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Intractable epilepsy

Letícia Pereira de Brito Sampaio
The ketogenic diet (KD), a high-fat, low-carbohydrate, and adequate-protein diet is an established, effective nonpharmacologic treatment option for intractable childhood epilepsy. The KD was developed in 1921 and even though it has been increasingly used worldwide in the past decade, many neurologists are not familiar with this therapeutic approach. In the past few years, alternative and more flexible KD variants have been developed to make the treatment easier and more palatable while reducing side effects and making it available to larger group of refractory epilepsy patients...
October 2016: Arquivos de Neuro-psiquiatria
Garima Shukla, Anupama Gupta, Priya Agarwal, Shivani Poornima
PURPOSE: Levetiracetam (LEV) is often chosen early in the treatment of refractory epilepsy; however, its adverse effects have largely been studied as part of clinical trials. Oxcarbazepine and valproate (VPA) are the other commonly used AEDs and, hence, serve as good comparators. This study was conducted to evaluate behavioral abnormalities and somnolence among patients with epilepsy being treated with LEV and/or OXC compared with those receiving VPA. METHOD: Data of consecutive patients attending our intractable epilepsy clinic over a 2 1/2-year period were reviewed, and patients with at least one seizure a month, who had been initiated on either or a combination of LEV, VPA, or OXC, were included for analysis...
October 15, 2016: Epilepsy & Behavior: E&B
Kazuhiro Muramatsu, Noriko Sawaura, Tomomi Ogata, Nishiki Makioka, Keiko Tomita, Toshino Motojima, Kuniko Ida, Kyoko Hazama, Hirokazu Arakawa
INTRODUCTION: Levetiracetam has a high tolerability and is effective against various seizure types and epilepsy syndromes. However, no study has specifically evaluated the efficacy of levetiracetam in children with refractory epilepsy based on magnetic resonance imaging (MRI) findings and the presence of intellectual disability (ID). METHODS: We retrospectively evaluated levetiracetam efficacy and safety in 49 pediatric patients who met the following inclusion criteria: (1) diagnosis of refractory epilepsy with first-line antiepileptic (AED) treatment ⩾2years, (2) younger than 20years old, and (3) received oral levetiracetam treatment for ⩾6months...
October 13, 2016: Brain & Development
Yu Kobayashi, Jun Tohyama, Tomoyuki Akiyama, Shinichi Magara, Hideshi Kawashima, Noriyuki Akasaka, Mitsuko Nakashima, Hirotomo Saitsu, Naomichi Matsumoto
Cerebral folate deficiency due to folate receptor 1 gene (FOLR1) mutations is an autosomal recessive disorder resulting from a brain-specific folate transport defect. It is characterized by late infantile onset, severe psychomotor regression, epilepsy, and leukodystrophy. We describe a consanguineous girl exhibiting severe developmental regression, intractable epilepsy, polyneuropathy, and profound hypomyelination with cortical involvement. Magnetic resonance imaging showed cortical disturbances in addition to profound hypomyelination and cerebellar atrophy...
October 12, 2016: Brain & Development
Tomokazu Kimizu, Yukitoshi Takahashi, Taikan Oboshi, Asako Horino, Takayoshi Koike, Shinsaku Yoshitomi, Tatsuo Mori, Tokito Yamaguchi, Hiroko Ikeda, Nobuhiko Okamoto, Mitsuko Nakashima, Hirotomo Saitsu, Mitsuhiro Kato, Naomichi Matsumoto, Katsumi Imai
INTRODUCTION: Mutations of SLC35A2 that encodes Golgi-localized Uridine diphosphate (UDP)-galactose transporter at Xp11.23 lead to congenital disorders of glycosylation (CDG). Although patients with CDG generally have diverse systemic symptoms, patients with a SLC35A2 mutation manifest predominantly disorders of the central nervous system (CNS). CASE REPORT: A female infant aged 12months was referred to our center because of intractable seizures. The patient was born with birth weight of 3228g after 40weeks of unremarkable gestation...
October 12, 2016: Brain & Development
Luis E Bruno-Blanch, Luis Enrique
Despite the introduction of more than 15 third generation antiepileptic drugs to the market from 1990 to the moment, about one third of the epileptic patients still suffer from refractory of intractable epilepsy. Several hypotheses seek to explain the failure of drug treatments to control epilepsy symptoms in such patients. The most studied one proposes that drug resistance might be related with regional overactivity of efflux transporters from the ATP-Binding Cassette (ABC) superfamily at the blood-brain barrier and/or the epileptic foci in the brain...
