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Intractable epilepsy

Matthew S Parsons, Aseem Sharma, Charles Hildebolt
RATIONALE AND OBJECTIVES: To test whether an image-processing algorithm can aid in visualization of mesial temporal sclerosis on magnetic resonance imaging by selectively increasing contrast-to-noise ratio (CNR) between abnormal hippocampus and normal brain. MATERIALS AND METHODS: In this Institutional Review Board-approved and Health Insurance Portability and Accountability Act-compliant study, baseline coronal fluid-attenuated inversion recovery images of 18 adults (10 females, eight males; mean age 41...
June 12, 2018: Academic Radiology
Anjuna Reghunath, Rohini Gupta Ghasi, Abhimanyu Bhargava, Narinder Kumar Bhambri
Posterior quadrantic dysplasia (PQD) is a rare cause of pediatric intractable epilepsy. It is a sporadic cortical development malformation that involves the posterior three lobes of a single hemisphere and spares the frontal cortex. Very few cases have been reported in the literature, mostly as anecdotal reports or as a part of large series of refractory epilepsy. It is essential to know about this lesser known entity and differentiate it from other more common similar anomalies such as multilobar cortical dysplasia and hemimegalencephaly as new motor-sparing neurosurgical disconnective procedures have led to dramatically reduced mortality and morbidity rates, apart from gifting the affected children a better quality of life...
January 2018: Journal of Pediatric Neurosciences
Barbara Hansen, Magdalena Szaflarski, E Martina Bebin, Jerzy P Szaflarski
Intractable epilepsy can be challenging for patients and for their families. Disability rates in patients are high, causing tremendous physical and emotional burden on family caregivers. Additionally, caregivers may experience affiliate stigma, where they perceive and internalize the negative societal views of a condition and exhibit a psychological response. Affiliate stigma has been rarely studied in caregivers of those with intractable epilepsy. This study examined the relationship between affiliate stigma and the levels of burden experienced by caregivers, as well as how these levels may vary between those caring for children and adults...
June 7, 2018: Epilepsy & Behavior: E&B
Thomas Mathew, Amrutha Avati, Delon D'Souza, Manjusha Therambil
Pontocerebellar hypoplasia type 6 (PCH6) is an autosomal recessive mitochondrial disease, typically characterized by pontine atrophy, vermian hypoplasia, infantile encephalopathy, generalized hypotonia, and intractable seizures. The purpose of this study is to describe the seizures and other neurological manifestations of RARS 2 gene mutations and to compare the clinical features with other causes of progressive myoclonic epilepsy. Detailed history, physical examination, and clinical and genetic work-up were performed in 2 siblings who presented with progressive myoclonic epilepsy...
June 2018: Epilepsia Open
Eric H Kossoff, Beth A Zupec-Kania, Stéphane Auvin, Karen R Ballaban-Gil, A G Christina Bergqvist, Robyn Blackford, Jeffrey R Buchhalter, Roberto H Caraballo, J Helen Cross, Maria G Dahlin, Elizabeth J Donner, Orkide Guzel, Rana S Jehle, Joerg Klepper, Hoon-Chul Kang, Danielle A Lambrechts, Y M Christiana Liu, Janak K Nathan, Douglas R Nordli, Heidi H Pfeifer, Jong M Rho, Ingrid E Scheffer, Suvasini Sharma, Carl E Stafstrom, Elizabeth A Thiele, Zahava Turner, Maria M Vaccarezza, Elles J T M van der Louw, Pierangelo Veggiotti, James W Wheless, Elaine C Wirrell
Ketogenic dietary therapies (KDTs) are established, effective nonpharmacologic treatments for intractable childhood epilepsy. For many years KDTs were implemented differently throughout the world due to lack of consistent protocols. In 2009, an expert consensus guideline for the management of children on KDT was published, focusing on topics of patient selection, pre-KDT counseling and evaluation, diet choice and attributes, implementation, supplementation, follow-up, side events, and KDT discontinuation. It has been helpful in outlining a state-of-the-art protocol, standardizing KDT for multicenter clinical trials, and identifying areas of controversy and uncertainty for future research...
June 2018: Epilepsia Open
Kavelin Rumalla, Kyle A Smith, Paul M Arnold, Theodore H Schwartz
BACKGROUND: Hospital readmissions can be detrimental to patients and may interfere with the potential benefits of the therapeutic procedure. Government agencies have begun to focus on reducing readmissions; however, the etiology of readmissions is lacking. OBJECTIVE: To report the national rates, risk factors, and outcomes associated with 30- and 90-d readmissions following surgery for intractable epilepsy. METHODS: We queried the Nationwide Readmissions Database from January to September 2013 using International Classification of Diseases, Ninth Edition, Clinical Modification codes to identify all patients with intractable epilepsy, who underwent hemispherectomy (01...
