptic seizures.

    Science.gov (United States)

    Allendorfer, Jane B; Szaflarski, Jerzy P

    2014-06-01

    There are multiple definitions of stress. For this review, as a reference point, we will use the concept of acute emotional/psychosocial stress ("stress"). The presence of acute stress has been reported to have a significant effect on seizure control, with several studies showing patients with seizure disorders being able to predict with reasonable accuracy seizure occurrence within the following hours or days. However, neuroimaging investigations of the pathophysiological mechanisms underlying stress reactivity (e.g., hypothalamic-pituitary-adrenal (HPA) axis activation) in humans, in general, and in patients with seizure disorders, in particular, are scarce. The reasons for this are multiple and likely include difficulty with designing appropriate probes that test various aspects of stress response, obtaining approval for studies that induce stress in patients who are prone to having stress-induced seizures, difficulties with assessing the physiological response to stress inside the scanner (e.g., heart rate, respiratory rate, oxygenation, cortisol levels, and galvanic skin responses), participant identification, and choice of epilepsy syndrome for investigation. With the recent explosion of neuroimaging literature focusing on correlating stress of various types and levels with cortical activations in healthy and diseased populations, it is incumbent upon us to examine the available neuroimaging data in patients with seizure disorders in order to identify the existing gaps and the needs/directions for future investigations. This approach is consistent with the goals of several of the 2014 Benchmarks for Epilepsy Research for the National Institute of Neurological Disorders and Stroke and the American Epilepsy Society. Copyright © 2014 Elsevier Inc. All rights reserved.

  8. Chediak-Higashi syndrome presenting in the accelerated phase

    African Journals Online (AJOL)

    Chediak-Higashi syndrome (CHS) is an extremely rare autosomal recessive disorder characterised by recurrent pyogenic ... Genetic studies suggest a mutation in the lysosomal trafficking ... neuropathy and seizures in adolescence and early adulthood. ... of ataxia, tremors, muscle weakness, sensory loss, cranial nerve. Fig.

  9. Epileptic seizures in patients with a posterior circulation infarct

    Directory of Open Access Journals (Sweden)

    Yüksel Kaplan

    2014-08-01

    Full Text Available OBJECTIVE: The aim of this study was to investigate the frequency of seizures and the clinical features of patients with seizures related to a posterior circulation infarct (POCI. METHODS: We reviewed all ischemic stroke patients admitted to our clinic between January 2011 and January 2012. The patients’ database information was retrospectively analyzed. Fifty-five patients with a POCI were included in the study. We reviewed all patients with epileptic seizures related to a POCI. Age, gender, recurrent stroke, risk factors, etiology, radiographic localization, the seizure type and onset time, and the electroencephalographic findings of patients were evaluated. We excluded all patients who had precipitating conditions during seizures such as taking drugs, acid-base disturbances, electrolyte imbalance, and history of epilepsy. RESULTS: Seizures were observed in four patients (3 male, 1 female with a POCI related epileptic seizures (7.2%. The etiology of strokes was cardiac-embolic in 3 patients and vertebral artery dissection in 1 patient. Seizures occurred in 2 patients as presenting finding, in 1 patient within 7 days, and 1 patient within 28 days. Primary generalized tonic-clonic seizures occurred in 3 patients and simple partial seizures with secondary generalization in 1 patient. Three patients had cerebellum infarction at the left hemisphere. One patient had lateral medullary infarction at the right side. The electroencephalographic findings of patients were normal. CONCLUSION: Studies involving patients with seizures related to a POCI are novel and few in number. Three patients with seizure had cerebellum infarction. The cerebellum in these patients may contribute via different mechanisms over seizure activity.

  10. Diagnosing psychogenic nonepileptic seizures: Video-EEG monitoring, suggestive seizure induction and diagnostic certainty.

    Science.gov (United States)

    Popkirov, Stoyan; Jungilligens, Johannes; Grönheit, Wenke; Wellmer, Jörg

    2017-08-01

    Psychogenic nonepileptic seizures (PNES) can remain undiagnosed for many years, leading to unnecessary medication and delayed treatment. A recent report by the International League Against Epilepsy Nonepileptic Seizures Task Force recommends a staged approach to the diagnosis of PNES (LaFrance, et al., 2013). We aimed to investigate its practical utility, and to apply the proposed classification to evaluate the role of long-term video-EEG monitoring (VEEG) and suggestive seizure induction (SSI) in PNES workup. Using electronic medical records, 122 inpatients (mean age 36.0±12.9years; 68% women) who received the diagnosis of PNES at our epilepsy center during a 4.3-year time period were included. There was an 82.8% agreement between diagnostic certainty documented at discharge and that assigned retroactively using the Task Force recommendations. In a minority of cases, having used the Task Force criteria could have encouraged the clinicians to give more certain diagnoses, exemplifying the Task Force report's utility. Both VEEG and SSI were effective at supporting high level diagnostic certainty. Interestingly, about one in four patients (26.2%) had a non-diagnostic ("negative") VEEG but a positive SSI. On average, this subgroup did not have significantly shorter mean VEEG recording times than VEEG-positive patients. However, VEEG-negative/SSI-positive patients had a significantly lower habitual seizure frequency than their counterparts. This finding emphasizes the utility of SSI in ascertaining the diagnosis of PNES in patients who do not have a spontaneous habitual event during VEEG due to, for example, low seizure frequency. Copyright © 2017 Elsevier Inc. All rights reserved.

  11. 14 CFR 13.17 - Seizure of aircraft.

    Science.gov (United States)

    2010-01-01

    ... 14 Aeronautics and Space 1 2010-01-01 2010-01-01 false Seizure of aircraft. 13.17 Section 13.17... INVESTIGATIVE AND ENFORCEMENT PROCEDURES Legal Enforcement Actions § 13.17 Seizure of aircraft. (a) Under... officer, or a Federal Aviation Administration safety inspector, authorized in an order of seizure issued...

  12. Sturge Weber Syndrome: review of literature with case illustration

    Directory of Open Access Journals (Sweden)

    Satyarthee Guru Dutta

    2017-03-01

    Full Text Available Sturge-Weber syndrome (SWS also called as encephalotrigeminal angiomatosis, is a sporadically occurring rare neuro-cutaneous syndrome, characterized by vascular malformation with capillary venous angiomas involving face, choroidal layer of eye globe and leptomeninges responsible for ophthamological as well as neurological signs and symptoms. Authors report an interesting case, a six year old girl, who presented with seizures, facial port wine stain and normal psychomotor development. CT scan showed left cerebral hemiatrophy, left frontal and parieto occipital calcification with cortical calcification in left high frontal convexity. Cranial MRI scan also confirmed finding of left cerebral hemiatrophy and also revealed presence of gyriform cortical calcification, prominent flow voids seen in left basal ganglia. Her seizure is well controlled with antiepileptic medication. The pertinent literature is reviewed and management of such cases is discussed briefly.

  13. Ictal technetium-99m ethyl cysteinate dimer single-photon emission tomographic findings and propagation of epileptic seizure activity in patients with extratemporal epilepsies

    International Nuclear Information System (INIS)

    Noachtar, S.; Arnold, S.; Werhahn, K.J.; Yousry, T.A.; Tatsch, K.

    1998-01-01

    We investigated the influence of the propagation of extratemporal epileptic seizure activity on the regional increase in cerebral blood flow, which is usually associated with epileptic seizure activity. Forty-two consecutive patients with extratemporal epilepsies were prospectively evaluated. All patients underwent ictal SPET studies with simultaneous electroencephalography (EEG) and video recordings of habitual seizures and imaging studies including cranial magnetic resonance imaging and positron emission tomography with 2-[ 18 F]-fluoro-2 deoxy-d-glucose. Propagation of epilptic seizure activity (PESA) was defined as the absence of hyperperfusion on ictal ECD SPET in the lobe of seizure onset, but its presence in another ipsilateral or contralateral lobe. Observers analysing the SPET images were not informed of the other results. PESA was observed in 8 of the 42 patients (19%) and was ipsilateral to the seizure onset in five (63%) of these eight patients. The time between clinical seizure onset and injection of the ECD tracer ranged from 14 to 61 s (mean 34 s). Seven patients (88%) with PESA had parieto-occipital epilepsy and one patient had a frontal epilepsy. PESA was statistically more frequent in patients with parieto-occipital lobe epilepsies (58%) than in the remaining extratemporal epilepsy syndromes (3%) (P<0.0002). These findings indicate that ictal SPET studies require simultaneous EEG-video recordings in patients with extratemporal epilepsies. PESA should be considered when interpreting ictal SPET studies in these patients. Patients with PESA are more likely to have parieto-occipital lobe epilepsy than seizure onset in other extratemporal regions. (orig./MG) (orig.)

  14. Predictors of seizure occurrence in children undergoing pre-surgical monitoring.

    Science.gov (United States)

    Harini, Chellamani; Singh, Kanwaljit; Takeoka, Masanori; Parulkar, Isha; Bergin, Ann Marie; Loddenkemper, Tobias; Kothare, Sanjeev V

    2013-10-01

    Long-Term-Monitoring (LTM) is a valuable tool for seizure localization/lateralization among children with refractory-epilepsy undergoing pre-surgical-monitoring. The aim of this study was to examine the factors predicting occurrence of single/multiple seizures in children undergoing pre-surgical monitoring in the LTM unit. Chart review was done on 95 consecutive admissions on 92 children (40 females) admitted to the LTM-unit for pre-surgical workup. Relationship between occurrence of multiple (≥ 3) seizures and factors such as home seizure-frequency, demographics, MRI-lesions/seizure-type and localization/AED usage/neurological-exam/epilepsy-duration was evaluated by logistic-regression and survival-analysis. Home seizure-frequency was further categorized into low (up-to 1/month), medium (up-to 1/week) and high (>1/week) and relationship of these categories to the occurrence of multiple seizures was evaluated. Mean length of stay was 5.24 days in all 3 groups. Home seizure frequency was the only factor predicting the occurrence of single/multiple seizures in children undergoing presurgical workup. Other factors (age/sex/MRI-lesions/seizure-type and localization/AED-usage/neurological-exam/epilepsy-duration) did not affect occurrence of single/multiple seizures or time-to-occurrence of first/second seizure. Analysis of the home-seizure frequency categories revealed that 98% admissions in high-frequency, 94% in the medium, and 77% in low-frequency group had at-least 1 seizure recorded during the monitoring. Odds of first-seizure increased in high vs. low-frequency group (p=0.01). Eighty-nine percent admissions in high-frequency, 78% in medium frequency, versus 50% in low-frequency group had ≥ 3 seizures. The odds of having ≥ 3 seizures increased in high-frequency (p=0.0005) and in medium-frequency (p=0.007), compared to low-frequency group. Mean time-to-first-seizure was 2.7 days in low-frequency, 2.1 days in medium, and 2 days in high-frequency group. Time-to-first-seizure

  15. A study of the dynamics of seizure propagation across micro domains in the vicinity of the seizure onset zone.

    Science.gov (United States)

    Basu, Ishita; Kudela, Pawel; Korzeniewska, Anna; Franaszczuk, Piotr J; Anderson, William S

    2015-08-01

    The use of micro-electrode arrays to measure electrical activity from the surface of the brain is increasingly being investigated as a means to improve seizure onset zone (SOZ) localization. In this work, we used a multivariate autoregressive model to determine the evolution of seizure dynamics in the [Formula: see text] Hz high frequency band across micro-domains sampled by such micro-electrode arrays. We showed that a directed transfer function (DTF) can be used to estimate the flow of seizure activity in a set of simulated micro-electrode data with known propagation pattern. We used seven complex partial seizures recorded from four patients undergoing intracranial monitoring for surgical evaluation to reconstruct the seizure propagation pattern over sliding windows using a DTF measure. We showed that a DTF can be used to estimate the flow of seizure activity in a set of simulated micro-electrode data with a known propagation pattern. In general, depending on the location of the micro-electrode grid with respect to the clinical SOZ and the time from seizure onset, ictal propagation changed in directional characteristics over a 2-10 s time scale, with gross directionality limited to spatial dimensions of approximately [Formula: see text]. It was also seen that the strongest seizure patterns in the high frequency band and their sources over such micro-domains are more stable over time and across seizures bordering the clinically determined SOZ than inside. This type of propagation analysis might in future provide an additional tool to epileptologists for characterizing epileptogenic tissue. This will potentially help narrowing down resection zones without compromising essential brain functions as well as provide important information about targeting anti-epileptic stimulation devices.

  16. Generalized epilepsy syndromes and callosal thickness: Differential effects between patients with juvenile myoclonic epilepsy and those with generalized tonic-clonic seizures alone.

    Science.gov (United States)

    Anastasopoulou, Stavroula; Kurth, Florian; Luders, Eileen; Savic, Ivanka

    2017-01-01

    The definition of two well-studied genetic generalized epilepsy syndromes (GGE) - juvenile myoclonic epilepsy (JME) and epilepsy with generalized tonic-clonic seizures alone (GTCS) - suggests the absence of structural cerebral abnormalities. Nevertheless, there are various reports of such abnormalities (especially in JME), where effects mainly occur within thalamus and mesial prefrontal regions. This raises the question of whether JME is particularly linked to midline structure abnormalities, which may also involve the corpus callosum. We studied callosal morphology in a well-matched sample of 22 JME patients, 15 GTCS patients, and 42 controls (CTL) for all of whom we obtained T1-weighted data on a 3T MRI scanner. More specifically, we measured callosal thickness at 100 equidistant points across the callosal surface, and subsequently compared the three groups (JME, GTCS, and CTL) against each other. Significant differences between JME patients and controls were observed within the callosal genu, anterior midbody, and isthmus, with thinner regions in JME patients. There were no significant differences between GTCS patients and controls, and also not between JME patients and GTCS patients. The present outcomes point to callosal abnormalities in JME patients suggesting an impairment of interhemisperic communication between prefrontal, motor, parietal and temporal cortices. These findings further support the notion that structural aberrations are present and differentiated across GGE syndromes, with significant callosal deviations from normality in JME. Copyright © 2016 Elsevier B.V. All rights reserved.

  17. The Anticonvulsant Effects of Ketogenic Diet on Epileptic Seizures and Potential Mechanisms.

    Science.gov (United States)

    Zhang, Yifan; Xu, Jingwei; Zhang, Kun; Yang, Wei; Li, Bingjin

    2018-01-01

    Epilepsy is a syndrome of brain dysfunction induced by the aberrant excitability of certain neurons. Despite advances in surgical technique and anti-epileptic drug in recent years, recurrent epileptic seizures remain intractable and lead to a serious morbidity in the world. The ketogenic diet refers to a high-fat, low-carbohydrate and adequate-protein diet. Currently, its beneficial effects on epileptic seizure reduction have been well established. However, the detailed mechanisms underlying the anti-epileptic effects of ketogenic diet are still poorly understood. In this article, the possible roles of ketogenic diet on epilepsy were discussed. Data was obtained from the websites including Web of Science, Medline, Pubmed, Scopus, based on these keywords: "Ketogenic diet" and "epilepsy". As shown in both clinical and basic studies, the therapeutic effects of ketogenic diet might involve neuronal metabolism, neurotransmitter function, neuronal membrane potential and neuron protection against ROS. In this review, we systematically reviewed the effects and possible mechanisms of ketogenic diet on epilepsy, which may optimize the therapeutic strategies against epilepsy. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  18. Detection of Epileptic Seizures with Multi-modal Signal Processing

    DEFF Research Database (Denmark)

    Conradsen, Isa

    convulsive seizures tested. Another study was performed, involving quantitative parameters in the time and frequency domain. The study showed, that there are several differences between tonic seizures and the tonic phase of GTC seizures and furthermore revealed differences of the epileptic (tonic and tonic...... phase of GTC) and simulated seizures. This was valuable information concerning a seizure detection algorithm, and the findings from this research provided evidence for a change in the definition of these seizures by the International League Against Epilepsy (ILAE). Our final study presents a novel...

  19. Towards an Online Seizure Advisory System—An Adaptive Seizure Prediction Framework Using Active Learning Heuristics

    NARCIS (Netherlands)

    Karuppiah Ramachandran, Vignesh Raja; Alblas, Huibert J.; Le Viet Duc, Duc Viet; Meratnia, Nirvana

    2018-01-01

    In the last decade, seizure prediction systems have gained a lot of attention because of their enormous potential to largely improve the quality-of-life of the epileptic patients. The accuracy of the prediction algorithms to detect seizure in real-world applications is largely limited because the

  20. SEIZURE PREDICTION: THE FOURTH INTERNATIONAL WORKSHOP

    Science.gov (United States)

    Zaveri, Hitten P.; Frei, Mark G.; Arthurs, Susan; Osorio, Ivan

    2010-01-01

    The recently convened Fourth International Workshop on Seizure Prediction (IWSP4) brought together a diverse international group of investigators, from academia and industry, including epileptologists, neurosurgeons, neuroscientists, computer scientists, engineers, physicists, and mathematicians who are conducting interdisciplinary research on the prediction and control of seizures. IWSP4 allowed the presentation and discussion of results, an exchange of ideas, an assessment of the status of seizure prediction, control and related fields and the fostering of collaborative projects. PMID:20674508

  1. Clinical intrafamilial variability in lethal familial neonatal seizure disorder caused by TBC1D24 mutations.

    Science.gov (United States)

    Lozano, Reymundo; Herman, Kristin; Rothfuss, Melanie; Rieger, Hillary; Bayrak-Toydemir, Pinar; Aprile, Davide; Fruscione, Floriana; Zara, Federico; Fassio, Anna

    2016-12-01

    TBC1D24-related disorders include a wide phenotypic ranging from mild to lethal seizure disorders, non-syndromic deafness, and composite syndromes such as DOORS (deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures). The TBC1D24 gene has a role in cerebral cortex development and in presynaptic neurotransmission. Here, we present a familial case of a lethal early-onset epileptic encephalopathy, associated with two novel compound heterozygous missense variants on the TBC1D24 gene, which were detected by exome sequencing. The detailed clinical data of the three siblings is summarized in order to support the variability of the phenotype, severity, and progression of this disorder among these family members. Functional studies demonstrated that the identified novel missense mutations result in a loss of expression of the protein, suggesting a correlation between residual expression, and the disease severity. This indicates that protein expression analysis is important for interpreting genetic results when novel variants are found, as well as for complementing clinical assessment by predicting the functional impact. Further analysis is necessary to delineate the clinical presentation of individuals with TBC1D24 pathogenic variants, as well as to develop markers for diagnosis, prognosis, and potential targeted treatments. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  2. Unilateral Thalamic Infarct Presenting as a Convulsive Seizure.

    Science.gov (United States)

    Kumar, Rajesh; Brohi, Hazim; Mughul, Afshan

    2017-09-01

    Lesions of the thalamus and those extending into midbrain can cause various types of movement disorders such as dystonia, asterixis and ballism-chorea. Seizures are rare manifestation of thalamic disorder. Occurrence of seizures in bilateral thalamic infarct has been reported; but seizures in unilateral thalamic infarct have been reported very rarely. Literature review showed only single case of perinatal unilateral thalamic infarct presenting with seizures. We are reporting a unique case of convulsive seizure at the onset of unilateral thalamic infarct in an adult male, which has never been reported to the best of our knowledge.

  3. 11p Microdeletion including WT1 but not PAX6, presenting with cataract, mental retardation, genital abnormalities and seizures: a case report

    DEFF Research Database (Denmark)

    Almind, Gitte J; Brøndum-Nielsen, Karen; Bangsgaard, Regitze

    2009-01-01

    , bilateral ptosis, genital abnormalities, seizures and a dysmorphic face. Cytogenetic analysis showed a deletion on 11p that was further characterized using FISH and MLPA analyses. The deletion (11p13-p12) located in the area between the deletions associated with the WAGR and Potocki-Shaffer syndromes had...

  4. Hungry Neurons: Metabolic Insights on Seizure Dynamics

    Directory of Open Access Journals (Sweden)

    Paolo Bazzigaluppi

    2017-10-01

    Full Text Available Epilepsy afflicts up to 1.6% of the population and the mechanisms underlying the appearance of seizures are still not understood. In past years, many efforts have been spent trying to understand the mechanisms underlying the excessive and synchronous firing of neurons. Traditionally, attention was pointed towards synaptic (dysfunction and extracellular ionic species (dysregulation. Recently, novel clinical and preclinical studies explored the role of brain metabolism (i.e., glucose utilization of seizures pathophysiology revealing (in most cases reduced metabolism in the inter-ictal period and increased metabolism in the seconds preceding and during the appearance of seizures. In the present review, we summarize the clinical and preclinical observations showing metabolic dysregulation during epileptogenesis, seizure initiation, and termination, and in the inter-ictal period. Recent preclinical studies have shown that 2-Deoxyglucose (2-DG, a glycolysis blocker is a novel therapeutic approach to reduce seizures. Furthermore, we present initial evidence for the effectiveness of 2-DG in arresting 4-Aminopyridine induced neocortical seizures in vivo in the mouse.