October 13, 2016: Mini Reviews in Medicinal Chemistry
Klajdi Puka, Mary Lou Smith
OBJECTIVE: Little is known about the long-term cognitive outcomes following pediatric epilepsy surgery. Although the evidence for change within the first 2 years is not compelling, the plasticity of the immature brain may allow for improvements in the long term. This study examined memory function in a cohort of surgical and nonsurgical patients at baseline and 4-11 years after. METHOD: Participants were 88 patients (mean age 20.05, standard deviation [SD] 4.21 years) with childhood-onset intractable epilepsy; 53 had undergone resective epilepsy surgery...
October 12, 2016: Epilepsia
Christina Eguizabal Love, Frank Webbe, Gunha Kim, Ki Hyeong Lee, Michael Westerveld, Christine M Salinas
OBJECTIVE: Children with epilepsy are vulnerable to executive dysfunction, but the relationship between executive functioning (EF) and quality of life (QOL) in children with epilepsy is not fully delineated. This exploratory study elucidated the relationship between ecological EF and QOL in pediatric intractable epilepsy. METHOD: Fifty-four consecutively referred pediatric epilepsy surgery candidates and their parents were administered IQ measures, the Behavior Rating Inventory of Executive Function (BRIEF), and the Quality of Life in Childhood Epilepsy (QOLCE) as part of a comprehensive neuropsychological evaluation...
October 8, 2016: Epilepsy & Behavior: E&B
Ping Ding, Shaohui Zhang, Junchen Zhang, Xiaohong Hu, Xiaoman Yu, Shuangshuang Liang, Chao Gao, Shuli Liang
AIMS: To prospectively study the surgical outcomes of unilateral anterior temporal lobectomy (ATL) in patients with intractable bilateral temporal lobe epilepsy (TLE) as well as two-staged contralateral hippocampal stimulation in patients after failed unilateral ATL. METHODS: Eighteen carefully selected patients with bilateral TLE underwent unilateral ATL. Five cases with failed ATL underwent two-staged contralateral hippocampal stimulation. Seizure control and changes in intelligence quotient (IQ), memory quotient, and quality of life (QOL) were analyzed 2-5 years after treatment...
October 11, 2016: Stereotactic and Functional Neurosurgery
Min-Jee Kim, Mi-Sun Yum, Eun-Hee Kim, Yun-Jeong Lee, Junkyo Lee, Seokho Hong, Su Jeong You, Yong Soon Hwang, Tae-Sung Ko
PURPOSE: This study aimed to evaluate the long-term efficacy of vagus nerve stimulation (VNS) in children and adolescents with intractable epilepsy and identify predictive factors for responsiveness to VNS. METHODS: Medical records of pediatric patients who underwent VNS implantation at two Korean tertiary centers were reviewed. At 0.5, 1, 3, and 5 years post-VNS implantation, the frequency of the most disabling seizures in each patient was assessed. Responders were defined as showing an overall 50 % reduction from baseline seizure frequency during follow-up...
October 6, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Sarah Aminoff Kelley, Eric Heath Kossoff
Electrical status epilepticus of sleep (ESES), with the activation of profuse amounts of epileptiform discharges in sleep, may lead to intractable epilepsy and neurocognitive decline in children. Numerous varied treatments including antiseizure medications, steroids, and surgery have been investigated as possible treatment options. The ketogenic diet (KD) is an additional treatment option which may add to our treatment armamentarium for ESES. The KD may theoretically improve ESES by affecting GABA systems and reducing inflammation...
September 23, 2016: Epilepsy Research
Samuel B Tomlinson, Arun Venkataraman
Surgical intervention often fails to achieve seizure-free results in patients with intractable epilepsy. Identifying features of the epileptic brain that dispose certain patients to unfavorable surgical outcomes is a central challenge for epilepsy researchers. In this NeuroForum, we review evidence suggesting that pathways of secondary seizure generalization distinguish patients with favorable (i.e., seizure-free) versus unfavorable (i.e., seizure-persistent) surgical outcomes. The network underpinnings and clinical implications of these findings are then discussed...
October 5, 2016: Journal of Neurophysiology
Nicholas Cothros, Jorge G Burneo, David A Steven
BACKGROUND: Approximately 30% of patients with epilepsy have medically intractable seizures, and a proportion of them are candidates for surgical treatment. The efficacy and safety of epilepsy surgery have been supported by a large number of studies, yet only a small minority of such patients in Ontario receive surgery. METHODS: Family physicians in Ontario were surveyed regarding demographics, referral practices and general knowledge about epilepsy surgery. Four hundred surveys were mailed to randomly selected family physicians using contact information from the College of Physicians and Surgeons of Ontario website...