June 4, 2018: Operative Neurosurgery (Hagerstown, Md.)
Alireza Mansouri, George M Ibrahim
No abstract text is available yet for this article.
June 4, 2018: JAMA Neurology
Shino Shimada, Hirokazu Oguni, Yui Otani, Aiko Nishikawa, Susumu Ito, Kaoru Eto, Tomoyuki Nakazawa, Keiko Yamamoto-Shimojima, Jun-Ichi Takanashi, Satoru Nagata, Toshiyuki Yamamoto
Microdeletions in the 1q44 region encompassing the HNRNPU gene have been associated with infantile spasms and hemiconvulsion-hemiplegia-epilepsy syndrome. Recent studies have revealed that heterozygous HNRNPU variants resulted in early onset epilepsy and severe intellectual disability. A de novo frameshift mutation in HNRNPU was identified in a 5-year-old boy with developmental delay associated with Rett-like features including stereotypic hand movements and respiratory abnormalities with episode of apnea and hyperpnea followed by falling...
May 29, 2018: Brain & Development
Rui Feng, Jie Hu, Jinsong Wu, Liqin Lang, Chengxin Ma, Bing Sun, Xin Gu, Li Pan
PURPOSE: High-density electroencephalographic source imaging (HD-ESI) has emerged as a useful tool for pre-surgical epilepsy workup. However, it is not routinely used in clinical evaluations due to several factors, one of which is the challenge associated with creating anatomically accurate head models. Reasonable solutions now exist and the present study aims to evaluate the use of these highly resolved individual head models in pre-surgical epilepsy evaluation. METHODS: Nine patients with intractable epilepsy who were candidates for resective epilepsy surgeries participated in the study...
May 19, 2018: Seizure: the Journal of the British Epilepsy Association
M Večeřa, J Šána, R Bútová, Š Reguli, M Hermanová, L Křen, R Lipina, M Smrčka, O Slabý
BACKGROUND: Glioblastoma (GBM) is the most frequent primary brain tumor characterized by an unfavourable prognosis despite multimodal therapy. Therefore, a lot of efforts and financial resources are dedicated to the research of new therapeutic targets and prognostic or predictive biomarkers. Long non-coding RNAs (lncRNAs) are regulators of gene expression which play a significant role in GBM pathology and, thus, present promising candidates. MATERIAL AND METHODS: Our study included 14 patients with GBM and 8 patients with intractable epilepsy from whom we acquired brain tissues during surgical intervention...
2018: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
Memoona Nasir, Maham Jehangir, Rashed Nazir Ahmed, Ejaz Khan, Arsalan Ahmad
Hemiconvulsion hemiplegia epilepsy (HHE) syndrome is a rare complication of prolonged focal seizures in children upto 4 years of age. It is usually idiopathic and seen in the setting of febrile seizures in otherwise normal children but less commonly is also associated with structural, infective, traumatic and degenerative diseases that predispose to seizures. It has 3 stages, the first of prolonged focal seizures, then the development of hemiplegia and then followed by final stage of development of epilepsy after a variable latent period...
April 2018: JPMA. the Journal of the Pakistan Medical Association
Lily C Wong-Kisiel, Thomas Blauwblomme, Mai-Lan Ho, Nathalie Boddaert, Joseph Parisi, Elaine Wirrell, Rima Nabbout
Focal cortical dysplasia (FCD) is the most common cause of intractable focal epilepsy in children, in whom seizures are most commonly pharmacoresistant from onset. This review summarizes the current understandings of the epidemiology, natural history, and the proposed mechanisms of epileptogenisis in FCD. Advances in neuroimaging techniques have enhanced the recognition of this pathology, which can be subtle. Illustrative neurophysiology and imaging examples are provided to help the clinicians identify diagnostic evidence of suspected FCD...
May 14, 2018: Epilepsy Research
Robert A McGovern, Soha Alomar, William E Bingaman, Jorge Gonzalez-Martinez
BACKGROUND: The management of medically refractory epilepsy patients who are not surgical candidates has remained challenging. Closed loop-or responsive-neurostimulation (RNS) is now an established therapy for the treatment of epilepsy with specific indications. The RNS® system (NeuroPace Inc, Mountainview, California) has recently been shown to be effective in reducing the seizure frequency of partial onset seizures. The electrode design consists of either intracerebral depth electrodes or subdural strip electrodes, and stereotaxis is typically used to guide placement into the EZ...
May 23, 2018: Operative Neurosurgery (Hagerstown, Md.)