  5. Seizures beget seizures in temporal lobe epilepsies: the boomerang effects of newly formed aberrant kainatergic synapses.

    Science.gov (United States)

    Ben-Ari, Yehezkel; Crepel, Valérie; Represa, Alfonso

    2008-01-01

    Do temporal lobe epilepsy (TLE) seizures in adults promote further seizures? Clinical and experimental data suggest that new synapses are formed after an initial episode of status epilepticus, however their contribution to the transformation of a naive network to an epileptogenic one has been debated. Recent experimental data show that newly formed aberrant excitatory synapses on the granule cells of the fascia dentate operate by means of kainate receptor-operated signals that are not present on naive granule cells. Therefore, genuine epileptic networks rely on signaling cascades that differentiate them from naive networks. Recurrent limbic seizures generated by the activation of kainate receptors and synapses in naive animals lead to the formation of novel synapses that facilitate the emergence of further seizures. This negative, vicious cycle illustrates the central role of reactive plasticity in neurological disorders.

  6. Population dose-response analysis of daily seizure count following vigabatrin therapy in adult and pediatric patients with refractory complex partial seizures.

    Science.gov (United States)

    Nielsen, Jace C; Hutmacher, Matthew M; Wesche, David L; Tolbert, Dwain; Patel, Mahlaqa; Kowalski, Kenneth G

    2015-01-01

    Vigabatrin is an irreversible inhibitor of γ-aminobutyric acid transaminase (GABA-T) and is used as an adjunctive therapy for adult patients with refractory complex partial seizures (rCPS). The purpose of this investigation was to describe the relationship between vigabatrin dosage and daily seizure rate for adults and children with rCPS and identify relevant covariates that might impact seizure frequency. This population dose-response analysis used seizure-count data from three pediatric and two adult randomized controlled studies of rCPS patients. A negative binomial distribution model adequately described daily seizure data. Mean seizure rate decreased with time after first dose and was described using an asymptotic model. Vigabatrin drug effects were best characterized by a quadratic model using normalized dosage as the exposure metric. Normalized dosage was an estimated parameter that allowed for individualized changes in vigabatrin exposure based on body weight. Baseline seizure rate increased with decreasing age, but age had no impact on vigabatrin drug effects after dosage was normalized for body weight differences. Posterior predictive checks indicated the final model was capable of simulating data consistent with observed daily seizure counts. Total normalized vigabatrin dosages of 1, 3, and 6 g/day were predicted to reduce seizure rates 23.2%, 45.6%, and 48.5%, respectively. © 2014, The American College of Clinical Pharmacology.

  7. Seizure Freedom in Children With Pathology-Confirmed Focal Cortical Dysplasia.

    Science.gov (United States)

    Mrelashvili, Anna; Witte, Robert J; Wirrell, Elaine C; Nickels, Katherine C; Wong-Kisiel, Lily C

    2015-12-01

    We evaluated the temporal course of seizure outcome in children with pathology-confirmed focal cortical dysplasia and explored predictors of sustained seizure freedom. We performed a single-center retrospective study of children ≤ 18 years who underwent resective surgery from January 1, 2000 through December 31, 2012 and had pathology-proven focal cortical dysplasia. Surgical outcome was classified as seizure freedom (Engel class I) or seizure recurrence (Engel classes II-IV). Fisher exact and nonparametric Wilcoxon ranksum tests were used, as appropriate. Survival analysis was based on seizure-free outcome. Patients were censored at the time of seizure recurrence or seizure freedom at last follow-up. Thirty-eight patients were identified (median age at surgery, 6.5 years; median duration of epilepsy, 3.3 years). Median time to last follow-up was 13.5 months (interquartile range, 7-41 months). Twenty patients (53%) were seizure free and 26 patients (68%) attained seizure freedom for a minimum of 3 months. Median time to seizure recurrence was 38 months (95% confidence interval, 6-109 months), and the cumulative seizure-free rate was 60% at 12 months (95% confidence interval, 43%-77%). Clinical features associated with seizure freedom at last follow-up included older age at seizure onset (P = .02), older age at surgery (P = .04), absent to mild intellectual disability before surgery (P = .05), and seizure freedom for a minimum of 3 months (P freedom included older age at seizure onset, older age at surgery, absent or mild intellectual disability at baseline, and seizure freedom for a minimum of 3 months. Copyright © 2015 Elsevier Inc. All rights reserved.

  8. Rapidly Learned Identification of Epileptic Seizures from Sonified EEG

    Directory of Open Access Journals (Sweden)

    Psyche eLoui

    2014-10-01

    Full Text Available Sonification refers to a process by which data are converted into sound, providing an auditory alternative to visual display. Currently, the prevalent method for diagnosing seizures in epilepsy is by visually reading a patient’s electroencephalogram (EEG. However, sonification of the EEG data provides certain advantages due to the nature of human auditory perception. We hypothesized that human listeners will be able to identify seizures from EEGs using the auditory modality alone, and that accuracy of seizure identification will increase after a short training session. Here we describe an algorithm we have used to sonify EEGs of both seizure and non-seizure activity, followed by a training study in which subjects listened to short clips of sonified EEGs and determine whether each clip was of seizure or normal activity, both before and after a short training session. Results show that before training subjects performed at chance level in differentiating seizures vs. non-seizures, but there was a significant improvement of accuracy after the training session. After training, subjects successfully distinguished seizures from non-seizures using the auditory modality alone. Further analyses using signal detection theory demonstrated improvement in sensitivity and reduction in response bias as a result of training. This study demonstrates the potential of sonified EEGs to be used for the detection of seizures. Future studies will attempt to increase accuracy using novel training and sonification modifications, with the goals of managing, predicting, and ultimately controlling seizures using sonification as a possible biofeedback-based intervention for epilepsy.

  9. Multi-modal Intelligent Seizure Acquisition (MISA) system - A new approach towards seizure detection based on full body motion measures

    DEFF Research Database (Denmark)

    Conradsen, Isa; Beniczky, Sándor; Wolf, Peter

    2009-01-01

    Many epilepsy patients cannot call for help during a seizure, because they are unconscious or because of the affection of their motor system or speech function. This can lead to injuries, medical complications and at worst death. An alarm system setting off at seizure onset could help to avoid...... hazards. Today no reliable alarm systems are available. A Multi-modal Intelligent Seizure Acquisition (MISA) system based on full body motion data seems as a good approach towards detection of epileptic seizures. The system is the first to provide a full body description for epilepsy applications. Three...... test subjects were used for this pilot project. Each subject simulated 15 seizures and in addition performed some predefined normal activities, during a 4-hour monitoring with electromyography (EMG), accelerometer, magnetometer and gyroscope (AMG), electrocardiography (ECG), electroencephalography (EEG...

  10. 27 CFR 555.186 - Seizure or forfeiture.

    Science.gov (United States)

    2010-04-01

    ... 27 Alcohol, Tobacco Products and Firearms 3 2010-04-01 2010-04-01 false Seizure or forfeiture. 555... Seizure or forfeiture. Any plastic explosive that does not contain a detection agent in violation of 18 U.S.C. 842(l)-(n) is subject to seizure and forfeiture, and all provisions of 19 U.S.C. 1595a...

  11. Anesthetic considerations for a pediatric patient with Wolf-Hirschhorn syndrome: a case report

    OpenAIRE

    Tsukamoto, Masanori; Yamanaka, Hitoshi; Yokoyama, Takeshi

    2017-01-01

    Wolf-Hirschhorn syndrome is a rare hereditary disease that results from a 4p chromosome deletion. Patients with this syndrome are characterized by craniofacial dysgenesis, seizures, growth delay, intellectual disability, and congenital heart disease. Although several cases have been reported, very little information is available on anesthetic management for patients with Wolf-Hirschhorn syndrome. We encountered a case requiring anesthetic management for a 2-year-old girl with Wolf-Hirschhorn ...

  12. Early follow-up data from seizure diaries can be used to predict subsequent seizures in same cohort by borrowing strength across participants

    Science.gov (United States)

    Hall, Charles B.; Lipton, Richard B.; Tennen, Howard; Haut, Sheryl R.

    2014-01-01

    Accurate prediction of seizures in persons with epilepsy offers opportunities for both precautionary measures and preemptive treatment. Previously identified predictors of seizures include patient-reported seizure anticipation, as well as stress, anxiety, and decreased sleep. In this study, we developed three models using 30 days of nightly seizure diary data in a cohort of 71 individuals with a history of uncontrolled seizures to predict subsequent seizures in the same cohort over a 30-day follow-up period. The best model combined the individual’s seizure history with that of the remainder of the cohort, resulting in 72% sensitivity for 80% specificity, and 0.83 area under the receiver operating characteristic curve. The possibility of clinically relevant prediction should be examined through electronic data capture and more specific and more frequent sampling, and with patient training to improve prediction. PMID:19138755

  13. A study of the dynamics of seizure propagation across micro domains in the vicinity of the seizure onset zone

    Science.gov (United States)

    Basu, Ishita; Kudela, Pawel; Korzeniewska, Anna; Franaszczuk, Piotr J.; Anderson, William S.

    2015-08-01

    Objective. The use of micro-electrode arrays to measure electrical activity from the surface of the brain is increasingly being investigated as a means to improve seizure onset zone (SOZ) localization. In this work, we used a multivariate autoregressive model to determine the evolution of seizure dynamics in the 70-110 Hz high frequency band across micro-domains sampled by such micro-electrode arrays. We showed that a directed transfer function (DTF) can be used to estimate the flow of seizure activity in a set of simulated micro-electrode data with known propagation pattern. Approach. We used seven complex partial seizures recorded from four patients undergoing intracranial monitoring for surgical evaluation to reconstruct the seizure propagation pattern over sliding windows using a DTF measure. Main results. We showed that a DTF can be used to estimate the flow of seizure activity in a set of simulated micro-electrode data with a known propagation pattern. In general, depending on the location of the micro-electrode grid with respect to the clinical SOZ and the time from seizure onset, ictal propagation changed in directional characteristics over a 2-10 s time scale, with gross directionality limited to spatial dimensions of approximately 9 m{{m}2}. It was also seen that the strongest seizure patterns in the high frequency band and their sources over such micro-domains are more stable over time and across seizures bordering the clinically determined SOZ than inside. Significance. This type of propagation analysis might in future provide an additional tool to epileptologists for characterizing epileptogenic tissue. This will potentially help narrowing down resection zones without compromising essential brain functions as well as provide important information about targeting anti-epileptic stimulation devices.

  14. Longitudinal changes in seizure outcomes after resection of cerebral cavernous malformations in patients presenting with seizures: a long-term follow-up of 46 patients.

    Science.gov (United States)

    Kim, Jiha; Kim, Chi Heon; Chung, Chun Kee

    2014-08-01

    Seizure is the most common presentation in patients with cerebral cavernous malformations (CCMs). Although many articles have documented seizure outcomes after resection of CCM, few have conducted long-term follow-ups; thus, the fluctuating seizure outcomes have been neglected. The purpose of this study is to describe long-term postoperative seizure outcomes in patients with CCM and to compare seizure outcomes between patients with sporadic seizures and those with chronic seizures. Forty-six patients with CCM presenting with seizures underwent surgery. The male-to-female ratio was 1:1, and the average age at initial seizure onset was 27.6 years. The mean preoperative seizure duration was 42.7 months. Patients were divided into two groups: a chronic group (N = 20) and a sporadic group (N = 26) according to seizure frequency and duration. The mean postoperative follow-up duration was 96.3 months, and the postoperative seizure outcomes were checked annually based upon Engel's classification. After the first year of follow-up, 80.8 % of the sporadic group and 75.0 % of the chronic group were evaluated as Engel class I. These rates increased to 100.0 % and 90.0 %, respectively, at the eighth year of follow-up. Overall, 29 (63.0 %) of the 46 patients experienced changes in seizure outcomes over the follow-up period. Despite their delayed improvements, the chronic group showed less favorable outcomes throughout follow-up (p = 0.025). Long-term follow-up is indispensable for accurately assessing postoperative seizure outcomes because these outcomes change continuously. We recommend earlier surgery to achieve seizure-free status in patients with CCM. However, even in the chronic group, surgery is recommended, considering the overall delayed improvement.

  15. Multiple Sclerosis: Can It Cause Seizures?

    Science.gov (United States)

    ... multiple sclerosis and epilepsy? Answers from B Mark Keegan, M.D. Epileptic seizures are more common in ... controlled with anti-seizure medication. With B Mark Keegan, M.D. Lund C, et al. Multiple sclerosis ...

  16. An Unusual Case of Posterior Reversible Encephalopathy Syndrome

    Directory of Open Access Journals (Sweden)

    Robert P. Zemple

    2017-07-01

    Full Text Available A 21-year-old pregnant female with no significant past medical history presented with acute onset headache and nausea as well as tonic-clonic seizures, then rapidly decompensated into a coma with complete absence of brainstem reflexes. The patient was ultimately diagnosed with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome and subsequent posterior reversible encephalopathy syndrome (PRES with brainstem involvement. Emergent delivery and blood pressure control resulted in rapid and complete neurologic recovery.

  17. Comparative sensitivity of quantitative EEG (QEEG) spectrograms for detecting seizure subtypes.

    Science.gov (United States)

    Goenka, Ajay; Boro, Alexis; Yozawitz, Elissa

    2018-02-01

    To assess the sensitivity of Persyst version 12 QEEG spectrograms to detect focal, focal with secondarily generalized, and generalized onset seizures. A cohort of 562 seizures from 58 patients was analyzed. Successive recordings with 2 or more seizures during continuous EEG monitoring for clinical indications in the ICU or EMU between July 2016 and January 2017 were included. Patient ages ranged from 5 to 64 years (mean = 36 years). There were 125 focal seizures, 187 secondarily generalized and 250 generalized seizures from 58 patients analyzed. Seizures were identified and classified independently by two epileptologists. A correlate to the seizure pattern in the raw EEG was sought in the QEEG spectrograms in 4-6 h EEG epochs surrounding the identified seizures. A given spectrogram was interpreted as indicating a seizure, if at the time of a seizure it showed a visually significant departure from the pre-event baseline. Sensitivities for seizure detection using each spectrogram were determined for each seizure subtype. Overall sensitivities of the QEEG spectrograms for detecting seizures ranged from 43% to 72%, with highest sensitivity (402/562,72%) by the seizure detection trend. The asymmetry spectrogram had the highest sensitivity for detecting focal seizures (117/125,94%). The FFT spectrogram was most sensitive for detecting secondarily generalized seizures (158/187, 84%). The seizure detection trend was the most sensitive for generalized onset seizures (197/250,79%). Our study suggests that different seizure types have specific patterns in the Persyst QEEG spectrograms. Identifying these patterns in the EEG can significantly increase the sensitivity for seizure identification. Copyright © 2018 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  18. Predictors of Recurrent Febrile Seizures in Iranian Children

    Directory of Open Access Journals (Sweden)

    Yousef Veisani

    2013-09-01

    Full Text Available A few factors appear to boost a child's risk of having recurrent febrile seizures, including young age during the first seizure, seizure type, and having immediate family members with a history of febrile seizures. The present study aimed to provide reliable information about recurrent febrile seizure in Iranian children. On the computerized literature valid on valid keyword with search in valid database PubMed, Scientific Information Databases (SID (, Global medical article limberly (Medlib, Iranian Biomedical Journals (Iran Medex, Iranian Journal Database (Magiran, and Google Scholar recruited in different geographic areas. To explore heterogeneity in studies I2 index was used. Meta-analysis used to data analysis with random effects model.Hospital data of 4,599 children with febrile seizure. Overall, 21 studies met our inclusion criteria. Febrile seizure in 2 age groups (<2 and 2-6 years were 55.8% (95% CI: 50.4-61.2 and 44.2% (95% CI: 38.8-61.2 respectively. Pooled recurrent rate of febrile seizure in Iran was 20.9% (95% CI: 12.3-29.5. In 28.8 (95% CI: 19.3-38.4, children there was positive family history. The mean prevalence of simple and complex seizures was 69.3% (95% CI: 59.5-79.0 and 28.3% (95% CI: 19.6-31.0 respectively. The rates in different geographical regions of central, east, and west of Iran, 25, 20.8 and 27.1% were estimated, respectively.According to the data the prevalence febrile seizure is higher in males and children under two years. Recurrence rate in Iran, similar to other studies performed in other regions of the world.

  19. Seizure precipitants (triggering factors) in patients with epilepsy.

    Science.gov (United States)

    Ferlisi, Monica; Shorvon, Simon

    2014-04-01

    adult epilepsy clinic population: (a) to identify the frequency of seizure precipitants (triggering factors) and their relative frequency in those with psychiatric disorders, and in those in remission or with active epilepsy, differences in frequency with regard to gender, seizure duration, number of drugs taken; (b) to determine which precipitants patients most commonly report; and (c) to identify differences in the distribution of precipitants among generalized, temporal, and extratemporal epilepsies. Consecutive patients attending a tertiary-care epilepsy clinic were prospectively and an open personal interview to identify and characterize seizure precipitants. Information about the epilepsy and clinical characteristics of patients was collected during the interview and from medical records. Of 104 patients, 97% cited at least one precipitant. Stress, sleep deprivation, and fatigue were the most frequently reported precipitants. Patients with psychological comorbidities reported a greater percentage of seizures with seizure precipitants. Patients with idiopathic generalized epilepsy seemed to be more sensitive to seizures during awakening and sleep deprivation, patients with extratemporal epilepsy reported more frequent seizures during sleep. There were no differences in frequency or type of seizure precipitants with regard to gender, seizure duration or frequency, and the number of antiepileptic drugs taken. The findings may have implications for the better management of epilepsy by increasing a focus on nonpharmacological therapy. The implications of the findings for nosology and causation of epilepsy are also briefly discussed. Copyright © 2014 Elsevier Inc. All rights reserved.

  20. Seizure Recognition and Observation: A Guide for Allied Health Professionals.

    Science.gov (United States)

    Epilepsy Foundation of America, Landover, MD.

    Intended for allied health professionals, this guide provides information on seizure recognition and classification to help them assist the patient, the family, and the treating physician in obtaining control of epileptic seizures. A section on seizure recognition describes epilepsy and seizures, covering seizure classification and the causes of…

  1. Inter-modality comparisons of seizure focus lateralization in complex partial seizures

    International Nuclear Information System (INIS)

    Meyer, P.T.; Cortes-Blanco, A.; Pourdehnad, M.; Desiderio, L.; Jang, S.; Alavi, A.; Levy-Reis, I.