September 2016: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
J Hinojosa, S Gil-Robles, B Pascual
Insula and paralimbic region represent a common location for gliomas in adulthood. However, limbic and paralimbic tumors are rare in children. Reports of pediatric insular tumors are scarce in literature, and most of them are included in adult's series, so their management and outcome can be outlined only after extracting data from these reports. Due to their predominantly low grade, they usually have a benign course for some time, what make them ideal candidates for total resection. However, their intricate location and spread to key areas, including the temporal lobe, make them a surgical challenge...
October 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Alvaro Lassaletta, Michal Zapotocky, Eric Bouffet, Cynthia Hawkins, Uri Tabori
Hemispheric low-grade gliomas account for the second most common location in pediatric low-grade gliomas (PLGGs) after the cerebellum. The pathological spectrum includes gangliogliomas, dysembryoplastic neuroepithelial tumors (DNETs), diffuse astrocytomas, pilocytic astrocytomas, and pleomorphic xanthoastrocytomas (PXAs), among others. Clinically, hemispheric PLGGs represent a well-recognized cause of intractable epilepsy in children and adolescents. With an excellent long-term outcome, surgery remains the cornerstone and patients with gross total resection typically do not need any further therapies...
October 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Xiangyu Liu, Taisuke Otsuki, Akio Takahashi, Takanobu Kaido
INTRODUCTION: The authors here present a rare case of a 3-month-old infant with unilateral Sturge-Weber syndrome (SWS) who had excellent seizure control and no aggravation of previous existed neurological deficits after vertical parasagittal hemispherotomy (VPH). To our knowledge, this patient with SWS was the youngest one who received VPH. CASE DESCRIPTION: The use of VPH results in a successful treatment of intractable epilepsy in a patient with seizure onset in early infancy...
2016: SpringerPlus
Jiong Yue, Wei Li, Chao Liang, Bing Chen, Xin Chen, Lukang Wang, Zhenle Zang, Sixun Yu, Shiyong Liu, Song Li, Hui Yang
Temporal lobe epilepsy (TLE) is a frequent form of focal intractable epilepsy in adults, but the specific mechanism underlying the epileptogenesis of TLE is still unknown. Human leukocyte immunoglobulin-like receptor B2 (LILRB2) (the murine homolog gene called paired immunoglobulin-like receptor B, or PirB), participates in the process of synaptic plasticity and neurite growth in the central nervous system (CNS), suggesting a potential role of LILRB2 in epilepsy. However, the expression pattern of LILRB2 and the downstream molecular signal in intractable TLE remains poorly understood...
November 2016: Experimental Neurology
Hae Yu Kim, Yun Jung Hur, Heung-Dong Kim, Kang Min Park, Sung Eun Kim, Tae Gyu Hwang
OBJECTIVE Thalamic stimulation can provoke electroencephalography (EEG) synchronization or desynchronization, which can help to reduce the occurrence of seizures in intractable epilepsy, though the underlying mechanism is not fully understood. Therefore, the authors investigated changes in EEG electrical activity to better understand the seizure-reducing effects of deep brain stimulation (DBS) in patients with intractable epilepsy. METHODS Electrical activation patterns in the epileptogenic brains of 3 patients were analyzed using classical low-resolution electromagnetic tomography analysis recursively applied (CLARA)...
September 16, 2016: Journal of Neurosurgery
Lucy F Robinson, Xiaosong He, Paul Barnett, Gaёlle E Doucet, Michael R Sperling, Ashwini Sharan, Joseph I Tracy
OBJECTIVE: Focal epilepsies, such as temporal lobe epilepsy (TLE), are known to disrupt network activity in areas outside the epileptogenic zone [Tracy et al., 2015]. We devised a measure of temporal instability of resting state functional connectivity (FC), capturing temporal variations of BOLD correlations between brain regions that is less confounded than the "sliding window" approach common in the literature. METHODS: We investigated healthy controls and unilateral TLE patients (right and left seizure focus groups), utilizing group ICA to identify the default mode network (DMN), a network associated with episodic memory, a key cognitive deficit in TLE...
September 15, 2016: Human Brain Mapping
Eun-Ik Son, Ji-Eun Kim
It is well known that there is high quality evidence of epilepsy surgery as an effective and safe option for patients with drug refractory epilepsy by advanced imaging technology and computerized electrophysiological facilities during recent three decades. However, it still remains debate regarding necessities of epilepsy surgery in terms of less satisfactory surgical outcome, especially in non-lesional neocortical epilepsies. This review is for the role of epileptic neurosurgeon rather than the role of epilepsy surgery, namely, the necessity of neurosurgeon's positive participation starting from the first visit of epilepsy patients followed by pertaining process by stages and its degree of contribution...
June 2016: Biomedical Journal
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