Christian Vollmar, Aurelia Peraud, Soheyl Noachtar
Neuroimaging is crucial for the evaluation of patients considered for resective epilepsy surgery. Multimodal image fusion is a new tool to integrate all available localizing information on the individual epileptogenic network in a three-dimensional (3D) manner to plan invasive EEG recordings and delineate the epileptogenic zone from the eloquent cortex for the neurosurgical planning of a tailored resection. Here, we illustrate the multimodal fusion of images from different modalities in a patient with medically intractable non-lesional frontal lobe epilepsy who underwent partial frontal lobe resection, rendering him seizure-free...
March 18, 2018: Curēus
Chun-Lei Han, Ming Ge, Yun-Peng Liu, Xue-Min Zhao, Kai-Liang Wang, Ning Chen, Wei Hu, Jian-Guo Zhang, Liang Li, Fan-Gang Meng
Temporal lobe epilepsy (TLE) is one of the most common types of intractable epilepsy, characterized by hippocampal neuron damage and hippocampal sclerosis. Long noncoding RNAs (lncRNAs) have been increasingly recognized as posttranscriptional regulators. However, their expression levels and functions in TLE remain largely unknown. In the present study, TLE rat model is used to explore the expression profiles of lncRNAs in the hippocampus of epileptic rats using microarray analysis. Our results demonstrate that H19 is the most pronouncedly differentiated lncRNA, significantly upregulated in the latent period of TLE...
May 23, 2018: Cell Death & Disease
L Gupta, P A M Hofman, R M H Besseling, J F A Jansen, W H Backes
BACKGROUND AND PURPOSE: Focal cortical dysplasia is a common cause of intractable epilepsy for which neurosurgery is an option. Delineations of a focal cortical dysplasia lesion on structural brain images may not necessarily reflect the functional borders of normal tissue. Our objective was to determine whether abnormalities in spontaneous blood oxygen level-dependent fluctuations arise in focal cortical dysplasia lesions and proximal regions. MATERIALS AND METHODS: Fourteen patients with focal cortical dysplasia-related epilepsy and 16 healthy controls underwent structural and resting-state functional MR imaging...
May 24, 2018: AJNR. American Journal of Neuroradiology
Kaijia Yu, Chang Liu, Tao Yu, Xueyuan Wang, Cuiping Xu, Duanyu Ni, Yongjie Li
The authors present a case of an out-of- body experience (OBE) that occurred in a child with intractable epilepsy when stimulating the anterior insular cortex (AIC) by intracranial electrodes. After destroying several sites precisely located at the left AIC by radiofrequency thermocoagulation, the patient's seizures disappeared, and the OBE could not be reproduced when repeatedly stimulating these sites. To our knowledge, it has never been reported that an OBE occurred in the AIC. We analyze the mechanism of an OBE mostly appearing in temporo-parietal junction (TPJ) and propose two perspectives for a possible mechanism involving the AIC causing the OBE...
May 21, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Xiu-Yu Du, Si-Chang Chen, Yu-Guang Guan, Jing-Jing Gu, Meng Zhao, Tian-Fu Li, Jun-Hong Pan, Guo-Ming Luan
OBJECTIVE: Hemispherectomy has been successfully used for medically intractable epilepsy patients. However, it is difficult to predict postoperative motor function. The aim of the present study was to analyze whether the preoperative asymmetry of cerebral peduncles could be used to predict motor function restoration before hemispherectomy for young patients with medically intractable epilepsy. METHODS: The clinical record and MRI data of 53 patients were retrospectively analyzed...
May 16, 2018: World Neurosurgery
Takeshi Matsuo, Kensuke Kawai, Kenji Ibayashi, Ichiro Shirouzu, Miki Sato
BACKGROUND: The lesionectomy is a general surgical procedure for treating focal epilepsy resulting from a structural abnormality, but some patients can not achieve favorable outcome, especially patients whose symptomatogenic zone is located in remote regions. Herein we propose the surgical disconnection of the seizure propagation pathway, which consists of short and long associating fibers linking the epileptogenic zone to the remote symptomatogenic zone, as an effective method of achieving favorable seizure outcomes in patients with posterior cortex epilepsy...
May 15, 2018: World Neurosurgery
Massimo Piacentino, Giacomo Beggio, Lara Zordan, Paolo Bonanni
Hippocampal deep brain stimulation (DBS) can provide an effective alternative for intractable temporal lobe epilepsy. In this case report, we describe a peculiar outcome after a post-traumatic wire-disconnection of a bilateral hippocampal DBS device. The patient presented a postoperative long-term significant reduction in seizure frequency even with an absent electric stimulation. This case gives the possibility to consider alternatives in epilepsy surgery, based on stimulation interference (lesional or electrical disturbing) in the epileptogenic zone...
May 13, 2018: Neurological Sciences
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