    2001-01-01

    Anterior temporal lobectomy offers a high chance of seizure-free outcome in patients suffering from drug-refractory complex partial seizure (CPS) originating from the temporal lobe. Other than EEG, several functional and morphologic imaging methods are used to define the spatial seizure origin. The present study was undertaken to compare the merits of fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET), magnetic resonance imaging (MRI) and single-voxel proton MR spectroscopy (MRS) for the lateralization of temporal lobe seizure foci. The clinical charts and imaging data of 43 consecutive CPS patients were reviewed. Based on surface EEG, 31 patients were classified with temporal lobe epilepsy (TLE; 25 lateralized, 6 not lateralized) and 12 with non-temporal lobe epilepsy. All were examined by FDG-PET, MRS and MRI within 6 weeks. FDG-PET and MRI were interpreted visually, while the N-acetyl-aspartate to creatine ratio was used for MRS interpretation. One FDG-PET scan was invalid due to seizure activity post injection. The MR spectra could not be evaluated in five cases bilaterally and three cases unilaterally for technical reasons. A total of 15 patients underwent anterior temporal lobectomy. All showed a beneficial postoperative outcome. When the proportions of agreement between FDG-PET (0.77), MRI (0.58) and MRS (0.56) and surface EEG in TLE cases were compared, there were no significant differences (P>0.10). However, FDG-PET showed a significantly higher agreement (0.93) than MRI (0.60; P=0.03) with the side of successful temporal lobectomy. The concordance of MRS with the side of successful temporal lobectomy was intermediate (0.75). When the results of functional and morphologic imaging were combined, no significant differences were found between the rates of agreement of FDG-PET/MRI and MRS/MRI with EEG (0.80 vs 0.68; P=0.50) and with the side of successful temporal lobectomy (0.87 vs 0.92; P=0.50) in TLE cases. However, MRS/MRI showed

  2. Inter-modality comparisons of seizure focus lateralization in complex partial seizures

    Energy Technology Data Exchange (ETDEWEB)

    Meyer, P.T.; Cortes-Blanco, A.; Pourdehnad, M.; Desiderio, L.; Jang, S.; Alavi, A. [Pennsylvania Univ., Philadelphia, PA (United States). Dept. of Radiology; Levy-Reis, I. [Pennsylvania Univ., Philadelphia, PA (United States). Dept. of Neurology

    2001-10-01

    Anterior temporal lobectomy offers a high chance of seizure-free outcome in patients suffering from drug-refractory complex partial seizure (CPS) originating from the temporal lobe. Other than EEG, several functional and morphologic imaging methods are used to define the spatial seizure origin. The present study was undertaken to compare the merits of fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET), magnetic resonance imaging (MRI) and single-voxel proton MR spectroscopy (MRS) for the lateralization of temporal lobe seizure foci. The clinical charts and imaging data of 43 consecutive CPS patients were reviewed. Based on surface EEG, 31 patients were classified with temporal lobe epilepsy (TLE; 25 lateralized, 6 not lateralized) and 12 with non-temporal lobe epilepsy. All were examined by FDG-PET, MRS and MRI within 6 weeks. FDG-PET and MRI were interpreted visually, while the N-acetyl-aspartate to creatine ratio was used for MRS interpretation. One FDG-PET scan was invalid due to seizure activity post injection. The MR spectra could not be evaluated in five cases bilaterally and three cases unilaterally for technical reasons. A total of 15 patients underwent anterior temporal lobectomy. All showed a beneficial postoperative outcome. When the proportions of agreement between FDG-PET (0.77), MRI (0.58) and MRS (0.56) and surface EEG in TLE cases were compared, there were no significant differences (P>0.10). However, FDG-PET showed a significantly higher agreement (0.93) than MRI (0.60; P=0.03) with the side of successful temporal lobectomy. The concordance of MRS with the side of successful temporal lobectomy was intermediate (0.75). When the results of functional and morphologic imaging were combined, no significant differences were found between the rates of agreement of FDG-PET/MRI and MRS/MRI with EEG (0.80 vs 0.68; P=0.50) and with the side of successful temporal lobectomy (0.87 vs 0.92; P=0.50) in TLE cases. However, MRS/MRI showed

  3. 43 CFR 3.16 - Seizure.

    Science.gov (United States)

    2010-10-01

    ... 43 Public Lands: Interior 1 2010-10-01 2010-10-01 false Seizure. 3.16 Section 3.16 Public Lands: Interior Office of the Secretary of the Interior PRESERVATION OF AMERICAN ANTIQUITIES § 3.16 Seizure. Any object of antiquity taken, or collection made, on lands owned or controlled by the United States, without...

  4. Cortical heterotopia in Aicardi's syndrome - CT findings

    International Nuclear Information System (INIS)

    Besenski, N.; Bosnjak, V.; Ligutic, I.; Marusic-Della Marina, B.

    1988-01-01

    The case of 5-month-old female infant with Aicardi's syndrome is presented. The main clinical features were severe developmental retardation and intractable epileptic seizures. Ophthalmoscopic examination revealed pathognomonic choriorethinopathy. Ultrasonic examination of the brain detected agenesis of the corpus callosum, whereas CT showed a coexisting malformation of the brain, i.e. cortical heterotopia of the gray matter. Agenesis of the corpus callosum is an entity well-recognized by sonography. However, ultrasonography is an insufficient modality for the visualization of cortical heterotopia which is common to all cases of Aicardi's syndrome. Therefore, in cases of suspected Aicardi's syndrome CT is recommended, as it enables the diagnosis of cortical heterotopia. (orig.)

  5. Recessive loss-of-function mutations in AP4S1 cause mild fever-sensitive seizures, developmental delay and spastic paraplegia through loss of AP-4 complex assembly

    DEFF Research Database (Denmark)

    Hardies, Katia; May, Patrick; Djémié, Tania

    2015-01-01

    We report two siblings with infantile onset seizures, severe developmental delay and spastic paraplegia, in whom whole-genome sequencing revealed compound heterozygous mutations in the AP4S1 gene, encoding the σ subunit of the adaptor protein complex 4 (AP-4). The effect of the predicted loss-of-...... in reported patients, highlighting that seizures are part of the clinical manifestation of the AP-4 deficiency syndrome. We also hypothesize that endosomal trafficking is a common theme between heritable spastic paraplegia and some inherited epilepsies....

  6. Various ketogenic diets can differently support brain resistance against experimentally evoked seizures and seizure-induced elemental anomalies of hippocampal formation.

    Science.gov (United States)

    Chwiej, J; Patulska, A; Skoczen, A; Matusiak, K; Janeczko, K; Ciarach, M; Simon, R; Setkowicz, Z

    2017-07-01

    In this paper the influence of two different ketogenic diets (KDs) on the seizure-evoked elemental anomalies of hippocampal formation was examined. To achieve this purpose normal and pilocarpine treated rats previously fed with one of the two high fat and carbohydrate restricted diets were compared with animals on standard laboratory diet. The ketogenic ratios of the examined KDs were equal to 5:1 (KD1) and 9:1 (KD2). KD1 and standard diet fed animals presented similar patterns of seizure-evoked elemental changes in hippocampal formation. Also the analysis of behavioral data recorded after pilocarpine injection did not show any significant differences in intensity and duration of seizures between KD1 and standard diet fed animals. Higher ketogenic ratio KD2 introduced in the normal hippocampal formation prolonged changes in the accumulation of P, K, Zn and Ca. Despite this, both the intensity and duration of seizures were significantly reduced in rats fed with KD2 which suggests that its saving action on the nerve tissue may protect brain from seizure propagation. Also seizure-evoked elemental anomalies in KD2 animals were different than those observed for rats both on KD1 and standard diets. The comparison of seizure experiencing and normal rats on KD2, did not show any statistically significant differences in elemental composition of CA1 and H hippocampal areas whilst in CA3 area only Zn level changed as a result of seizures. DG was the area mostly affected by seizures in KD2 fed rats but areal densities of all examined elements increased in this hippocampal region. Copyright © 2017 Elsevier GmbH. All rights reserved.

  7. Loss of MeCP2 From Forebrain Excitatory Neurons Leads to Cortical Hyperexcitation and Seizures

    Science.gov (United States)

    Zhang, Wen; Peterson, Matthew; Beyer, Barbara; Frankel, Wayne N.

    2014-01-01

    Mutations of MECP2 cause Rett syndrome (RTT), a neurodevelopmental disorder leading to loss of motor and cognitive functions, impaired social interactions, and seizure at young ages. Defects of neuronal circuit development and function are thought to be responsible for the symptoms of RTT. The majority of RTT patients show recurrent seizures, indicating that neuronal hyperexcitation is a common feature of RTT. However, mechanisms underlying hyperexcitation in RTT are poorly understood. Here we show that deletion of Mecp2 from cortical excitatory neurons but not forebrain inhibitory neurons in the mouse leads to spontaneous seizures. Selective deletion of Mecp2 from excitatory but not inhibitory neurons in the forebrain reduces GABAergic transmission in layer 5 pyramidal neurons in the prefrontal and somatosensory cortices. Loss of MeCP2 from cortical excitatory neurons reduces the number of GABAergic synapses in the cortex, and enhances the excitability of layer 5 pyramidal neurons. Using single-cell deletion of Mecp2 in layer 2/3 pyramidal neurons, we show that GABAergic transmission is reduced in neurons without MeCP2, but is normal in neighboring neurons with MeCP2. Together, these results suggest that MeCP2 in cortical excitatory neurons plays a critical role in the regulation of GABAergic transmission and cortical excitability. PMID:24523563

  8. Intravenous Carbamazepine for Adults With Seizures.

    Science.gov (United States)

    Vickery, P Brittany; Tillery, Erika E; DeFalco, Alicia Potter

    2018-03-01

    To review the pharmacology, pharmacokinetics, efficacy, safety, dosage and administration, potential drug-drug interactions, and place in therapy of the intravenous (IV) formulation of carbamazepine (Carnexiv) for the treatment of seizures in adult patients. A comprehensive PubMed and EBSCOhost search (1945 to August 2017) was performed utilizing the keywords carbamazepine, Carnexiv, carbamazepine intravenous, IV carbamazepine, seizures, epilepsy, and seizure disorder. Additional data were obtained from literature review citations, manufacturer's product labeling, and Lundbeck website as well as Clinicaltrials.gov and governmental sources. All English-language trials evaluating IV carbamazepine were analyzed for this review. IV carbamazepine is FDA approved as temporary replacement therapy for treatment of adult seizures. Based on a phase I trial and pooled data from 2 open-label bioavailability studies comparing oral with IV dosing, there was no noted indication of loss of seizure control in patients switched to short-term replacement antiepileptic drug therapy with IV carbamazepine. The recommended dose of IV carbamazepine is 70% of the patient's oral dose, given every 6 hours via 30-minute infusions. The adverse effect profile of IV carbamazepine is similar to that of the oral formulation, with the exception of added infusion-site reactions. IV carbamazepine is a reasonable option for adults with generalized tonic-clonic or focal seizures, previously stabilized on oral carbamazepine, who are unable to tolerate oral medications for up to 7 days. Unknown acquisition cost and lack of availability in the United States limit its use currently.

  9. Etiology and Outcome of Neonatal Seizures

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2006-04-01

    Full Text Available The prognostic value of seizure etiology, neurologic examination, EEG, and neuroimaging in the neurodevelopmental outcome of 89 term infants with neonatal seizures was determined at the Children’s Hospital and Harvard Medical School, Boston, MA.

  10. Prenatal exposure to ionizing radiation and subsequent development of seizures

    International Nuclear Information System (INIS)

    Dunn, K.; Yoshimaru, H.; Otake, M.; Annegers, J.F.; Schull, W.J.

    1990-01-01

    Seizures are a frequent sequela of impaired brain development and can be expected to affect more children with radiation-related brain damage than children without such damage. This report deals with the incidence and type of seizures among survivors prenatally exposed to the atomic bombing of Hiroshima and Nagasaki, and their association with specific stages of prenatal development at the time of irradiation. Fetal radiation dose was assumed to be equal to the dose to the maternal uterus. Seizures here include all references in the clinical record to seizure, epilepsy, or convulsion. Histories of seizures were obtained at biennial routine clinical examinations starting at about the age of 2 years. These clinical records were used to classify seizures as febrile or unprovoked (without precipitating cause). No seizures were ascertained among subjects exposed 0-7 weeks after fertilization at doses higher than 0.10 Gy. The incidence of seizures was highest with irradiation at the eighth through the 15th week after fertilization among subjects with doses exceeding 0.10 Gy and was linearly related to the level of fetal exposure. This obtains for all seizures without regard to the presence of fever or precipitating causes, and for unprovoked seizures. When the 22 cases of severe mental retardation were excluded, the increase in seizures was only suggestively significant and only for unprovoked seizures. After exposure at later stages of development, there was no increase in recorded seizures

  11. Modeling Seizure Self-Prediction: An E-Diary Study

    Science.gov (United States)

    Haut, Sheryl R.; Hall, Charles B.; Borkowski, Thomas; Tennen, Howard; Lipton, Richard B.

    2013-01-01

    Purpose A subset of patients with epilepsy successfully self-predicted seizures in a paper diary study. We conducted an e-diary study to ensure that prediction precedes seizures, and to characterize the prodromal features and time windows that underlie self-prediction. Methods Subjects 18 or older with LRE and ≥3 seizures/month maintained an e-diary, reporting AM/PM data daily, including mood, premonitory symptoms, and all seizures. Self-prediction was rated by, “How likely are you to experience a seizure [time frame]”? Five choices ranged from almost certain (>95% chance) to very unlikely. Relative odds of seizure (OR) within time frames was examined using Poisson models with log normal random effects to adjust for multiple observations. Key Findings Nineteen subjects reported 244 eligible seizures. OR for prediction choices within 6hrs was as high as 9.31 (1.92,45.23) for “almost certain”. Prediction was most robust within 6hrs of diary entry, and remained significant up to 12hrs. For 9 best predictors, average sensitivity was 50%. Older age contributed to successful self-prediction, and self-prediction appeared to be driven by mood and premonitory symptoms. In multivariate modeling of seizure occurrence, self-prediction (2.84; 1.68,4.81), favorable change in mood (0.82; 0.67,0.99) and number of premonitory symptoms (1,11; 1.00,1.24) were significant. Significance Some persons with epilepsy can self-predict seizures. In these individuals, the odds of a seizure following a positive prediction are high. Predictions were robust, not attributable to recall bias, and were related to self awareness of mood and premonitory features. The 6-hour prediction window is suitable for the development of pre-emptive therapy. PMID:24111898

  12. The Emperor Dom Pedro II: his convulsive seizures when a boy O imperador Dom Pedro II: as suas crises convulsivas quando menino

    Directory of Open Access Journals (Sweden)

    Marleide da Mota Gomes

    2007-12-01

    Full Text Available INTRODUCTION: Dom Pedro II, the Prince Heir and Emperor under regency, in a delicate period of the construction of the Brazilian nation, had convulsive seizures. OBJECTIVE: To investigate the convulsive seizures and related syndromes of Dom Pedro II and his family, besides the physicians in charge of the health care. METHOD: Narrative review based on primary and secondary sources. CONCLUSION: The scattered and self-limited convulsive seizures associated with physical and mental integrity favored a benign prognosis. Dom Pedro and his family presented rich history of epileptic seizures and febrile convulsion. This variety resembles the diagnosis of generalized epilepsy with febrile seizures plus that seems to be a combination of several syndromes with shared genetic susceptibility.INTRODUÇÃO: Dom Pedro, o príncipe herdeiro e imperador sob regência, em período delicado da formação da nação brasileira, apresentou crises convulsivas que geraram preocupação para o país. OBJETIVO: Investigar a história da epilepsia de Dom Pedro II e da sua família e procurar identificar quais tipos de crises epilépticas estavam presentes, além dos médicos envolvidos com os cuidados de saúde. MÉTODO: Revisão narrativa baseada em fontes primárias e secundárias. CONCLUSÃO: As crises convulsivas esparsas e auto-limitadas associadas a higidez física e mental de Dom Pedro II sugerem um prognóstico benigno. A história de epilepsia idiopática e convulsões febris no imperador e em outros membros da sua família aponta para o diagnóstico mais provável de Epilepsia Generalizada com Convulsões Febris Plus que é determinada por uma combinação de alguns tipos de manifestações epilépticas com suscetibilidade genética compartilhada.

  13. Biotelemetry system for Epilepsy Seizure Control

    Energy Technology Data Exchange (ETDEWEB)

    Smith, LaCurtise; Bohnert, George W.

    2009-07-02

    The Biotelemetry System for Epilepsy Seizure Control Project developed and tested an automated telemetry system for use in an epileptic seizure prevention device that precisely controls localized brain temperature. This project was a result of a Department of Energy (DOE) Global Initiatives for Proliferation Prevention (GIPP) grant to the Kansas City Plant (KCP), Argonne National Laboratory (ANL), and Pacific Northwest National Laboratory (PNNL) to partner with Flint Hills Scientific, LLC, Lawrence, KS and Biophysical Laboratory Ltd (BIOFIL), Sarov, Russia to develop a method to help control epileptic seizures.

  14. Hypoparathyroidism Causing Seizures: When Epilepsy Does Not Fit

    Directory of Open Access Journals (Sweden)

    Faheem Seedat

    2018-01-01

    Full Text Available A 24-year-old man presented to the Chris Hani Baragwanath Academic Hospital emergency department with recurrent seizures having previously been diagnosed with epilepsy from age 14. The biochemical investigations and brain imaging were suggestive of seizures secondary to hypocalcemia, and a diagnosis of idiopathic hypoparathyroidism was confirmed. After calcium and vitamin D replacement, the patient recovered well and is seizure free, and off antiepileptic therapy. This case highlights the occurrence of brain calcinosis in idiopathic hypoparathyroidism; the occurrence of acute symptomatic seizures due to provoking factors other than epilepsy; and the importance, in the correct clinical setting, of considering alternative, and sometimes treatable, causes of seizures other than epilepsy.

  15. Cognitive-behavioral therapy for psychogenic nonepileptic seizures

    Science.gov (United States)

    Goldstein, L.H.; Chalder, T.; Chigwedere, C.; Khondoker, M.R.; Moriarty, J.; Toone, B.K.; Mellers, J.D.C.

    2010-01-01

    Objective: To compare cognitive-behavioral therapy (CBT) and standard medical care (SMC) as treatments for psychogenic nonepileptic seizures (PNES). Methods: Our randomized controlled trial (RCT) compared CBT with SMC in an outpatient neuropsychiatric setting. Sixty-six PNES patients were randomized to either CBT (plus SMC) or SMC alone, scheduled to occur over 4 months. PNES diagnosis was established by video-EEG telemetry for most patients. Exclusion criteria included comorbid history of epilepsy, <2 PNES/month, and IQ <70. The primary outcome was seizure frequency at end of treatment and at 6-month follow-up. Secondary outcomes included 3 months of seizure freedom at 6-month follow-up, measures of psychosocial functioning, health service use, and employment. Results: In an intention-to-treat analysis, seizure reduction following CBT was superior at treatment end (group × time interaction p < 0.0001; large to medium effect sizes). At follow-up, the CBT group tended to be more likely to have experienced 3 months of seizure freedom (odds ratio 3.125, p = 0.086). Both groups improved in some health service use measures and on the Work and Social Adjustment Scale. Mood and employment status showed no change. Conclusions: Our findings suggest that cognitive-behavioral therapy is more effective than standard medical care alone in reducing seizure frequency in PNES patients. Classification of evidence: This study provides Class III evidence that CBT in addition to SMC, as compared to SMC alone, significantly reduces seizure frequency in patients with PNES (change in median monthly seizure frequency: baseline to 6 months follow-up, CBT group, 12 to 1.5; SMC alone group, 8 to 5). GLOSSARY AED = antiepileptic drug; CBT = cognitive-behavioral therapy; CI = confidence interval; DSM-IV = Diagnostic and Statistical Manual of Mental Disorders, 4th edition; HADS = Hospital Anxiety and Depression Scale; IQR = interquartile range; ITT = intention-to-treat; OR = odds ratio; PNES

  16. The debate: Treatment after the first seizure - The CONTRA.

    Science.gov (United States)

    Steinhoff, Bernhard J

    2017-07-01

    In many instances antiepileptic drug (AED) treatment of epilepsy patients is sustained for a long time and is even a lifelong therapy. Chronic drug treatment naturally means the potential burden of adverse effects. The prognosis to remain seizure-free is good after a first seizure even without AEDs. Therefore one has to consider the possibility that freedom from seizures when AED treatment has been initiated after the first seizure may not in fact be the result of the AED treatment. On the other hand seizure-free patients without severe side effects most probably will not consider discontinuing AEDs. Therefore in these cases it will not be possible to discover whether AEDs are really necessary to maintain freedom from seizures. Furthermore it has been shown that the long-term prognosis is independent of whether AED treatment started after the first or a following seizure. Therefore in most instances AED treatment after a first seizure should be avoided. Copyright © 2016 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  17. Multicenter clinical assessment of improved wearable multimodal convulsive seizure detectors.

    Science.gov (United States)

    Onorati, Francesco; Regalia, Giulia; Caborni, Chiara; Migliorini, Matteo; Bender, Daniel; Poh, Ming-Zher; Frazier, Cherise; Kovitch Thropp, Eliana; Mynatt, Elizabeth D; Bidwell, Jonathan; Mai, Roberto; LaFrance, W Curt; Blum, Andrew S; Friedman, Daniel; Loddenkemper, Tobias; Mohammadpour-Touserkani, Fatemeh; Reinsberger, Claus; Tognetti, Simone; Picard, Rosalind W

    2017-11-01

    New devices are needed for monitoring seizures, especially those associated with sudden unexpected death in epilepsy (SUDEP). They must be unobtrusive and automated, and provide false alarm rates (FARs) bearable in everyday life. This study quantifies the performance of new multimodal wrist-worn convulsive seizure detectors. Hand-annotated video-electroencephalographic seizure events were collected from 69 patients at six clinical sites. Three different wristbands were used to record electrodermal activity (EDA) and accelerometer (ACM) signals, obtaining 5,928 h of data, including 55 convulsive epileptic seizures (six focal tonic-clonic seizures and 49 focal to bilateral tonic-clonic seizures) from 22 patients. Recordings were analyzed offline to train and test two new machine learning classifiers and a published classifier based on EDA and ACM. Moreover, wristband data were analyzed to estimate seizure-motion duration and autonomic responses. The two novel classifiers consistently outperformed the previous detector. The most efficient (Classifier III) yielded sensitivity of 94.55%, and an FAR of 0.2 events/day. No nocturnal seizures were missed. Most patients had seizure frequency. When increasing the sensitivity to 100% (no missed seizures), the FAR is up to 13 times lower than with the previous detector. Furthermore, all detections occurred before the seizure ended, providing reasonable latency (median = 29.3 s, range = 14.8-151 s). Automatically estimated seizure durations were correlated with true durations, enabling reliable annotations. Finally, EDA measurements confirmed the presence of postictal autonomic dysfunction, exhibiting a significant rise in 73% of the convulsive seizures. The proposed multimodal wrist-worn convulsive seizure detectors provide seizure counts that are more accurate than previous automated detectors and typical patient self-reports, while maintaining a tolerable FAR for ambulatory monitoring. Furthermore, the multimodal system

  18. Panayiotopoulos syndrome and symptomatic occipital lobe epilepsy of childhood: a clinical and EEG study.

    Science.gov (United States)

    Tata, Gulten; Guveli, Betul Tekin; Dortcan, Nimet; Cokar, Ozlem; Kurucu, Hatice; Demirbilek, Veysi; Dervent, Aysin

    2014-06-01

    Panayiotopoulos syndrome (PS) is an age-related seizure susceptibility syndrome that affects the central autonomic system. Although the majority of the few ictal recordings obtained so far suggest an occipital origin, semiological and interictal EEG data appear to favour more extensive involvement. In this study, the characteristics (including those based on semiology and EEG) of children with Panayiotopoulos syndrome (n=24) and those with lesion-related, symptomatic occipital lobe epilepsy (SOLE) (n=23) were compared. Detailed semiological information and EEG parameters including the localisation, distribution, density (n/sec), reactivity, and morphological characteristics of spike-wave foci and their relationship with different states of vigilance were compared between the two groups. The age at seizure onset was significantly younger in patients with symptomatic occipital lobe epilepsy than in those with PS (mean age at onset: 3.4 versus 5.6 years, respectively; p=0.044). Autonomic seizures (p=0.001) and ictal syncope (p=0.055) were more frequent in PS than in symptomatic occipital lobe epilepsy (87.5% and 37.5% versus 43.5% and 13%, respectively). The interictal spike-wave activity increased significantly during non-rapid eye movement (non-REM) sleep in both groups. The spike waves in non-REM seen in PS tended to spread mainly to central and centro-temporal regions. The results indicate that although common features do exist, Panayiotopoulos syndrome differs from symptomatic occipital lobe epilepsy and has a unique low epileptogenic threshold related to particular brain circuits.

  19. Treatment Outcome Of Seizures Associated With Intracranial Cavernous Angiomas

    Directory of Open Access Journals (Sweden)

    Nievera Conrad C

    1999-01-01

    Full Text Available Seizures are among the typical presentations of intracranial cavernous angiomas (ICA. Twenty-one patients (age range: 2 to 53 years treated for seizures associated with ICA between 1983 and 1997 were restrospectively studied to evaluate their outcome following medical or surgical intervention. The mean interval between seizure onset and initial presentation at our institution was 7.6 years. Seizures were simple partial in 3 patients, complex partial in 15 and secondarily generalized tonic-clonic in 13. The commonest site of the lesion was the temporal lobe (52%. Multiple angiomas were observed in 5 (24% patients. Seven (32% patients were medically-managed with antiepileptic therapy and 14 (68% underwent either lesionectomy with resection of the epileptogenic zone (9 patients or temporal lobectomy (5 patients. Mean follow-up time was 4 years (range: 3 months to 14 years. Of the medically-managed patients, 3 (43% remained seizure-free whereas 4 (57% continued to have seizures with an average frequency of one per day. Of the surgically-managed patients, 12 (86% became seizure-free and 2 (14% had no more than two seizures per year. Surgery appears to be extremely effective in the management of seizures associated with ICA and should receive a strong and early consideration in patients who fail medical therapy.

  20. Seizures induced by carbachol, morphine, and leucine-enkephalin: a comparison.

    Science.gov (United States)

    Snead, O C

    1983-04-01

    The electrical, behavioral, and pharmacological properties of seizures induced by morphine, leucine-enkephalin, and the muscarinic cholinergic agonist carbachol were examined and compared. Low-dose carbachol given intracerebroventricularly (ICV) produced seizures similar electrically to those produced by ICV morphine and leucine-enkephalin, although there was some difference in site of subcortical origin of onset. Carbachol and morphine were similar in that they had the same anticonvulsant profile, produced similar behavioral changes, caused generalized absence seizures in low doses and generalized convulsive seizures in high doses, and were capable of chemical kindling. However, opiate-induced seizures were not overcome by cholinergic antagonists, nor were carbachol seizures blocked by opiate antagonists. These data suggest that there may be a common noncholinergic, nonopiatergic system involved in mediating carbachol- and morphine-induced seizures but not enkephalin seizures.

  1. Oculocerebrocutaneous syndrome : report of three additional cases and aetiological considerations

    NARCIS (Netherlands)

    Moog, U; deDieSmulders, C; Systermans, JMJ; Cobben, JM

    1997-01-01

    The oculocerebrocutaneous (OCC) syndrome is characterized by the presence of orbital cysts and microphthalmia/anophthalmia, focal hypo- and aplastic skin defects, skin appendages and cerebral malformations. Most of the patients suffer from psychomotor retardation and seizures. To date, 23 patients

  2. Første danske patient med et genkendeligt genetisk KBG-syndrom

    DEFF Research Database (Denmark)

    Bayat, Allan; Møller, Lisbeth Birk; Hjortshøj, Tina Duelund

    2018-01-01

    KBG syndrome is a rare condition characterised by macrodontia of the upper central incisors, distinctive craniofacial findings, short stature, skeletal anomalies, and neurologic involvement including global developmental delay, seizures, and intellectual disability. This is a case report of a sev...

  3. Baraitser∓Winter syndrome: An additional Arab patient

    African Journals Online (AJOL)

    Maha M. Abou Henedy

    Since then, 19 more cases had been reported showing char- acteristic clinical features resembling that of Baraitser–Winter syndrome [2–13]. Consequently, the phenotypic spectrum had been broadened including; microcornea, microphthalmia, microcephaly, trigonocephaly, gyral malformation, seizures, hypotonia, cardiac ...

  4. Probability of detection of clinical seizures using heart rate changes.

    Science.gov (United States)

    Osorio, Ivan; Manly, B F J

    2015-08-01

    Heart rate-based seizure detection is a viable complement or alternative to ECoG/EEG. This study investigates the role of various biological factors on the probability of clinical seizure detection using heart rate. Regression models were applied to 266 clinical seizures recorded from 72 subjects to investigate if factors such as age, gender, years with epilepsy, etiology, seizure site origin, seizure class, and data collection centers, among others, shape the probability of EKG-based seizure detection. Clinical seizure detection probability based on heart rate changes, is significantly (pprobability of detecting clinical seizures (>0.8 in the majority of subjects) using heart rate is highest for complex partial seizures, increases with a patient's years with epilepsy, is lower for females than for males and is unrelated to the side of hemisphere origin. Clinical seizure detection probability using heart rate is multi-factorially dependent and sufficiently high (>0.8) in most cases to be clinically useful. Knowledge of the role that these factors play in shaping said probability will enhance its applicability and usefulness. Heart rate is a reliable and practical signal for extra-cerebral detection of clinical seizures originating from or spreading to central autonomic network structures. Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  5. Nodding syndrome in Mundri county, South Sudan: Environmental ...

    African Journals Online (AJOL)

    Background: Nodding Syndrome is a seizure disorder of children in Mundri County, Western Equatoria, South Sudan. The disorder is reported to be spreading in South Sudan and northern Uganda. Objective: To describe environmental, nutritional, infectious, and other factors that existed before and during the de novo 1991 ...

  6. CDKL5 mutations cause infantile spasms, early onset seizures, and severe mental retardation in female patients

    Science.gov (United States)

    Archer, H L; Evans, J; Edwards, S; Colley, J; Newbury‐Ecob, R; O'Callaghan, F; Huyton, M; O'Regan, M; Tolmie, J; Sampson, J; Clarke, A; Osborne, J

    2006-01-01

    Objective To determine the frequency of mutations in CDKL5 in both male and female patients with infantile spasms or early onset epilepsy of unknown cause, and to consider whether the breadth of the reported phenotype would be extended by studying a different patient group. Methods Two groups of patients were investigated for CDKL5 mutations. Group 1 comprised 73 patients (57 female, 16 male) referred to Cardiff for CDKL5 analysis, of whom 49 (42 female, 7 male) had epileptic seizure onset in the first six months of life. Group 2 comprised 26 patients (11 female, 15 male) with infantile spasms previously recruited to a clinical trial, the UK Infantile Spasms Study. Where a likely pathogenic mutation was identified, further clinical data were reviewed. Results Seven likely pathogenic mutations were found among female patients from group 1 with epileptic seizure onset in the first six months of life, accounting for seven of the 42 in this group (17%). No mutations other than the already published mutation were found in female patients from group 2, or in any male patient from either study group. All patients with mutations had early signs of developmental delay and most had made little developmental progress. Further clinical information was available for six patients: autistic features and tactile hypersensitivity were common but only one had suggestive Rett‐like features. All had a severe epileptic seizure disorder, all but one of whom had myoclonic jerks. The EEG showed focal or generalised changes and in those with infantile spasms, hypsarrhythmia. Slow frequencies were seen frequently with a frontal or fronto‐temporal predominance and high amplitudes. Conclusions The spectrum of the epileptic seizure disorder, and associated EEG changes, in those with CDKL5 mutations is broader than previously reported. CDKL5 mutations are a significant cause of infantile spasms and early epileptic seizures in female patients, and of a later intractable seizure disorder

  7. Functional characterization of the 1,5-benzodiazepine clobazam and its major active metabolite N-desmethylclobazam at human GABA(A receptors expressed in Xenopus laevis oocytes.

    Directory of Open Access Journals (Sweden)

    Harriet Hammer

    Full Text Available The 1,5-benzodiazepine clobazam is indicated for the adjunctive treatment of seizures associated with Lennox-Gastaut syndrome in patients 2 years of age or older in the United States, and for treatment of anxiety and various forms of epilepsy elsewhere. Clobazam has been reported to exhibit different in vivo adverse effects and addiction liability profile than the classic 1,4-benzodiazepines. In this study, it was investigated whether the in vitro pharmacological properties of clobazam and its major active metabolite N-desmethylclobazam could explain some of these clinical differences. The functional properties of the two 1,5-benzodiazepines were characterized at the human γ-aminobutyric acid type A receptor (GABA(AR subtypes α1β2γ(2S, α2β2γ(2S, α3β2γ(2S, α5β2γ(2S and α6β2δ expressed in Xenopus laevis oocytes by use of two-electrode voltage-clamp electrophysiology and compared to those exhibited by the 1,4-benzodiazepine clonazepam. All three compounds potentiated GABA EC20-evoked responses through the α(1,2,3,5β2γ(2S GABA(ARs in a reversible and concentration-dependent manner, with each displaying similar EC50 values at the four subtypes. Furthermore, the degrees of potentiation of the GABA EC20 currents through the four receptors mediated by saturating modulator concentrations did not differ substantially for any of the three benzodiazepines. The three compounds were substantially less potent (200-3900 fold as positive allosteric modulators at the α6β2δ GABA(AR than at the α(1,2,3,5β2γ(2S receptors. Interestingly, however, clobazam and especially N-desmethylclobazam were highly efficacious potentiators of α6β2δ receptor signaling. Although this activity component is unlikely to contribute to the in vivo effects of clobazam/N-desmethylclobazam, the 1,5-benzodiazepine could constitute an interesting lead for novel modulators targeting this low-affinity binding site in GABAARs. In conclusion, the non

  8. A systematic review of the incidence and prevalence of sleep disorders and seizure disorders in multiple sclerosis

    DEFF Research Database (Denmark)

    Marrie, Ruth Ann; Reider, Nadia; Cohen, Jeffrey

    2015-01-01

    was 3.09% (95% CI: 2.01-4.16%). For sleep disorders we evaluated 18 studies; none were population-based. The prevalence ranged from 0-1.6% for narcolepsy, 14.4-57.5% for restless legs syndrome, 2.22-3.2% for REM behavior disorder, and 7.14-58.1% for obstructive sleep apnea. CONCLUSION: This review......BACKGROUND: Several studies have suggested that comorbid neurologic disorders are more common than expected in multiple sclerosis (MS). OBJECTIVE: To estimate the incidence and prevalence of comorbid seizure disorders and sleep disorders in persons with MS and to evaluate the quality of studies...... suggests that seizure disorders and sleep disorders are common in MS, but highlights gaps in the epidemiological knowledge of these conditions in MS worldwide. Other than central-western Europe and North America, most regions are understudied....

  9. Unusual presentation of Sturge-Weber syndrome: Progressive megalencephaly with bilateral cutaneous and cortical involvement

    Directory of Open Access Journals (Sweden)

    Kundan Mittal

    2014-01-01

    Full Text Available The Sturge Weber syndrome is characterized by developmental delay, seizures in infancy, unilateral cutaneous lesions with ipsilateral leptomeningeal enhancement. We report an unusual presentation of Sturge Weber syndrome with bilateral port wine nevus on the trunk and face along with bilateral cortical involvement in a developmentally normal child with progressive megalencephaly.

  10. Complex partial seizure, disruptive behaviours and the Nigerian ...

    African Journals Online (AJOL)

    Background: Complex partial seizure is an epileptic seizure which results in impairment of responsiveness or awareness such as altered level of consciousness. Complex partial seizures are often preceded by an aura such as depersonalization, feelings of de javu, jamais vu and fear. The ictal phase of complex partial ...

  11. 26 CFR 301.7321-1 - Seizure of property.

    Science.gov (United States)

    2010-04-01

    ... 26 Internal Revenue 18 2010-04-01 2010-04-01 false Seizure of property. 301.7321-1 Section 301... ADMINISTRATION PROCEDURE AND ADMINISTRATION Other Offenses § 301.7321-1 Seizure of property. Any property subject... director or assistant regional commissioner (alcohol, tobacco, and firearms). Upon seizure of property by...

  12. 8 CFR 274.1 - Seizure and forfeiture authority.

    Science.gov (United States)

    2010-01-01

    ... 8 Aliens and Nationality 1 2010-01-01 2010-01-01 false Seizure and forfeiture authority. 274.1 Section 274.1 Aliens and Nationality DEPARTMENT OF HOMELAND SECURITY IMMIGRATION REGULATIONS SEIZURE AND FORFEITURE OF CONVEYANCES § 274.1 Seizure and forfeiture authority. Any officer of Customs and Border...

  13. Indomethacin treatment prior to pentylenetetrazole-induced seizures downregulates the expression of il1b and cox2 and decreases seizure-like behavior in zebrafish larvae.

    Science.gov (United States)

    Barbalho, Patrícia Gonçalves; Lopes-Cendes, Iscia; Maurer-Morelli, Claudia Vianna

    2016-03-09

    It has been demonstrated that the zebrafish model of pentylenetetrazole (PTZ)-evoked seizures and the well-established rodent models of epilepsy are similar pertaining to behavior, electrographic features, and c-fos expression. Although this zebrafish model is suitable for studying seizures, to date, inflammatory response after seizures has not been investigated using this model. Because a relationship between epilepsy and inflammation has been established, in the present study we investigated the transcript levels of the proinflammatory cytokines interleukin-1 beta (il1b) and cyclooxygenase-2 (cox2a and cox2b) after PTZ-induced seizures in the brain of zebrafish 7 days post fertilization. Furthermore, we exposed the fish to the nonsteroidal anti-inflammatory drug indomethacin prior to PTZ, and we measured its effect on seizure latency, number of seizure behaviors, and mRNA expression of il1b, cox2b, and c-fos. We used quantitative real-time PCR to assess the mRNA expression of il1b, cox2a, cox2b, and c-fos, and visual inspection was used to monitor seizure latency and the number of seizure-like behaviors. We found a short-term upregulation of il1b, and we revealed that cox2b, but not cox2a, was induced after seizures. Indomethacin treatment prior to PTZ-induced seizures downregulated the mRNA expression of il1b, cox2b, and c-fos. Moreover, we observed that in larvae exposed to indomethacin, seizure latency increased and the number of seizure-like behaviors decreased. This is the first study showing that il1b and cox-2 transcripts are upregulated following PTZ-induced seizures in zebrafish. In addition, we demonstrated the anticonvulsant effect of indomethacin based on (1) the inhibition of PTZ-induced c-fos transcription, (2) increase in seizure latency, and (3) decrease in the number of seizure-like behaviors. Furthermore, anti-inflammatory effect of indomethacin is clearly demonstrated by the downregulation of the mRNA expression of il1b and cox2b. Our results

  14. Widespread EEG changes precede focal seizures.

    Directory of Open Access Journals (Sweden)

    Piero Perucca

    Full Text Available The process by which the brain transitions into an epileptic seizure is unknown. In this study, we investigated whether the transition to seizure is associated with changes in brain dynamics detectable in the wideband EEG, and whether differences exist across underlying pathologies. Depth electrode ictal EEG recordings from 40 consecutive patients with pharmacoresistant lesional focal epilepsy were low-pass filtered at 500 Hz and sampled at 2,000 Hz. Predefined EEG sections were selected immediately before (immediate preictal, and 30 seconds before the earliest EEG sign suggestive of seizure activity (baseline. Spectral analysis, visual inspection and discrete wavelet transform were used to detect standard (delta, theta, alpha, beta and gamma and high-frequency bands (ripples and fast ripples. At the group level, each EEG frequency band activity increased significantly from baseline to the immediate preictal section, mostly in a progressive manner and independently of any modification in the state of vigilance. Preictal increases in each frequency band activity were widespread, being observed in the seizure-onset zone and lesional tissue, as well as in remote regions. These changes occurred in all the investigated pathologies (mesial temporal atrophy/sclerosis, local/regional cortical atrophy, and malformations of cortical development, but were more pronounced in mesial temporal atrophy/sclerosis. Our findings indicate that a brain state change with distinctive features, in the form of unidirectional changes across the entire EEG bandwidth, occurs immediately prior to seizure onset. We postulate that these changes might reflect a facilitating state of the brain which enables a susceptible region to generate seizures.

  15. Individualized Low-Amplitude Seizure Therapy: Minimizing Current for Electroconvulsive Therapy and Magnetic Seizure Therapy

    Science.gov (United States)

    Peterchev, Angel V; Krystal, Andrew D; Rosa, Moacyr A; Lisanby, Sarah H

    2015-01-01

    Electroconvulsive therapy (ECT) at conventional current amplitudes (800–900 mA) is highly effective but carries the risk of cognitive side effects. Lowering and individualizing the current amplitude may reduce side effects by virtue of a less intense and more focal electric field exposure in the brain, but this aspect of ECT dosing is largely unexplored. Magnetic seizure therapy (MST) induces a weaker and more focal electric field than ECT; however, the pulse amplitude is not individualized and the minimum amplitude required to induce a seizure is unknown. We titrated the amplitude of long stimulus trains (500 pulses) as a means of determining the minimum current amplitude required to induce a seizure with ECT (bilateral, right unilateral, bifrontal, and frontomedial electrode placements) and MST (round coil on vertex) in nonhuman primates. Furthermore, we investigated a novel method of predicting this amplitude-titrated seizure threshold (ST) by a non-convulsive measurement of motor threshold (MT) using single pulses delivered through the ECT electrodes or MST coil. Average STs were substantially lower than conventional pulse amplitudes (112–174 mA for ECT and 37.4% of maximum device amplitude for MST). ST was more variable in ECT than in MST. MT explained 63% of the ST variance and is hence the strongest known predictor of ST. These results indicate that seizures can be induced with less intense electric fields than conventional ECT that may be safer; efficacy and side effects should be evaluated in clinical studies. MT measurement could be a faster and safer alternative to empirical ST titration for ECT and MST. PMID:25920013

  16. Orgasm Induced Seizures: A Rare Phenomenon

    African Journals Online (AJOL)

    testing of the brain revealed no structural abnormality. His blood examination findings were ... A variety of stimuli can cause reflex seizures, Some triggers include light, music and cognitive phenomenon. There are case reports ... seizures cause great personal distress and significantly affect marital relationships. Though ...

  17. Picrotoxin-induced seizures modified by morphine and opiate antagonists.

    Science.gov (United States)

    Thomas, J; Nores, W L; Kenigs, V; Olson, G A; Olson, R D

    1993-07-01

    The effects of naloxone, Tyr-MIF-1, and MIF-1 on morphine-mediated changes in susceptibility to picrotoxin-induced seizures were studied. Rats were pretreated with naloxone, MIF-1, Tyr-MIF-1, or saline. At 15-min intervals, they received a second pretreatment of morphine or saline and then were tested for seizures following a convulsant dose of picrotoxin. Several parameters of specific categories of seizures were scored. Morphine increased the number of focal seizure episodes, duration of postseizure akinesis, and incidence of generalized clonic seizures. Naloxone tended to block the morphine-mediated changes in susceptibility. Tyr-MIF-1 had effects similar to naloxone on duration of postseizure immobility but tended to potentiate the effects of morphine on focal seizure episodes. The effects of morphine and the opiate antagonists on focal seizure episodes and postseizure duration suggest the general involvement of several types of opiate receptors in these picrotoxin-induced behaviors. However, the observation of antagonistic effects for Tyr-MIF-1 on immobility but agonistic effects for focal seizures suggests that the type of effect exerted by opiate agents may depend upon other neuronal variables.

  18. The epileptology of Koolen-de Vries syndrome: Electro-clinico-radiologic findings in 31 patients.

    Science.gov (United States)

    Myers, Kenneth A; Mandelstam, Simone A; Ramantani, Georgia; Rushing, Elisabeth J; de Vries, Bert B; Koolen, David A; Scheffer, Ingrid E

    2017-06-01

    This study was designed to describe the spectrum of epilepsy phenotypes in Koolen-de Vries syndrome (KdVS), a genetic syndrome involving dysmorphic features, intellectual disability, hypotonia, and congenital malformations, that occurs secondary to 17q21.31 microdeletions and heterozygous mutations in KANSL1. We were invited to attend a large gathering of individuals with KdVS and their families. While there, we recruited individuals with KdVS and seizures, and performed thorough phenotyping. Additional subjects were included who approached us after the family support group brought attention to our research via social media. Inclusion criteria were genetic testing results demonstrating 17q21.31 deletion or KANSL1 mutation, and at least one seizure. Thirty-one individuals were studied, aged 2-35 years. Median age at seizure onset was 3.5 years, and 9 of 22 had refractory seizures 2 years after onset. Focal impaired awareness seizures were the most frequent seizure type occurring in 20 of 31, usually with prominent autonomic features. Twenty-one patients had prolonged seizures and, at times, refractory status epilepticus. Electroencephalography (EEG) showed focal/multifocal epileptiform discharges in 20 of 26. MRI studies of 13 patients were reviewed, and all had structural anomalies. Corpus callosum dysgenesis, abnormal hippocampi, and dilated ventricles were the most common, although periventricular nodular heterotopia, focal cortical dysplasia, abnormal sulcation, and brainstem and cerebellum abnormalities were also observed. One patient underwent epilepsy surgery for a lesion that proved to be an angiocentric glioma. The typical epilepsy phenotype of KdVS involves childhood-onset focal seizures that are prolonged and have prominent autonomic features. Multifocal epileptiform discharges are the typical EEG pattern. Structural brain abnormalities may be universal, including signs of abnormal neuroblast migration and abnormal axonal guidance. Epilepsy surgery should

  19. Reversible Posterior Leukoencephalopathy Syndrome Induced by Pazopanib

    International Nuclear Information System (INIS)

    Chelis, Leonidas; Kakolyris, Stylianos; Souftas, Vasilios; Amarantidis, Kiriakos; Xenidis, Nikolaos; Chamalidou, Eleni; Dimopoulos, Prokopios; Michailidis, Prodromos; Christakidis, Evagelos; Prassopoulos, Panagiotis

    2012-01-01

    The reversible posterior leukoencephalopathy syndrome is a clinical/radiological syndrome characterized by headache, seizures, impaired vision, acute hypertension, and typical magnetic resonance imaging findings. There are several reports in the literature that depict its occurrence in cancer patients. The list of common anticancer and supportive care drugs that predispose to reversible posterior leukoencephalopathy syndrome is expanding and includes not only a large number of chemotherapeutic agents but also an increased number of new targeted drugs, particularly angiogenesis inhibitors such as bevacizumab,sorefenib and sunitinib. Pazopanib is an oral tyrosine kinase inhibitor targeting vascular endothelial growth factor receptor, platelet-derived growth factor receptor, and c-Kit which after a positive phase III randomized clinical trial in patients with advanced renal cell cancer received FDA approval for the treatment of advanced renal cell carcinoma. Until now no cases of reversible posterior leukoencephalopathy syndrome induced by pazopanib have been reported. We present the case of a 40 years old female patient with heavily pre-treated metastatic renal cell carcinoma who received pazopanib as salvage treatment. After 21 days of pazopanib therapy the patient referred to the emergency department with epileptic seizure, impaired vision at both eyes and headache. MRI of the brain revealed subcortical oedema at the occipital and parietal lobes bilaterally. She was treated with anticonvulsants, i.v. administration of mannitol and antihypertensives and she recovered completely from her symptoms and was discharged on the tenth hospital day. A brain MRI performed 3 weeks after showed that the subcortical oedema had been subsided. In conclusion this is the first case of pazopanib induced reversible posterior leukoencephalopathy syndrome. Although usually reversible, this syndrome is a serious and potentially life threatening adverse effect, if untreated, that should

  20. Reversible Posterior Leukoencephalopathy Syndrome Induced by Pazopanib

    Directory of Open Access Journals (Sweden)

    Chelis Leonidas

    2012-10-01

    Full Text Available Abstract Background The reversible posterior leukoencephalopathy syndrome is a clinical/radiological syndrome characterized by headache, seizures, impaired vision, acute hypertension, and typical magnetic resonance imaging findings. There are several reports in the literature that depict its occurrence in cancer patients. The list of common anticancer and supportive care drugs that predispose to reversible posterior leukoencephalopathy syndrome is expanding and includes not only a large number of chemotherapeutic agents but also an increased number of new targeted drugs, particularly angiogenesis inhibitors such as bevacizumab,sorefenib and sunitinib. Pazopanib is an oral tyrosine kinase inhibitor targeting vascular endothelial growth factor receptor, platelet-derived growth factor receptor, and c-Kit which after a positive phase III randomized clinical trial in patients with advanced renal cell cancer received FDA approval for the treatment of advanced renal cell carcinoma. Until now no cases of reversible posterior leukoencephalopathy syndrome induced by pazopanib have been reported. Case report We present the case of a 40 years old female patient with heavily pre-treated metastatic renal cell carcinoma who received pazopanib as salvage treatment. After 21 days of pazopanib therapy the patient referred to the emergency department with epileptic seizure, impaired vision at both eyes and headache. MRI of the brain revealed subcortical oedema at the occipital and parietal lobes bilaterally. She was treated with anticonvulsants, i.v. administration of mannitol and antihypertensives and she recovered completely from her symptoms and was discharged on the tenth hospital day. A brain MRI performed 3 weeks after showed that the subcortical oedema had been subsided. Conclusion In conclusion this is the first case of pazopanib induced reversible posterior leukoencephalopathy syndrome. Although usually reversible, this syndrome is a serious and

  1. Management of epilepsy in patients with Rett syndrome: perspectives and considerations.

    Science.gov (United States)

    Krajnc, Natalija

    2015-01-01

    Rett syndrome (RTT) is a common neurodevelopmental disorder that appears in infancy with regression of acquired motor skills, loss of purposeful activity, hand stereotypies, loss of acquired spoken language, and seizures. Epilepsy affects the majority of patients in a specific clinical stage of the disease and is drug resistant in approximately one-third of cases. The association of epilepsy and even drug-resistant epilepsy has been reported in certain genotypes of the methyl-CpG-binding protein 2 mutation, which is present in a majority of patients with classical RTT. The evolution of electroencephalographic abnormalities accompanying the clinical development of the syndrome is well described, but much less is known about the seizure semiology and the effectiveness of specific antiepileptic drugs. The aim of this review is to present the clinical and electrophysiological aspects of epilepsy in RTT and the current treatment approach.

  2. Electric Stimulation of Ear Reduces the Effect of Toll-Like Receptor 4 Signaling Pathway on Kainic Acid-Induced Epileptic Seizures in Rats

    Directory of Open Access Journals (Sweden)

    En-Tzu Liao

    2018-01-01

    Full Text Available Epilepsy is a common clinical syndrome with recurrent neuronal discharges in the temporal lobe, cerebral cortex, and hippocampus. Clinical antiepileptic medicines are often ineffective or of little benefit in 30% of epileptic patients and usually cause severe side effects. Emerging evidence indicates the crucial role of inflammatory mediators in epilepsy. The current study investigates the role of toll-like receptor 4 (TLR4 and its underlying mechanisms in kainic acid- (KA- induced epileptic seizures in rats. Experimental KA injection successfully initiated an epileptic seizure accompanied by increased expression of TLR4 in the prefrontal cortex, hippocampus, and somatosensory cortex. In addition, calcium-sensitive phosphorylated Ca2+/calmodulin-dependent protein kinase II (pCaMKIIα increased after the initiation of the epileptic seizure. Furthermore, downstream-phosphorylated signal-regulated kinase (ERK, c-Jun NH2-terminal protein kinase (JNK, and p38 kinase simultaneously increased in these brain areas. Moreover, the transcriptional factor phosphorylated nuclear factor-κB (pNF-κB increased, suggesting that nucleus transcription was affected. Furthermore, the aforementioned molecules decreased by an electric stimulation (ES of either 2 Hz or 15 Hz of the ear in the three brain areas. Accordingly, we suggest that ES of the ear can successfully control epileptic seizures by regulating the TLR4 signaling pathway and has a therapeutic benefit in reducing epileptic seizures.

  3. Sex and Hormonal influences on Seizures and Epilepsy

    Science.gov (United States)

    Velíšková, Jana; DeSantis, Kara A.

    2012-01-01

    Epilepsy is the third most common chronic neurological disorder. Clinical and experimental evidence supports the role of sex and influence of sex hormones on seizures and epilepsy as well as alterations of the endocrine system and levels of sex hormones by epileptiform activity. Conversely, seizures are sensitive to changes in sex hormone levels, which in turn may affect the seizure-induced neuronal damage. The effects of reproductive hormones on neuronal excitability and seizure-induced damage are complex to contradictory and depend on different mechanisms, which have to be accounted for in data interpretation. Both estradiol and progesterone/allopregnanolone may have beneficial effects for patients with epilepsy. Individualized hormonal therapy should be considered as adjunctive treatment in patients with epilepsy to improve seizure control as well as quality of life. PMID:22504305

  4. A case of idiopathic intracranial calcifications - Hahr syndrome

    International Nuclear Information System (INIS)

    Georgiev, R.; Balev, B.; Georgieva, M.; Georgiev, J.

    2006-01-01

    Full text: The purpose of the study is to review the clinical manifestation, imaging characteristics and pathophysiology of the Fahr syndrome and to present a case of the Fahr syndrome from our clinic. The Fahr syndrome is a rare neurodegenerative disorder, characterized by seizures, tetany, psychomotor retardation, development of a spastic paralysis, athetosis and parkinson like syndrome. It is inherited by an AR way but in affected families (relatives) an AD way is also possible. Sporadic cases have been known. Recently a possible chromosome locus on 14q was proved. Probably the case in point is a group of anomalies, in which symmetrically and bilaterally significant calcifications in the region of the basal ganglia, dentate nuclei in cerebellum and centrum semiovale are found. It is not clear yet whether these calcifications are a result from a 'metastatic' deposition because of a local destruction of the blood-brain barrier or are due to a disturbance in the neuronic calcium metabolism. The X-ray findings could be accidental in an asymptomatic patient but a progressive development of an extrapyramidal syndrome may be also observed. Our case is a 37 years old woman with seizures with loss of consciousness, convulsions and urine incontinence. The complaints are dated from the age of 5 years old. The X-ray images disclosed striking non-natural calcifications in globus pallidus, putamen, n.caudatus, thalami, n.dentati, cerebellum.The blood test revealed normal serum levels of calcium, phosphorus, alkaline phosphatase. The CT findings put together with the typical clinical history and the normal blood test were a prerequisite for this diagnosis

  5. Treatment Of Seizures In The Elderly

    Directory of Open Access Journals (Sweden)

    Aleem MA

    2005-01-01

    Full Text Available The increasing life expectancy over the preceding decades and trend towards further increase means that the elderly is now a growing section of the population. Seizures are a particularly common disorder in the age group. Considering that above the age of 50 years, one is prone to suffer from atleast one chromic illness, the interplay between associated medical and neurologic diseases and seizures need to be understood. These comorbidities like hypertension, cerebrovascular accidents, diabetes, renal failure and others not only contribute to seizures, they may also interfere with their appropriate treatment. Seizures, on the other hand, may be the cause of added morbidity like fractures, head injury and poor self esteem which may lead to poor quality of life. In addition, the unique pharmacokinetics, pharmacodynamics and side effect profile of the various antieplileptic drugs in the elderly and the multiple drug interactions, require judicious use along with regular monitoring. However, an ideal antiepileptic drug for the elderly is yet to be found.

  6. Counselling adults who experience a first seizure.

    Science.gov (United States)

    Legg, Karen T; Newton, Mark

    2017-07-01

    A first seizure can result in significant uncertainty, fear and apprehension. One of the key roles of the clinician in the setting of first seizure is to provide accurate, timely information and counselling. We review the numerous components to be considered when counselling an adult patient after a first seizure. We provide a framework and manner to provide that counselling. We focus on an individualized approach and provide recommendations and information on issues of diagnosis, etiology, prognosis, the role and importance of medical testing, lifestyle considerations, driving, medication and other key counselling considerations. Accurate, timely counselling can allay fears and anxieties, remove misconceptions and reduce the risk for injury in seizure recurrence. Copyright © 2016 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  7. Evaluation of the pentylenetetrazole seizure threshold test in epileptic mice as surrogate model for drug testing against pharmacoresistant seizures.

    Science.gov (United States)

    Töllner, Kathrin; Twele, Friederike; Löscher, Wolfgang

    2016-04-01

    Resistance to antiepileptic drugs (AEDs) is a major problem in epilepsy therapy, so that development of more effective AEDs is an unmet clinical need. Several rat and mouse models of epilepsy with spontaneous difficult-to-treat seizures exist, but because testing of antiseizure drug efficacy is extremely laborious in such models, they are only rarely used in the development of novel AEDs. Recently, the use of acute seizure tests in epileptic rats or mice has been proposed as a novel strategy for evaluating novel AEDs for increased antiseizure efficacy. In the present study, we compared the effects of five AEDs (valproate, phenobarbital, diazepam, lamotrigine, levetiracetam) on the pentylenetetrazole (PTZ) seizure threshold in mice that were made epileptic by pilocarpine. Experiments were started 6 weeks after a pilocarpine-induced status epilepticus. At this time, control seizure threshold was significantly lower in epileptic than in nonepileptic animals. Unexpectedly, only one AED (valproate) was less effective to increase seizure threshold in epileptic vs. nonepileptic mice, and this difference was restricted to doses of 200 and 300 mg/kg, whereas the difference disappeared at 400mg/kg. All other AEDs exerted similar seizure threshold increases in epileptic and nonepileptic mice. Thus, induction of acute seizures with PTZ in mice pretreated with pilocarpine does not provide an effective and valuable surrogate method to screen drugs for antiseizure efficacy in a model of difficult-to-treat chronic epilepsy as previously suggested from experiments with this approach in rats. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Jervell and Lange-Nielson Syndrome masquerading as intractable epilepsy

    Directory of Open Access Journals (Sweden)

    Jagdish P Goyal

    2012-01-01

    Full Text Available The long QT syndrome (LQTS is a cause of syncope and sudden death. Jervell and Lange-Nielson syndrome (JLNS is an uncommon form of LQTS, having autosomal recessive transmission, and is associated with congenital deafness. We report a case of JLNS in a child who presented to us with refractory epilepsy. The cardiac cause of seizures was suspected as the child was hypotensive and pulseless during the episode of seizures. The child was diagnosed as JLNS based on Schwartz diagnostic criteria for LQTS and congenital sensorineural deafness. The child responded well to β-blocker therapy. Antiepileptic drugs were stopped. The screening of family members with ECG revealed a QT interval more than required for diagnosis of LQTS but they were asymptomatic. All asymptomatic family members were also put on metoprolol. All of them showed great improvement with the reduction of the QT interval on ECG. The patient was doing well on immediate follow-up.

  9. Hyperspherical Manifold for EEG Signals of Epileptic Seizures

    Directory of Open Access Journals (Sweden)

    Tahir Ahmad

    2012-01-01

    Full Text Available The mathematical modelling of EEG signals of epileptic seizures presents a challenge as seizure data is erratic, often with no visible trend. Limitations in existing models indicate a need for a generalized model that can be used to analyze seizures without the need for apriori information, whilst minimizing the loss of signal data due to smoothing. This paper utilizes measure theory to design a discrete probability measure that reformats EEG data without altering its geometric structure. An analysis of EEG data from three patients experiencing epileptic seizures is made using the developed measure, resulting in successful identification of increased potential difference in portions of the brain that correspond to physical symptoms demonstrated by the patients. A mapping then is devised to transport the measure data onto the surface of a high-dimensional manifold, enabling the analysis of seizures using directional statistics and manifold theory. The subset of seizure signals on the manifold is shown to be a topological space, verifying Ahmad's approach to use topological modelling.

  10. Behaviors induced or disrupted by complex partial seizures.

    Science.gov (United States)

    Leung, L S; Ma, J; McLachlan, R S

    2000-09-01

    We reviewed the neural mechanisms underlying some postictal behaviors that are induced or disrupted by temporal lobe seizures in humans and animals. It is proposed that the psychomotor behaviors and automatisms induced by temporal lobe seizures are mediated by the nucleus accumbens. A non-convulsive hippocampal afterdischarge in rats induced an increase in locomotor activity, which was suppressed by the injection of dopamine D(2) receptor antagonist in the nucleus accumbens, and blocked by inactivation of the medial septum. In contrast, a convulsive hippocampal or amygdala seizure induced behavioral hypoactivity, perhaps by the spread of the seizure into the frontal cortex and opiate-mediated postictal depression. Mechanisms underlying postictal psychosis, memory disruption and other long-term behavioral alterations after temporal lobe seizures, are discussed. In conclusion, many of the changes of postictal behaviors observed after temporal lobe seizures in humans may be found in animals, and the basis of the behavioral change may be explained as a change in neural processing in the temporal lobe and the connecting subcortical structures.

  11. Proton MR spectroscopy in patients with acute temporal lobe seizures.

    Science.gov (United States)

    Castillo, M; Smith, J K; Kwock, L

    2001-01-01

    Decreases in N-acetyl aspartate (NAA) as seen by proton MR spectroscopy are found in hippocampal sclerosis, and elevated levels of lipids/lactate have been observed after electroconvulsive therapy. Our purpose was to determine whether increased levels of lipids/lactate are found in patients with acute seizures of hippocampal origin. Seventeen patients with known temporal lobe epilepsy underwent proton MR spectroscopy of the mesial temporal lobes within 24 hours of their last seizure. Four of them were restudied when they were seizure-free. Five healthy individuals were used as control subjects. All MR spectroscopy studies were obtained using a single-voxel technique with TEs of 135 and 270. The relationship between the presence of lipids/lactate and seizures was tested using Fisher's exact test. Mean and standard deviations for NAA/creatine (Cr) were obtained in the hippocampi in patients with seizures on initial and follow-up studies and these values were compared with those in the control subjects. Seizure lateralization was obtained in 15 patients. Of the 17 seizure locations that involved hippocampi, 16 showed lipids/lactate by proton MR spectroscopy. Of the 13 hippocampi not directly affected by seizures, 10 showed no lipids/lactate and three showed lipids/lactate. The relationship between lipids/lactate and seizure location was confirmed. A comparison of NAA/Cr ratios for the involved hippocampi with those in control subjects showed significant differences on initial MR spectroscopy; however, no significant difference was found between acute and follow-up NAA/Cr ratios in hippocampi affected by seizures. Lipids/lactate were present in the hippocampi of patients with acute seizures and decreased when the patients were seizure-free. Thus, lipids/lactate may be a sensitive marker for acute temporal lobe seizures.

  12. Clinical spectrum of seizures and efficacy of anticonvulsive treatment in children

    International Nuclear Information System (INIS)

    Mahmud, S.; Zman, S.Q.

    2017-01-01

    Objective: To study the clinical spectrum of seizures and efficacy of anticonvulsive treatment in children. Study Design: A descriptive study. Place and Duration of Study: Military Hospital (MH) Rawalpindi from October 2011 to March 2012. Material and Methods: One hundred children of either gender aged 1 month to 12 years presenting with seizures at Military Hospital Rawalpindi were evaluated and consented to participate in the study. All children with a febrile seizures were evaluated. The seizures were classified according to international league against epilepsy guidelines. Antiepileptic treatment regimen was evaluated in terms of number of drugs, correct dosage and efficacy in control of seizures. Results: It was observed that generalized seizures were (58 percent) followed by focal seizures (32 percent) in children. Valproic acid was prescribed in (51 percent) cases. Epilepsy was diagnosed in (56 percent) followed by cerebral palsy (20 percent), post meningoencephalitis sequalae (11 percent), intracranial hemorrhage (7 percent) and leukodystrophies (3 percnet) as underlying cause of seizures. Statistically significant association was seen between age groups and diagnosis (p value=0.001); age groups and types of seizures (p value=0.046); correct dosage of antiepileptics and control of seizures (p value=0.007); compliance to treatment and control of seizures (p value=0.007). Conclusion: Generalized seizures are the commonest form followed by focal seizures. Epilepsy was the common etiology of seizures in all age groups in children. Cerebral palsy was the second leading cause of seizures in children followed by post meningoencephalitis, stroke and leukodystrophies. Valproic acid was the most commonly prescribed antiepileptic. Normal delivery with delayed cry was the major risk factor for cerebral palsy. Prescription of appropriate antiepileptics according to diagnosis in optimum dosage and compliance to treatment affect control of seizures in children. (author)

  13. Barriers to Seizure Management in Schools: Perceptions of School Nurses.

    Science.gov (United States)

    Terry, Debbie; Patel, Anup D; Cohen, Daniel M; Scherzer, Daniel; Kline, Jennifer

    2016-12-01

    The purpose of this study was to assess school nurses' perceptions of barriers to optimal management of seizures in schools. Eighty-three school nurses completed an electronic survey. Most agreed they felt confident they could identify a seizure (97.6%), give rectal diazepam (83.8%), and handle cluster seizures (67.1%), but fewer were confident they could give intranasal midazolam (63.3%), had specific information about a student's seizures (56.6%), or could swipe a vagus nerve stimulator magnet (47.4%). Nurses were more likely to be available at the time of a seizure in rural (17/20) (85%) versus suburban (21/34) (62%) or urban (8/25) (32%) schools (P = .001). School nurses are comfortable managing seizures in the school setting. However, a specific seizure plan for each child and education on intranasal midazolam and vagus nerve stimulator magnet use are needed. A barrier in urban schools is decreased availability of a nurse to identify seizures and administer treatment. © The Author(s) 2016.

  14. Paradoxical Seizure Response to Phenytoin in an Epileptic Heroin Addict.

    Science.gov (United States)

    Vasagar, Brintha; Verma, Beni R; Dewberry, Robert G; Pula, Thaddeus

    2015-06-01

    Phenytoin has a narrow therapeutic window and seizures can occur at both ends of the spectrum. A 41-year-old man with a history of a seizure disorder and heroin addiction presented with dizziness following 2 generalized tonic-clonic seizures that occurred earlier that day. The patient had received a loading dose of phenytoin for seizures associated with a subtherapeutic level 5 days previously. Initial evaluation revealed an elevated phenytoin level of 32.6 mcg/mL and an opiate-positive toxicology screen. Levetiracetam was started on the day of presentation and phenytoin was held until the level returned to the therapeutic range. The patient's dizziness resolved and he had no additional seizures. Evaluation for reversible causes of seizure activity along with anticonvulsant administration is generally the standard of care for breakthrough seizures. Phenytoin blood levels, if supratherapeutic, may be at least partially responsible for breakthrough seizure activity; in this circumstance, holding phenytoin and temporarily adding another anticonvulsant may be indicated.

  15. 28 CFR 0.86 - Seizure of gambling devices.

    Science.gov (United States)

    2010-07-01

    ... 28 Judicial Administration 1 2010-07-01 2010-07-01 false Seizure of gambling devices. 0.86 Section... Bureau of Investigation § 0.86 Seizure of gambling devices. The Director, Associate Director, Assistants... General to make seizures of gambling devices (18 U.S.C. 1955(d), 15 U.S.C. 1171 et seq.) and wire or oral...

  16. Febrile seizures prior to sudden cardiac death

    DEFF Research Database (Denmark)

    Stampe, Niels Kjær; Glinge, Charlotte; Jabbari, Reza

    2018-01-01

    Aims: Febrile seizure (FS) is a common disorder affecting 2-5% of children up to 5 years of age. The aim of this study was to determine whether FS in early childhood are over-represented in young adults dying from sudden cardiac death (SCD). Methods and results: We included all deaths (n = 4595...... with FS was sudden arrhythmic death syndrome (5/8; 62.5%). Conclusion: In conclusion, this study demonstrates a significantly two-fold increase in the frequency of FS prior to death in young SCD cases compared with the two control groups, suggesting that FS could potentially contribute in a risk......) nationwide and through review of all death certificates, we identified 245 SCD in Danes aged 1-30 years in 2000-09. Through the usage of nationwide registries, we identified all persons admitted with first FS among SCD cases (14/245; 5.7%) and in the corresponding living Danish population (71 027/2 369 785...

  17. Defining the phenotype associated with microduplication reciprocal to Sotos syndrome microdeletion

    DEFF Research Database (Denmark)

    Novara, Francesca; Stanzial, Franco; Rossi, Elena

    2014-01-01

    NSD1 point mutations, submicroscopic deletions and intragenic deletions are the major cause of Sotos syndrome, characterized by pre-postnatal generalized overgrowth with advanced bone age, learning disability, seizures, distinctive facial phenotype. Reverse clinical phenotype due to 5q35...

  18. Right-sided vagus nerve stimulation inhibits induced spinal cord seizures.

    Science.gov (United States)

    Tubbs, R Shane; Salter, E George; Killingsworth, Cheryl; Rollins, Dennis L; Smith, William M; Ideker, Raymond E; Wellons, John C; Blount, Jeffrey P; Oakes, W Jerry

    2007-01-01

    We have previously shown that left-sided vagus nerve stimulation results in cessation of induced spinal cord seizures. To test our hypothesis that right-sided vagus nerve stimulation will also abort seizure activity, we have initiated seizures in the spinal cord and then performed right-sided vagus nerve stimulation in an animal model. Four pigs were anesthetized and placed in the lateral position and a small laminectomy performed in the lumbar region. Topical penicillin, a known epileptogenic drug to the cerebral cortex and spinal cord, was next applied to the dorsal surface of the exposed cord. With the exception of the control animal, once seizure activity was discernible via motor convulsion or increased electrical activity, the right vagus nerve previously isolated in the neck was stimulated. Following multiple stimulations of the vagus nerve and with seizure activity confirmed, the cord was transected in the midthoracic region and vagus nerve stimulation performed. Right-sided vagus nerve stimulation resulted in cessation of spinal cord seizure activity in all animals. Transection of the spinal cord superior to the site of seizure induction resulted in the ineffectiveness of vagus nerve stimulation in causing cessation of seizure activity in all study animals. As with left-sided vagus nerve stimulation, right-sided vagus nerve stimulation results in cessation of induced spinal cord seizures. Additionally, the effects of right-sided vagus nerve stimulation on induced spinal cord seizures involve descending spinal pathways. These data may aid in the development of alternative mechanisms for electrical stimulation for patients with medically intractable seizures and add to our knowledge regarding the mechanism for seizure cessation following peripheral nerve stimulation.

  19. Evaluation of anticonvulsant actions of dibromophenyl enaminones using in vitro and in vivo seizure models.

    Directory of Open Access Journals (Sweden)

    Mohamed G Qaddoumi

    Full Text Available Epilepsy and other seizure disorders are not adequately managed with currently available drugs. We recently synthesized a series of dibromophenyl enaminones and demonstrated that AK6 and E249 were equipotent to previous analogs but more efficacious in suppressing neuronal excitation. Here we examined the actions of these lead compounds on in vitro and in vivo seizure models. In vitro seizures were induced in the hippocampal slice chemically (zero Mg2+ buffer and picrotoxin and electrically using patterned high frequency stimulation (HFS of afferents. In vivo seizures were induced in rats using the 6 Hz and the maximal electroshock models. AK6 (10 µM and E249 (10 µM depressed the amplitude of population spikes recorded in area CA1 of the hippocampus by -50.5±4.3% and -40.1±3.1% respectively, with partial recovery after washout. In the zero Mg2+ model, AK6 (10 µM depressed multiple population spiking (mPS by -59.3±6.9% and spontaneous bursts (SBs by -65.9±7.2% and in the picrotoxin-model by -43.3±7.2% and -50.0±8.3%, respectively. Likewise, E249 (10 µM depressed the zero-Mg2+-induced mPS by -48.8±9.5% and SBs by -55.8±15.5%, and in the picrotoxin model by -37.1±5.5% and -56.5±11.4%, respectively. They both suppressed post-HFS induced afterdischarges and SBs. AK6 and E249 dose-dependently protected rats in maximal electroshock and 6 Hz models of in vivo seizures after 30 min pretreatment. Their level of protection in both models was similar to that obtained with phenytoin Finally, while AK6 had no effect on locomotion in rats, phenytoin significantly decreased locomotion. AK6 and E249, suppressed in vitro and in vivo seizures to a similar extent. Their in vivo activities are comparable with but not superior to phenytoin. The most efficacious, AK6 produced no locomotor suppression while phenytoin did. Thus, AK6 and E249 may be excellent candidates for further investigation as potential agents for the treatment of epilepsy syndromes

  20. CURRENT APPROACHES TO THERAPY OF RETT’S SYNDROME (A REVIEW OF LITERATURE

    Directory of Open Access Journals (Sweden)

    N. Yu. Borovikova

    2015-01-01

    Full Text Available Antiepileptic therapy is one of the most urgent problems in the treatment of Rett’s syndrome. By taking into account a common concurrence of generalized and focal seizures with diffuse epileptiform activity on the electroencephalogram (EEG in Rett’s syndrome, there are effective broad-spectrum antiepileptic drugs (AEDs: valproates, topiramate, levetiracetam, lamotrigine. Carbamazepine is effective for focal seizures and in the absence of diffuse EEG changes. For atypical absences, ethosuximide may be added to valproates, topiramate, or levetiracetam. Reflex seizures show a high resistance; their frequency is occasionally reduced by AEDs in combination with neuroleptics. Sleep hygiene, as well as medication (clonidine, zolpidem, trazodone, melatonin, risperidone are recommended to correct various sleep disorders. Dopamine agonists, as well as L-carnitine are used for the drug correction of movement disorders in Rett’s syndrome. Therapeutic exercises are one of the most optimal ways to correct movement disorders. Orthopedic correction, including surgery, is indicated for skeletal deformities. Vitamin D used for long periods of time is beneficial, by considering its deficiency and osteoporosis at a fracture risk in Rett’s syndrome patients who receive AEDs particularly long. A special high-fat diet and a fractional diet in small portions are used in the therapy of cachexia and growth retardation due to oral dysfunction and malnutrition. A cardiological follow-up is needed in abnormalities, such as prolonged Q interval, tachyarrhythmia, and cardiac structural anomalies. Systematic learning to maintain communication and motor skills are of importance. In this case a special role is played by music therapy that exerts a calming effect on patients and partially compensates for loss of contact with the environment.

  1. Precipitating factors and therapeutic outcome in epilepsy with generalized tonic-clonic seizures.

    Science.gov (United States)

    Bauer, J; Saher, M S; Burr, W; Elger, C E

    2000-10-01

    The aim of the study was to evaluate the influence of precipitating factors and therapy on the outcome of epilepsy with generalized tonic-clonic seizures. Retrospective analysis of data from 34 patients (mean age at seizure onset 19 years; mean duration of follow-up 9.2 years) suffering from epilepsy of either cryptogenic or remote symptomatic (n = 19), or idiopathic (n = 15) etiology. The total number of seizures in all patients was 146. Without treatment 97 seizures manifested during 90.5 years without treatment (1.07 seizures/year), during treatment with carbamazepine or valproate 49 seizures occurred within 224 years (0.2 seizures/year). The frequency of seizures was significantly lower during treatment. Precipitating factors were found in relation to 31% of seizures in patients with remote symptomatic or cryptogenic epilepsy, and for 51% of seizures in patients with idiopathic epilepsy. There was a low frequency of seizures in patients with generalized tonic-clonic seizures. Precipitating factors are common. Antiepileptic drug treatment is effective.

  2. Endorphin mediation of post-ictal effects of kindled seizures in rats.

    Science.gov (United States)

    Kelsey, J E; Belluzzi, J D

    1982-12-16

    Brief electrical stimulation of the enkephalin-rich globus pallidus at 1-h intervals produced kindled, clonic seizures in rats as rapidly as similar stimulation of the amygdala. Massing the kindling trials at 10-min intervals inhibited the occurrence of subsequent seizures, especially following globus pallidus stimulation. Naloxone (20 mg/kg), an opiate receptor antagonist, reversed this post-ictal inhibition of seizures following massed trials, but had no effect on seizures kindled at 1-h intervals. Thus, endorphin-released during seizures do not appear to mediate the production of kindled seizures, but do appear to mediate the transient posts ictal inhibition of seizures.

  3. Seizure detection algorithms based on EMG signals

    DEFF Research Database (Denmark)

    Conradsen, Isa

    Background: the currently used non-invasive seizure detection methods are not reliable. Muscle fibers are directly connected to the nerves, whereby electric signals are generated during activity. Therefore, an alarm system on electromyography (EMG) signals is a theoretical possibility. Objective...... on the amplitude of the signal. The other algorithm was based on information of the signal in the frequency domain, and it focused on synchronisation of the electrical activity in a single muscle during the seizure. Results: The amplitude-based algorithm reliably detected seizures in 2 of the patients, while...... the frequency-based algorithm was efficient for detecting the seizures in the third patient. Conclusion: Our results suggest that EMG signals could be used to develop an automatic seizuredetection system. However, different patients might require different types of algorithms /approaches....

  4. Control of seizures by ketogenic diet-induced modulation of metabolic pathways.

    Science.gov (United States)

    Clanton, Ryan M; Wu, Guoyao; Akabani, Gamal; Aramayo, Rodolfo

    2017-01-01

    Epilepsy is too complex to be considered as a disease; it is more of a syndrome, characterized by seizures, which can be caused by a diverse array of afflictions. As such, drug interventions that target a single biological pathway will only help the specific individuals where that drug's mechanism of action is relevant to their disorder. Most likely, this will not alleviate all forms of epilepsy nor the potential biological pathways causing the seizures, such as glucose/amino acid transport, mitochondrial dysfunction, or neuronal myelination. Considering our current inability to test every individual effectively for the true causes of their epilepsy and the alarming number of misdiagnoses observed, we propose the use of the ketogenic diet (KD) as an effective and efficient preliminary/long-term treatment. The KD mimics fasting by altering substrate metabolism from carbohydrates to fatty acids and ketone bodies (KBs). Here, we underscore the need to understand the underlying cellular mechanisms governing the KD's modulation of various forms of epilepsy and how a diverse array of metabolites including soluble fibers, specific fatty acids, and functional amino acids (e.g., leucine, D-serine, glycine, arginine metabolites, and N-acetyl-cysteine) may potentially enhance the KD's ability to treat and reverse, not mask, these neurological disorders that lead to epilepsy.

  5. Spatiotemporal differences in the c-fos pathway between C57BL/6J and DBA/2J mice following flurothyl-induced seizures: A dissociation of hippocampal Fos from seizure activity.

    Science.gov (United States)

    Kadiyala, Sridhar B; Papandrea, Dominick; Tuz, Karina; Anderson, Tara M; Jayakumar, Sachidhanand; Herron, Bruce J; Ferland, Russell J

    2015-01-01

    Significant differences in seizure characteristics between inbred mouse strains highlight the importance of genetic predisposition to epilepsy. Here, we examined the genetic differences between the seizure-resistant C57BL/6J (B6) mouse strain and the seizure-susceptible DBA/2J (D2) strain in the phospho-Erk and Fos pathways to examine seizure-induced neuronal activity to uncover potential mechanistic correlates to these disparate seizure responsivities. Expression of neural activity markers was examined following 1, 5, or 8 seizures, or after 8 seizures, a 28 day rest period, and a final flurothyl rechallenge. Two brain regions, the hippocampus and ventromedial nucleus of the hypothalamus (VMH), had significantly different Fos expression profiles following seizures. Fos expression was highly robust in B6 hippocampus following one seizure and remained elevated following multiple seizures. Conversely, there was an absence of Fos (and phospho-Erk) expression in D2 hippocampus following one generalized seizure that increased with multiple seizures. This lack of Fos expression occurred despite intracranial electroencephalographic recordings indicating that the D2 hippocampus propagated ictal discharge during the first flurothyl seizure suggesting a dissociation of seizure discharge from Fos and phospho-Erk expression. Global transcriptional analysis confirmed a dysregulation of the c-fos pathway in D2 mice following 1 seizure. Moreover, global analysis of RNA expression differences between B6 and D2 hippocampus revealed a unique pattern of transcripts that were co-regulated with Fos in D2 hippocampus following 1 seizure. These expression differences could, in part, account for D2's seizure susceptibility phenotype. Following 8 seizures, a 28 day rest period, and a final flurothyl rechallenge, ∼85% of B6 mice develop a more complex seizure phenotype consisting of a clonic-forebrain seizure that uninterruptedly progresses into a brainstem seizure. This seizure phenotype

  6. Seizure outcome after AED failure in pediatric focal epilepsy: impact of underlying etiology.

    Science.gov (United States)

    Wirrell, Elaine C; Wong-Kisiel, Lily C; Nickels, Katherine C

    2014-05-01

    This study aimed to identify long-term seizure outcome in pediatric nonsyndromic focal epilepsy after failure of serial antiepileptic drugs (AEDs) due to lack of efficacy. Children (1 month-17 years) with new-onset focal epilepsy not meeting the criteria for a defined electroclinical syndrome diagnosed between 1980 and 2009 while residing in Olmsted County, MN, were retrospectively identified. Medical records of those followed for ≥2 years were reviewed to assess etiology, the number of AEDs that failed due to lack of efficacy, and seizure outcome at final follow-up. Etiology was classified into structural/metabolic, genetic, or unknown. Favorable outcome was defined as seizure freedom ≥1 year, on or off AEDs, without prior epilepsy surgery. Poor outcome was defined as ongoing seizures in the preceding year or having undergone prior epilepsy surgery. Nonsyndromic focal epilepsy accounted for 275/468 (59%) of all patients with newly diagnosed epilepsy--of these, 256 (93%) were followed for a minimum of two years and were included in the study. Median duration of follow-up was 10.0 years. At least one AED had failed due to lack of efficacy in 100 (39.1%) children. Favorable outcomes occurred in 149/156 (95.5%) children with no AED failure, 16/30 (53.3%) with one AED failure, 8/25 (32%) with two AED failures, and only 2/45 (4.4%) with three AED failures. After two AED failures, the seizures of nearly one-quarter of children who had epilepsy with an unknown cause responded favorably to the third AED compared with only 7.8% of the cohort that had epilepsy with a structural/metabolic cause. Children with a remote brain insult had a significantly higher likelihood of favorable outcome with serial AEDs than those with other structural abnormalities. Etiology is an important determinant of pharmacoresistance in nonsyndromic focal epilepsy. Surgical evaluation should be considered after failure of 1-2 AEDs in those who have epilepsy with structural causes, excluding

  7. De novo loss-of-function mutations in CHD2 cause a fever-sensitive myoclonic epileptic encephalopathy sharing features with Dravet syndrome

    DEFF Research Database (Denmark)

    Suls, Arvid; Jaehn, Johanna A; Kecskés, Angela

    2013-01-01

    Dravet syndrome is a severe epilepsy syndrome characterized by infantile onset of therapy-resistant, fever-sensitive seizures followed by cognitive decline. Mutations in SCN1A explain about 75% of cases with Dravet syndrome; 90% of these mutations arise de novo. We studied a cohort of nine Dravet...

  8. Seizure, Fit or Attack? The Use of Diagnostic Labels by Patients with Epileptic or Non-Epileptic Seizures

    Science.gov (United States)

    Plug, Leendert; Sharrack, Basil; Reuber, Markus

    2010-01-01

    We present an analysis of the use of diagnostic labels such as "seizure", "attack", "fit", and "blackout" by patients who experience seizures. While previous research on patients' preferences for diagnostic terminology has relied on questionnaires, we assess patients' own preferences and their responses to a doctor's use of different labels…

  9. 19 CFR 162.93 - Failure to issue notice of seizure.

    Science.gov (United States)

    2010-04-01

    ... 19 Customs Duties 2 2010-04-01 2010-04-01 false Failure to issue notice of seizure. 162.93 Section... OF THE TREASURY (CONTINUED) INSPECTION, SEARCH, AND SEIZURE Civil Asset Forfeiture Reform Act § 162.93 Failure to issue notice of seizure. If Customs does not send notice of a seizure of property in...

  10. EEG epileptiform abnormalities at admission to a rehabilitation department predict the risk of seizures in disorders of consciousness following a coma.

    Science.gov (United States)

    Bagnato, Sergio; Boccagni, Cristina; Sant'Angelo, Antonino; Prestandrea, Caterina; Virgilio, Vittorio; Galardi, Giuseppe

    2016-03-01

    Seizures affect about a quarter of patients with disorders of consciousness (DOC) after a coma. We investigated whether the presence of epileptiform abnormalities (EAs) in the electroencephalogram (EEG) of patients with DOC may predict the occurrence of seizures. Moreover, we evaluated whether EAs have a prognostic role in these patients. This was a retrospective single-center cohort study of patients hospitalized between January 2005 and December 2014 in a rehabilitation department (mean time from acute brain injury: 46.1 days). We analyzed 30-minute EEGs at admittance for 112 patients with unresponsive wakefulness syndrome (UWS) or in a minimally conscious state (MCS), then compared occurrence of seizures over the following three months across patients with absent, unilateral, and bilateral EAs (generalized or bilateral independent). Outcomes at three months were assessed in the same groups using the Coma Recovery Scale Revised. Epileptiform abnormalities were observed in 38 patients (33.9%). Of these, 25 were unilateral, and 13 were bilateral. Seizures occurred in 84.6% of patients with bilateral EAs, which was significantly higher than in patients without EAs (10.8%, p<0.001) or with unilateral EAs (24%, p=0.001). The presence of EAs was not related to etiology or different DOC and did not significantly affect outcomes at three months. Patients with EAs at admission to a rehabilitation department have an increased risk of seizures. Specifically, most patients with bilateral EAs had seizures within the following 3 months. Evaluation of EAs in EEGs of patients with DOC may give valuable information in the management of antiepileptic drug treatment. Copyright © 2015 Elsevier Inc. All rights reserved.

  11. Mechanisms of morphine enhancement of spontaneous seizure activity.

    Science.gov (United States)

    Saboory, Ehsan; Derchansky, Miron; Ismaili, Mohammed; Jahromi, Shokrollah S; Brull, Richard; Carlen, Peter L; El Beheiry, Hossam

    2007-12-01

    High-dose opioid therapy can precipitate seizures; however, the mechanism of such a dangerous adverse effect remains poorly understood. The aim of our study was to determine whether the neuroexcitatory activity of high-dose morphine is mediated by selective stimulation of opioid receptors. Mice hippocampi were resected intact and bathed in low magnesium artificial cerebrospinal fluid to induce spontaneous seizure-like events recorded from CA1 neurons. Application of morphine had a biphasic effect on the recorded spontaneous seizure-like events. In a low concentration (10 microM), morphine depressed electrographic seizure activity. Higher morphine concentrations (30 and 100 microM) enhanced seizure activity in an apparent dose-dependent manner. Naloxone, a nonselective opiate antagonist blocked the proconvulsant action of morphine. Selective mu and kappa opiate receptor agonists and antagonists enhanced and suppressed the spontaneous seizure activity, respectively. On the contrary, delta opioid receptor ligands did not have an effect. The proseizure effect of morphine is mediated through selective stimulation of mu and kappa opiate receptors but not the activation of the delta receptor system. The observed dose-dependent mechanism of morphine neuroexcitation underscores careful adjustment and individualized opioid dosing in the clinical setting.

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

    Science.gov (United States)

    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.

  13. Methadone-induced Torsades de Pointes Masquerading as Seizures

    Directory of Open Access Journals (Sweden)

    David C. Traficante

    2017-01-01

    Full Text Available The authors herein present the case of a 53-year-old female who was being treated as an outpatient for seizure disorder but was also receiving high-dose methadone therapy. She presented to the emergency department (ED for what appeared to be a seizure and was found to have a prolonged QT interval, as well as runs of paroxysmal polymorphic ventricular tachycardia with seizure-like activity occurring during the arrhythmia. The markedly prolonged QT interval corrected after treatment with intravenous magnesium; subsequent electroencephalogram, neurology and cardiology consultations confirmed the cause of the recurrent seizure-like episodes to be secondary to the cardiotoxic effects of methadone.

  14. Sex dimorphism in seizure-controlling networks.

    Science.gov (United States)

    Giorgi, Fillippo Sean; Galanopoulou, Aristea S; Moshé, Solomon L

    2014-12-01

    Males and females show a different predisposition to certain types of seizures in clinical studies. Animal studies have provided growing evidence for sexual dimorphism of certain brain regions, including those that control seizures. Seizures are modulated by networks involving subcortical structures, including thalamus, reticular formation nuclei, and structures belonging to the basal ganglia. In animal models, the substantia nigra pars reticulata (SNR) is the best studied of these areas, given its relevant role in the expression and control of seizures throughout development in the rat. Studies with bilateral infusions of the GABA(A) receptor agonist muscimol have identified distinct roles of the anterior or posterior rat SNR in flurothyl seizure control, that follow sex-specific maturational patterns during development. These studies indicate that (a) the regional functional compartmentalization of the SNR appears only after the third week of life, (b) only the male SNR exhibits muscimol-sensitive proconvulsant effects which, in older animals, is confined to the posterior SNR, and (c) the expression of the muscimol-sensitive anticonvulsant effects become apparent earlier in females than in males. The first three postnatal days are crucial in determining the expression of the muscimol-sensitive proconvulsant effects of the immature male SNR, depending on the gonadal hormone setting. Activation of the androgen receptors during this early period seems to be important for the formation of this proconvulsant SNR region. We describe molecular/anatomical candidates underlying these age- and sex-related differences, as derived from in vitro and in vivo experiments, as well as by [(14)C]2-deoxyglucose autoradiography. These involve sex-specific patterns in the developmental changes in the structure or physiology or GABA(A) receptors or of other subcortical structures (e.g., locus coeruleus, hippocampus) that may affect the function of seizure-controlling networks

  15. Modulation of autonomic activity in neurological conditions: Epilepsy and Tourette syndrome

    OpenAIRE

    Yoko eNagai

    2015-01-01

    This manuscript considers the central but neglected role of the autonomic nervous system in the expression and control of seizures in epilepsy (small) and tics in Tourette Syndrome (TS). In epilepsy, consideration of autonomic involvement is typically confined to differential diagnoses (e.g., syncope), or in relation to Sudden Unexpected Death in Epilepsy (SUDEP). Investigation is more limited in Tourette Syndrome. The role of the autonomic nervous system in the generation and prevention of e...

  16. 77 FR 11437 - Inspection Service Authority; Seizure and Forfeiture

    Science.gov (United States)

    2012-02-27

    ... POSTAL SERVICE 39 CFR Part 233 Inspection Service Authority; Seizure and Forfeiture AGENCY: Postal... Service's rules and regulations regarding the seizure and forfeiture of property into three sections, 39.... The proposed revision consolidates sections 233.8 and 233.9, and treats seizures involving personal...

  17. Seizure tests distinguish intermittent fasting from the ketogenic diet

    Science.gov (United States)

    Hartman, Adam L.; Zheng, Xiangrong; Bergbower, Emily; Kennedy, Michiko; Hardwick, J. Marie

    2010-01-01

    Summary Purpose Calorie restriction can be anticonvulsant in animal models. The ketogenic diet was designed to mimic calorie restriction and has been assumed to work by the same mechanisms. We challenged this assumption by profiling the effects of these dietary regimens in mice subjected to a battery of acute seizure tests. Methods Juvenile male NIH Swiss mice received ketogenic diet or a normal diet fed in restricted quantities (continuously or intermittently) for ~ 12 days, starting at 3–4 weeks of age. Seizures were induced by the 6 Hz test, kainic acid, maximal electroshock, or pentylenetetrazol. Results The ketogenic and calorie-restricted diets often had opposite effects depending on the seizure test. The ketogenic diet protected from 6 Hz–induced seizures, whereas calorie restriction (daily and intermittent) increased seizure activity. Conversely, calorie restriction protected juvenile mice against seizures induced by kainic acid, whereas the ketogenic diet failed to protect. Intermittent caloric restriction worsened seizures induced by maximal electroshock but had no effect on those induced by pentylenetetrazol. Discussion In contrast to a longstanding hypothesis, calorie restriction and the ketogenic diet differ in their acute seizure test profiles, suggesting that they have different underlying anticonvulsant mechanisms. These findings highlight the importance of the 6 Hz test and its ability to reflect the benefits of ketosis and fat consumption. PMID:20477852

  18. Seizure tests distinguish intermittent fasting from the ketogenic diet.

    Science.gov (United States)

    Hartman, Adam L; Zheng, Xiangrong; Bergbower, Emily; Kennedy, Michiko; Hardwick, J Marie

    2010-08-01

    Calorie restriction can be anticonvulsant in animal models. The ketogenic diet was designed to mimic calorie restriction and has been assumed to work by the same mechanisms. We challenged this assumption by profiling the effects of these dietary regimens in mice subjected to a battery of acute seizure tests. Juvenile male NIH Swiss mice received ketogenic diet or a normal diet fed in restricted quantities (continuously or intermittently) for ∼12 days, starting at 3-4 weeks of age. Seizures were induced by the 6 Hz test, kainic acid, maximal electroshock, or pentylenetetrazol. The ketogenic and calorie-restricted diets often had opposite effects depending on the seizure test. The ketogenic diet protected from 6 Hz-induced seizures, whereas calorie restriction (daily and intermittent) increased seizure activity. Conversely, calorie restriction protected juvenile mice against seizures induced by kainic acid, whereas the ketogenic diet failed to protect. Intermittent caloric restriction worsened seizures induced by maximal electroshock but had no effect on those induced by pentylenetetrazol. In contrast to a longstanding hypothesis, calorie restriction and the ketogenic diet differ in their acute seizure test profiles, suggesting that they have different underlying anticonvulsant mechanisms. These findings highlight the importance of the 6 Hz test and its ability to reflect the benefits of ketosis and fat consumption. Wiley Periodicals, Inc. © 2010 International League Against Epilepsy.

  19. KohlschutterTonz Syndrome : Mutations in ROGDI and Evidence of Genetic Heterogeneity

    NARCIS (Netherlands)

    Tucci, Arianna; Kara, Eleanna; Schossig, Anna; Wolf, Nicole I.; Plagnol, Vincent; Fawcett, Katherine; Paisan-Ruiz, Coro; Moore, Matthew; Hernandez, Dena; Musumeci, Sebastiano; Tennison, Michael; Hennekam, Raoul; Palmeri, Silvia; Malandrini, Alessandro; Raskin, Salmo; Donnai, Dian; Hennig, Corina; Tzschach, Andreas; Hordijk, Roel; Bast, Thomas; Wimmer, Katharina; Lo, Chien-Ning; Shorvon, Simon; Mefford, Heather; Eichler, Evan E.; Hall, Roger; Hayes, Ian; Hardy, John; Singleton, Andrew; Zschocke, Johannes; Houlden, Henry

    KohlschutterTonz syndrome (KTS) is a rare autosomal recessive disorder characterized by amelogenesis imperfecta, psychomotor delay or regression and seizures starting early in childhood. KTS was established as a distinct clinical entity after the first report by Kohlschutter in 1974, and to date,

  20. Management of Posterior Reversible Syndrome in Preeclamptic Women

    Directory of Open Access Journals (Sweden)

    S. Poma

    2014-01-01

    Full Text Available Posterior reversible encephalopathy syndrome (PRES is a neurological syndrome associated with a number of conditions including preeclampsia. It is characterized by seizures, alteration of consciousness, visual disturbances, and symmetric white matter abnormalities, typically in the posterior parietooccipital regions of the cerebral hemispheres, at computed tomography (CT and magnetic resonance (MRI. We report three new cases of PRES in preeclamptic patients and describe the management of these patients. We present a brief review of other cases in the literature, with particular attention to the anesthetic management.

  1. Temperature, age, and recurrence of febrile seizure

    NARCIS (Netherlands)

    M. van Stuijvenberg (Margriet); E.W. Steyerberg (Ewout); G. Derksen-Lubsen (Gerarda); H.A. Moll (Henriëtte)

    1998-01-01

    textabstractOBJECTIVE: Prediction of a recurrent febrile seizure during subsequent episodes of fever. DESIGN: Study of the data of the temperatures, seizure recurrences, and baseline patient characteristics that were collected at a randomized placebo controlled trial of ibuprofen

  2. Cocaine-Associated Seizures and Incidence of Status Epilepticus

    Directory of Open Access Journals (Sweden)

    Majlesi, Nima DO

    2010-05-01

    Full Text Available Objectives: Acute complications from cocaine abuse are commonly treated in the emergency department (ED; one of the most consequential is status epilepticus. The incidence of this complication is not clearly defined in the prior literature on cocaine-associated sequelae. We evaluated the incidence of status epilepticus in patients with seizures secondary to suspected cocaine use.Methods: We performed a retrospective multi-center study of patients with seizures resulting from cocaine use. We identified study subjects at 15 hospitals by record review and conducted a computer-assisted records search to identify patients with seizures for each institution over a four-year period. We selected subjects from this group on the basis of cocaine use and determined the occurrence of status epilepticus among them. Data were collected on each subject using a standardized data collection form.Results: We evaluated 43 patients in the ED for cocaine-associated seizures. Their age range was 17 to 54, with a mean age was 31 years; 53% were male. Of 43 patients, 42 experienced a single tonic-clonic seizure and one developed status epilepticus. All patients had either a history of cocaine use or positive urine drug screen for cocaine.Conclusion: Despite reported cases of status epilepticus with cocaine-induced seizures, the incidence of this complication was unclear based on prior literature. This study shows that most cocaine-associated seizures are self-limited. [West J Emerg Med. 2010; 11(2:157-160.

  3. Disparity in regional cerebral blood flow during electrically induced seizure

    DEFF Research Database (Denmark)

    Sestoft, D; Meden, P; Hemmingsen, R

    1993-01-01

    This is a presentation of 2 cases in which the intraictal regional cerebral blood flow distribution was measured with the 99mTc-HMPAO single photon emission computerized tomography technique during an electrically induced seizure. Although the seizure was verified as generalized on electroencepha......This is a presentation of 2 cases in which the intraictal regional cerebral blood flow distribution was measured with the 99mTc-HMPAO single photon emission computerized tomography technique during an electrically induced seizure. Although the seizure was verified as generalized...... electroencephalography-verified generalized seizures....

  4. Early seizure detection in an animal model of temporal lobe epilepsy

    Science.gov (United States)

    Talathi, Sachin S.; Hwang, Dong-Uk; Ditto, William; Carney, Paul R.

    2007-11-01

    The performance of five seizure detection schemes, i.e., Nonlinear embedding delay, Hurst scaling, Wavelet Scale, autocorrelation and gradient of accumulated energy, in their ability to detect EEG seizures close to the seizure onset time were evaluated to determine the feasibility of their application in the development of a real time closed loop seizure intervention program (RCLSIP). The criteria chosen for the performance evaluation were, high statistical robustness as determined through the predictability index, the sensitivity and the specificity of a given measure to detect an EEG seizure, the lag in seizure detection with respect to the EEG seizure onset time, as determined through visual inspection and the computational efficiency for each detection measure. An optimality function was designed to evaluate the overall performance of each measure dependent on the criteria chosen. While each of the above measures analyzed for seizure detection performed very well in terms of the statistical parameters, the nonlinear embedding delay measure was found to have the highest optimality index due to its ability to detect seizure very close to the EEG seizure onset time, thereby making it the most suitable dynamical measure in the development of RCLSIP in rat model with chronic limbic epilepsy.

  5. Long-Term Effects of Ketogenic Diet on Subsequent Seizure-Induced Brain Injury During Early Adulthood: Relationship of Seizure Thresholds to Zinc Transporter-Related Gene Expressions.

    Science.gov (United States)

    Tian, Tian; Li, Li-Li; Zhang, Shu-Qi; Ni, Hong

    2016-12-01

    The divalent cation zinc is associated with cortical plasticity. However, the mechanism of zinc in the pathophysiology of cortical injury-associated neurobehavioral damage following neonatal seizures is uncertain. We have previously shown upregulated expression of ZnT-3; MT-3 in hippocampus of neonatal rats submitted to flurothyl-induced recurrent seizures, which was restored by pretreatment with ketogenic diet (KD). In this study, utilizing a novel "twist" seizure model by coupling early-life flurothyl-induced seizures with later exposure to penicillin, we further investigated the long-term effects of KD on cortical expression of zinc homeostasis-related genes in a systemic scale. Ten Sprague-Dawley rats were assigned each averagely into the non-seizure plus normal diet (NS + ND), non-seizure plus KD (NS + KD), recurrent seizures plus normal diet (RS + ND) and recurrent seizures plus KD (RS + KD) group. Recurrent seizures were induced by volatile flurothyl during P9-P21. During P23-P53, rats in NS + KD and RS + KD groups were dieted with KD. Neurological behavioral parameters of brain damage (plane righting reflex, cliff avoidance reflex, and open field test) were observed at P43. At P63, we examined seizure threshold using penicillin, then the cerebral cortex were evaluated for real-time RT-PCR and western blot study. The RS + ND group showed worse performances in neurological reflex tests and reduced latencies to myoclonic seizures induced by penicillin compared with the control, which was concomitant with altered expressions of ZnT-7, MT-1, MT-2, and ZIP7. Specifically, there was long-term elevated expression of ZIP7 in RS + ND group compared with that in NS + ND that was restored by chronic ketogenic diet (KD) treatment in RS + KD group, which was quite in parallel with the above neurobehavioral changes. Taken together, these findings indicate that the long-term altered expression of the metal transporter ZIP7 in adult cerebral cortex might

  6. Opiate modification of amygdaloid-kindled seizures in rats.

    Science.gov (United States)

    Stone, W S; Eggleton, C E; Berman, R F

    1982-05-01

    Male Long-Evans rats were stereotaxically implanted bilaterally with bipolar electrodes in the central amygdala. Rats were then kindled once daily for 1 sec until 3 consecutive Stage V [25] kindled seizures were elicited. On the following day, animals were injected (IP) with either saline, naloxone (10 mg/kg), naltrexone (10mg/kg) or morphine sulfate (10 mg/kg) and again stimulated at the kindling stimulation parameters. Saline injected animals continued to show long bilateral AD's and behaviors (i.e., forelimb clonus, rearing, falling) typical of Stage V kindled animals. In contrast, rats injected with naloxone or naltrexone showed reduced behavioral seizures. Potentiation of post-ictal spiking by morphine in amygdaloid-kindled rats was also observed supporting previous reports [7,21]. In a second experiment, the reduction of kindled seizure serverity by naloxone was systematically replicated. It is concluded that opiates can significantly modify amygdaloid-kindled seizures, and that brain endorphins may play a role in the development or maintenance of an amygdaloid-kindled seizure focus.

  7. [Oral loading dose of phenytoin in the treatment of serial seizures, prevention of seizure recurrence and rapid drug substitution].

    Science.gov (United States)

    Sokić, D; Janković, S M

    1994-01-01

    Over a period of nine months twenty-five epileptic patients were treated with the oral loading dose of phenytoin. The dose ranged from 12 to 23 mg/kg body weight during 1 to 12 hours. In 20 patients with serial seizures or intolerance to other antiepileptic drugs this treatment was effective. Seizures also stopped in 2 of 4 patients with serial partial motor seizures. These 2 patients required both higher loading dose and faster rate of administration than the other patients. A patient with epilepsia partialis continua failed to respond to the treatment. Patients that received phenytoin through the naso-gastric tube, in respect to oral administration, required higher doses to obtain therapeutic plasma levels of phenytoin. One patient had mild nausea, 3 mild dizziness, and 1 tinitus on the first day of the treatment. There was no correlation between a given dose and the achieved phenytoin plasma levels. In our opinion the therapy with oral loading dose of phenytoin is highly effective in the treatment of serial generalized seizures and rapid antiepileptic drug substitution, and partially effective in the prevention of partial motor seizures. It produces only mild and transient side-effects.

  8. The determinants of seizure severity in Nigerian epileptics | Imam ...

    African Journals Online (AJOL)

    This assesses generalisation of seizures, falls, injuries, urinary incontinence, warning interval before loss of consciousness, automatisms and time of recovery on a graded scale. Results: The most frequent indices of seizure severity in Nigerian epileptics is the generalisation of seizures in 85.7% of subjects, incontinence of ...

  9. 19 CFR 162.21 - Responsibility and authority for seizures.

    Science.gov (United States)

    2010-04-01

    ... 19 Customs Duties 2 2010-04-01 2010-04-01 false Responsibility and authority for seizures. 162.21 Section 162.21 Customs Duties U.S. CUSTOMS AND BORDER PROTECTION, DEPARTMENT OF HOMELAND SECURITY... authority for seizures. (a) Seizures by Customs officers. Property may be seized, if available, by any...

  10. 26 CFR 403.25 - Personal property subject to seizure.

    Science.gov (United States)

    2010-04-01

    ... 26 Internal Revenue 18 2010-04-01 2010-04-01 false Personal property subject to seizure. 403.25... AND ADMINISTRATION DISPOSITION OF SEIZED PERSONAL PROPERTY Seizures and Forfeitures § 403.25 Personal property subject to seizure. Personal property may be seized by the Commissioner of Internal Revenue or his...

  11. 76 FR 26660 - Consolidation of Seizure and Forfeiture Regulations

    Science.gov (United States)

    2011-05-09

    ... CFR Parts 8 and 9 [Docket No. OAG 127; AG Order No. 3263-2011] RIN 1105-AA74 Consolidation of Seizure... seizure and forfeiture regulations, to conform those regulations to the Civil Asset Forfeiture Reform Act..., Tobacco, Firearms, and Explosives'' and generally transferred law enforcement functions, and seizure and...

  12. Sotos syndrome: An interesting disorder with gigantism.

    Science.gov (United States)

    Nalini, A; Biswas, Arundhati

    2008-07-01

    We report the case of a 16-year-old boy diagnosed to have Sotos syndrome, with rare association of bilateral primary optic atrophy and epilepsy. He presented with accelerated linear growth, facial gestalt, distinctive facial features, seizures and progressive diminution of vision in both eyes. He had features of gigantism from early childhood. An MRI showed that brain and endocrine functions were normal. This case is of interest, as we have to be aware of this not so rare disorder. In addition to the classic features, there were two unusual associations with Sotos syndrome in the patient.

  13. Sotos syndrome: An interesting disorder with gigantism

    Science.gov (United States)

    Nalini, A.; Biswas, Arundhati

    2008-01-01

    We report the case of a 16-year-old boy diagnosed to have Sotos syndrome, with rare association of bilateral primary optic atrophy and epilepsy. He presented with accelerated linear growth, facial gestalt, distinctive facial features, seizures and progressive diminution of vision in both eyes. He had features of gigantism from early childhood. An MRI showed that brain and endocrine functions were normal. This case is of interest, as we have to be aware of this not so rare disorder. In addition to the classic features, there were two unusual associations with Sotos syndrome in the patient. PMID:19893668

  14. Sotos syndrome: An interesting disorder with gigantism

    Directory of Open Access Journals (Sweden)

    Nalini A

    2008-01-01

    Full Text Available We report the case of a 16-year-old boy diagnosed to have Sotos syndrome, with rare association of bilateral primary optic atrophy and epilepsy. He presented with accelerated linear growth, facial gestalt, distinctive facial features, seizures and progressive diminution of vision in both eyes. He had features of gigantism from early childhood. An MRI showed that brain and endocrine functions were normal. This case is of interest, as we have to be aware of this not so rare disorder. In addition to the classic features, there were two unusual associations with Sotos syndrome in the patient.

  15. Abnormal radionuclide cerebral angiograms and scans due to seizures

    International Nuclear Information System (INIS)

    Stevens, J.S.; Mishkin, F.S.

    1975-01-01

    The effect of recent seizures on the brain scan was determined in a retrospective study of patients who had had seizures. All patients who underwent brain scanning within eight days of seizures and who did not have a specific intracranial lesion were included. The /sup 99m/Tc-pertechnetate cerebral angiogram and/or delayed scan was abnormal in 73 percent of 22 patients. The data suggest that if seizures occur within six days of the brain imaging, the image is likely to be abnormal. (auth)

  16. 75 FR 38599 - Qualification of Drivers; Exemption Applications; Epilepsy and Seizure Disorders

    Science.gov (United States)

    2010-07-02

    ...-0203] Qualification of Drivers; Exemption Applications; Epilepsy and Seizure Disorders AGENCY: Federal... individuals with seizure disorders to operate CMVs in interstate commerce. DATES: Comments must be received on... anti-seizure medication. Drivers with a history of epilepsy/seizures off anti-seizure medication and...

  17. 76 FR 18822 - Qualification of Drivers; Exemption Applications; Epilepsy and Seizure Disorders

    Science.gov (United States)

    2011-04-05

    ...-0089] Qualification of Drivers; Exemption Applications; Epilepsy and Seizure Disorders AGENCY: Federal... individuals with seizure disorders to operate CMVs in interstate commerce. DATES: Comments must be received on... anti-seizure medication. Drivers with a history of epilepsy/seizures off anti-seizure medication and...

  18. Early symptomatic and late seizures in Kosovar children with bacterial meningitis.

    Science.gov (United States)

    Namani, Sadie A; Kuchar, Ernest; Koci, Remzie; Mehmeti, Murat; Dedushi, Kreshnike

    2011-11-01

    Despite the dramatic decrease of mortality rate among children with bacterial meningitis in recent decades, some patients are left with neurologic sequelae. The purpose of this study was to analyze the occurrence of seizures as predictors for meningitis-related deaths or neurological sequelae including late seizures. This study uses a retrospective chart review of 277 children (aged 0-16 years, median 2 years, 162 boys) treated for bacterial meningitis in University Clinical Centre in Prishtina (Kosovo). Of the 277 children treated for bacterial meningitis, 60 children (22%) manifested seizures prior to admission, 57 children (21%) had seizures after admission, and late seizures were diagnosed in 24 children (9%). The risk for adverse outcome was significantly higher in patients who had seizures prior to admission (52/60) and in patients who manifested seizures later than 24 h (41/41; RR 8.17 and 6.78 respectively, p < 0.0001). All children who manifested late seizures were diagnosed with meningitis-related acute neurologic complications: subdural effusion (18), hydrocephalus (6), intracranial bleeding (1), and subdural empyema (2). Of the 60 children who presented seizures prior to admission, only 11 manifested late seizures. Seizures prior to admission were predictors of high risk of adverse outcome in bacterial meningitis in children. The risk of secondary epilepsy (9%) occurred only in children with evident structural neurologic complications during the acute phase of bacterial meningitis.

  19. Mild overexpression of Mecp2 in mice causes a higher susceptibility toward seizures.

    Science.gov (United States)

    Bodda, Chiranjeevi; Tantra, Martesa; Mollajew, Rustam; Arunachalam, Jayamuruga P; Laccone, Franco A; Can, Karolina; Rosenberger, Albert; Mironov, Sergej L; Ehrenreich, Hannelore; Mannan, Ashraf U

    2013-07-01

    An intriguing finding about the gene encoding methyl-CpG binding protein 2 (MeCP2) is that the loss-of-function mutations cause Rett syndrome and duplication (gain-of-function) of MECP2 leads to another neurological disorder termed MECP2 duplication syndrome. To ensure proper neurodevelopment, a precise regulation of MeCP2 expression is critical, and any gain or loss of MeCP2 over a narrow threshold level may lead to postnatal neurological impairment. To evaluate MeCP2 dosage effects, we generated Mecp2(WT_EGFP) transgenic (TG) mouse in which MeCP2 (endogenous plus TG) is mildly overexpressed (approximately 1.5×). The TG MeCP2(WT_EGFP) fusion protein is functionally active, as cross breeding of these mice with Mecp2 knockout mice led to alleviation of major phenotypes in the null mutant mice, including premature lethality. To characterize the Mecp2(WT_EGFP) mouse model, we performed an extensive battery of behavioral tests, which revealed that these mice manifest increased aggressiveness and higher pentylenetetrazole (PTZ)-induced seizure propensity. Evaluation of neuronal parameters revealed a reduction in the number of tertiary branching sites and increased spine density in Mecp2(WT_EGFP) transgenic (TG) neurons. Treatment of TG neurons with epileptogenic compound-PTZ led to a marked increase in amplitude and frequency of calcium spikes. Based on our ex vivo and in vivo data, we conclude that epileptic seizures are manifested as the first symptom when MeCP2 is mildly overexpressed in mice. Copyright © 2013 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  20. Effect of epileptic seizures on the cerebrospinal fluid--A systematic retrospective analysis.

    Science.gov (United States)

    Tumani, Hayrettin; Jobs, Catherine; Brettschneider, Johannes; Hoppner, Anselm C; Kerling, Frank; Fauser, Susanne

    2015-08-01

    Analyses of the cerebrospinal fluid (CSF) are obligatory when epileptic seizures manifest for the first time in order to exclude life-threatening causes or treatable diseases such as acute infections or autoimmune encephalitis. However, there are only few systematic investigations on the effect of seizures themselves on CSF parameters and the significance of these parameters in differential diagnosis. CSF samples of 309 patients with epileptic and 10 with psychogenic seizures were retrospectively analyzed. CSF samples were collected between 1999 and 2008. Cell counts, the albumin quotient, lactate and Tau-protein levels were determined. Findings were correlated with seizure types, seizure etiology (symptomatic, cryptogenic, occasional seizure), and seizure duration. Pathological findings were only observed in patients with epileptic but not with psychogenic seizures. The lactate concentration was elevated in 14%, the albumin quotient in 34%, and the Tau protein level in 36% of CSF samples. Cell counts were only slightly elevated in 6% of patients. Different seizure types influenced all parameters except for the cell count: In status epilepticus highest, in simple partial seizures lowest values were seen. Symptomatic partial and generalized epileptic seizures had significantly higher Tau-protein levels than cryptogenic partial seizures. In patients with repetitive and occasional epileptic seizures, higher Tau-protein levels were seen than in those with psychogenic seizures. Duration of epileptic seizures was positively correlated with the albumin quotient, lactate and Tau-protein levels. High variability of investigated CSF parameters within each subgroup rendered a clear separation between epileptic and psychogenic seizures impossible. Elevated cell counts are infrequently observed in patients with epileptic seizures and should therefore not uncritically be interpreted as a postictal phenomenon. However, blood-CSF barrier disruption, increased glucose metabolism

  1. pre-hospital management of febrile seizures in children seen

    African Journals Online (AJOL)

    INTRODUCTION. A febrile seizure refers to a seizure occurring in infancy or childhood usually between three months and five years of age as a result of elevated body temperature in the absence of pathology in the brain.1 Febrile seizures are commonly encountered in emergency paediatric practice and have been ...

  2. [Brain lateralization and seizure semiology: ictal clinical lateralizing signs].

    Science.gov (United States)

    Horváth, Réka; Kalmár, Zsuzsanna; Fehér, Nóra; Fogarasi, András; Gyimesi, Csilla; Janszky, József

    2008-07-30

    Clinical lateralizing signs are the phenomena which can unequivocally refer to the hemispheric onset of epileptic seizures. They can improve the localization of epileptogenic zone during presurgical evaluation, moreover, their presence can predict a success of surgical treatment. Primary sensory phenomena such as visual aura in one half of the field of vision or unilateral ictal somatosensory sensation always appear on the contralateral to the focus. Periictal unilateral headache, although it is an infrequent symptom, is usually an ipsilateral sign. Primary motor phenomena like epileptic clonic, tonic movements, the version of head ubiquitously appear contralateral to the epileptogenic zone. Very useful lateralization sign is the ictal hand-dystonia which lateralizes to the contralateral hemisphere in nearly 100%. The last clonus of the secondarily generalized tonic-clonic seizure lateralizes to the ipsilateral hemisphere in 85%. The fast component of ictal nystagmus appears in nearly 100% on the contralateral side of the epileptic focus. Vegetative symptoms during seizures arising from temporal lobe such as spitting, nausea, vomiting, urinary urge are typical for seizures originating from non-dominant (right) hemisphere. Ictal pallor and cold shivers are dominant hemispheric lateralization signs. Postictal unilateral nose wiping refers to the ipsilateral hemispheric focus compared to the wiping hand. Ictal or postictal aphasia refers to seizure arising from dominant hemisphere. Intelligable speech during complex partial seizures appears in non-dominant seizures. Automatism with preserved consciousness refers to the seizures of non-dominant temporal lobe.

  3. Why do seizures occur when they do? Situations perceived to be associated with increased or decreased seizure likelihood in people withepilepsy and intellectual disability

    OpenAIRE

    Illingworth, Josephine L; Watson, Peter; Ring, Howard Anton

    2014-01-01

    Seizure precipitants are commonly reported in the general population of people with epilepsy. However, there has been little research in this area in people with epilepsy and intellectual disability (ID). We conducted a survey of the situations associated with increased or decreased seizure likelihood in this population. The aim of the research was to identify situations of increased seizure likelihood (SISLs) and situations of decreased seizure likelihood (SDSLs) reported by carers of people...

  4. Hyperfusion syndrome after carotid endarterectomy

    International Nuclear Information System (INIS)

    Harrison, P.B.; Wong, M.J.; Belzberg, A.; Holden, J.

    1991-01-01

    This article describes the CT scans of two patients who, following carotid endarterectomy, developed headache and seizures suggestive of hyperperfusion syndrome. Their CT scans demonstrated ipsilateral mass effect and white matter hypodensity. One patient progressed to hemorrhage and died. Although infarction is described as the commonest neurologic event to occur after carotid endarterectomy, autopsy or cerebral blood flow studies in these patients suggests that the changes were due to hyperperfusion rather that infarction. (orig.)

  5. Clinical and psychosocial characteristics of children with nonepileptic seizures

    Directory of Open Access Journals (Sweden)

    Chinta Sri

    2008-01-01

    Full Text Available Objective: The aim of this study is to present a comprehensive profile of clinical and psychosocial characteristics of children with psychogenic nonepileptic seizures and to assess the short-term outcome of these patients. Materials and Methods: The subjects were consecutive cases of children with a diagnosis of nonepileptic seizures (N=17, mean age = 10.7 years, S.D. = 1.26 and two groups of control groups matched on age and sex: true seizure group and healthy controls. All the children were recruited from the out-patient services of the Department of Pediatrics of a tertiary care teaching hospital in North India. Detailed history taking and clinical examination was done in the case of every child. A standard 18 channel EEG was done in all the children and a video EEG was done in 12 cases of children with nonepileptic seizures. The Childhood Psychopathology Measurement Schedule (CPMS and Life Events Scale for Indian Children (LESIC were used to measure the children′s emotional and behavioral functioning at home, and the number of life events and the stress associated with these events in the preceding year and the year before that. Short-term outcome was examined three to six months after the diagnosis of nonepileptic seizures was made. Results: Unresponsiveness without marked motor manifestations was the most common "ictal" characteristic of the nonepileptic seizures. Pelvic thrusting, upper and lower limb movements, head movements, and vocalization were observed in less than one-third of the patients. Increased psychosocial stress and significantly higher number of life events in the preceding year were found to characterize children with nonepileptic seizures, as compared to the two control groups. The nonepileptic seizures and true seizures groups had a higher proportion of children with psychopathology scores in the clinically significant maladjustment range, as compared to those in the healthy control group. A majority of the patients

  6. What is the fate of disconnected brain tissue in a child with Rasmussen syndrome? A case report

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    Kim, S.J.; Park, Y.D. [Dept. of Neurology (Child), Medical Coll. of Georgia, Augusta, GA (United States); Pillai, J. [Dept. of Radiology, Medical Coll. of Georgia, Augusta, GA (United States)

    2003-04-01

    We describe a case of Rasmussen syndrome in a 7-year-old boy, presenting with epilepsia partialis continua, hemiplegia, and progressive mental deterioration. The initial MRI examination was normal, followed by progressive left hemispheric cortical atrophy and abnormal high signal intensity over the left occipital, parietal, and cingulate gyral areas over an 18-month period. On the basis of the clinical diagnosis and biopsy findings of Rasmussen syndrome, functional hemispherectomy was carried out at 7.5 years of age with alleviation of clinical seizures for the following 44 months. The follow-up MRI demonstrated atrophic changes involving the remaining left hemisphere with increased signal and cortical volume loss, as well as the absence of abnormal signal in the right hemisphere at 10 years of age. Our MRI findings are consistent with the progression of Rasmussen syndrome in the ipsilateral hemisphere even after functional-hemispherectomy without clinical seizures. (orig.)

  7. What is the fate of disconnected brain tissue in a child with Rasmussen syndrome? A case report

    International Nuclear Information System (INIS)

    Kim, S.J.; Park, Y.D.; Pillai, J.

    2003-01-01

    We describe a case of Rasmussen syndrome in a 7-year-old boy, presenting with epilepsia partialis continua, hemiplegia, and progressive mental deterioration. The initial MRI examination was normal, followed by progressive left hemispheric cortical atrophy and abnormal high signal intensity over the left occipital, parietal, and cingulate gyral areas over an 18-month period. On the basis of the clinical diagnosis and biopsy findings of Rasmussen syndrome, functional hemispherectomy was carried out at 7.5 years of age with alleviation of clinical seizures for the following 44 months. The follow-up MRI demonstrated atrophic changes involving the remaining left hemisphere with increased signal and cortical volume loss, as well as the absence of abnormal signal in the right hemisphere at 10 years of age. Our MRI findings are consistent with the progression of Rasmussen syndrome in the ipsilateral hemisphere even after functional-hemispherectomy without clinical seizures. (orig.)

  8. Management of a severe forceful breather with Rett syndrome using carbogen.

    Science.gov (United States)

    Smeets, Eric E J; Julu, Peter O O; van Waardenburg, Dick; Engerström, Ingegerd Witt; Hansen, Stig; Apartopoulos, Flora; Curfs, Leopold M G; Schrander-Stumpel, Connie T R M

    2006-11-01

    We have used a novel neurophysiological technique in the NeuroScope system in combination with conventional electroencephalography (EEG) to monitor both brainstem and cortical activity simultaneously in real-time in a girl with Rett syndrome. The presenting clinical features in our patient were severe sleep disturbances, irregular breathing in the awake state dominated by Valsalva's type of breathing followed by tachypnoea and very frequent attacks of seizures and vacant spells. Our novel neurophysiological data showed that the patient was a Forceful Breather according to the breathing categories in Rett syndrome. She had frequent abnormal spontaneous brainstem activation (ASBA) preceded by severe attacks of hypocapnoea, which was caused by a combination of Valsalva's type of breathing and tachypnoea and all these together were responsible for the seizures and non-epileptic vacant spells. The ASBA was not detectable in conventional EEG and there were no epileptiform changes in the EEG during the seizures and vacant spells caused by the hypocapnic attacks, therefore these were pseudo-seizures.