Yongxin Li , Ya Wang , Zhen Tan , Qian Chen , Wenhua Huang
{"title":"Longitudinal brain functional and structural connectivity changes after hemispherotomy in two pediatric patients with drug-resistant epilepsy","authors":"Yongxin Li , Ya Wang , Zhen Tan , Qian Chen , Wenhua Huang","doi":"10.1016/j.ebcr.2018.11.003","DOIUrl":"10.1016/j.ebcr.2018.11.003","url":null,"abstract":"<div><p>The main focus of the present study was to explore the longitudinal changes in the brain executive control system and default mode network after hemispherotomy. Resting-state functional magnetic resonance imaging and diffusion tensor imaging were collected in two children with drug-resistnt epilepsy underwent hemispherotomy. Two patients with different curative effects showed different trajectories of brain connectivity after surgery. The failed hemispherotomy might be due to the fact that the synchrony of epileptic neurons in both hemispheres is preserved by residual neural pathways. Loss of interhemispheric correlations with increased intrahemispheric correlations can be considered as neural marker for evaluating the success of hemispherotomy.</p></div>","PeriodicalId":56365,"journal":{"name":"Epilepsy and Behavior Case Reports","volume":"11 ","pages":"Pages 58-66"},"PeriodicalIF":0.0,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.ebcr.2018.11.003","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36922144","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alejandro Viloria-Alebesque , Elena Bellosta-Diago , Sonia Santos-Lasaosa , José Ángel Mauri-Llerda
{"title":"Familial association of genetic generalised epilepsy with limb-girdle muscular dystrophy through a mutation in CAPN3","authors":"Alejandro Viloria-Alebesque , Elena Bellosta-Diago , Sonia Santos-Lasaosa , José Ángel Mauri-Llerda","doi":"10.1016/j.ebcr.2019.03.003","DOIUrl":"10.1016/j.ebcr.2019.03.003","url":null,"abstract":"","PeriodicalId":56365,"journal":{"name":"Epilepsy and Behavior Case Reports","volume":"11 ","pages":"Pages 122-124"},"PeriodicalIF":0.0,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.ebcr.2019.03.003","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37173979","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Sub-lobar dysplasia — A comprehensive evaluation with neuroimaging, magnetoencephalography and histopathology","authors":"Kenchaiah Raghavendra , Ganne Chaitanya , Bhargava Goutham , Anita Mahadevan , Ravindranadh Chowdary Mundlamuri , Rose Dawn Bharath , Mariyappa Narayannan , Malla Bhaskar Rao , Arimappamagan Arivazhagan , Parthasarthy Satishchandra , Sanjib Sinha","doi":"10.1016/j.ebcr.2017.11.002","DOIUrl":"10.1016/j.ebcr.2017.11.002","url":null,"abstract":"<div><p>Sublobar dysplasia, a rare cortical malformation has been defined in only 8 patients to date. It was identified on the basis of histopathological features and MRI findings. We report a right temporal sublobar dysplasia, with detailed evaluation including neuroimaging, magnetoencephalography and histopathology to further characterize the pathology. Additional pathological features included a deep collateral sulcus in the basal right temporal lobe, thinned out right corticospinal tract, and bilateral asymmetric basal ganglia changes. Magnetoencephalograpy localized the seizure focus to the posterior margin of the dysplasia. Histopathological evaluation helped exclude other types of dysplasia. Similar to a previous study, the child had Engel 1a outcome.</p></div>","PeriodicalId":56365,"journal":{"name":"Epilepsy and Behavior Case Reports","volume":"9 ","pages":"Pages 22-25"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.ebcr.2017.11.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36041448","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lilit Mnatsakanyan , Sumeet Vadera , Christopher W. Ingalls , Jie Zheng , Mona Sazgar , Frank P. Hsu , Jack J. Lin
{"title":"Language recovery after epilepsy surgery of the Broca's area","authors":"Lilit Mnatsakanyan , Sumeet Vadera , Christopher W. Ingalls , Jie Zheng , Mona Sazgar , Frank P. Hsu , Jack J. Lin","doi":"10.1016/j.ebcr.2017.06.002","DOIUrl":"10.1016/j.ebcr.2017.06.002","url":null,"abstract":"<div><p>Epilepsy surgery is indicated in select patients with drug-resistant focal epilepsy. Seizure freedom or significant reduction of seizure burden without risking new neurological deficits is the expected goal of epilepsy surgery. Typically, when the seizure onset zone overlaps with eloquent cortex, patients are excluded from surgery.</p><p>We present a patient with drug-resistant frontal lobe epilepsy who underwent successful surgery with resection of Broca's area, primarily involving the pars triangularis (BA 45). We report transient expressive aphasia followed by recovery of speech. This case provides new insights into adult neuroplasticity of the language network.</p></div>","PeriodicalId":56365,"journal":{"name":"Epilepsy and Behavior Case Reports","volume":"9 ","pages":"Pages 42-45"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.ebcr.2017.06.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36041872","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of interictal dysphoric disorder comorbid with interictal psychosis: Part of the same spectrum or separate entities?","authors":"Susumu Morita , Go Taniguchi , Hidetaka Tamune , Yousuke Kumakura , Shinsuke Kondo , Kiyoto Kasai","doi":"10.1016/j.ebcr.2018.07.007","DOIUrl":"10.1016/j.ebcr.2018.07.007","url":null,"abstract":"<div><p>Depressive disorders in epilepsy often present characteristic clinical manifestations atypical in primary, endogenous depression. Here, we report a case of a 64-year-old woman with right mesial temporal lobe epilepsy, who complained of bizarre, antipsychotic-refractory cenesthetic hallucinations in her interictal phase, and was hospitalized after a suicide attempt. Detailed clinical observations revealed mood symptoms, which led to the diagnosis of interictal dysphoric disorder comorbid with interictal psychosis. Sertraline with low-dose aripiprazole markedly alleviated both depressive and psychotic symptoms. This case suggested that the two diagnostic entities may overlap and that depressive symptoms tend to be concurrent when concurring with psychosis, which hampers the appropriate choice of a treatment option.</p></div>","PeriodicalId":56365,"journal":{"name":"Epilepsy and Behavior Case Reports","volume":"10 ","pages":"Pages 102-105"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.ebcr.2018.07.007","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36467830","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Katie M. Kinnear, Nicole M. Warner, Alan M. Haltiner, Michael J. Doherty
{"title":"Continuous monitoring devices and seizure patterns by glucose, time and lateralized seizure onset","authors":"Katie M. Kinnear, Nicole M. Warner, Alan M. Haltiner, Michael J. Doherty","doi":"10.1016/j.ebcr.2018.03.001","DOIUrl":"10.1016/j.ebcr.2018.03.001","url":null,"abstract":"<div><h3>Objectives</h3><p>To investigate if glucose levels influence seizure patterns.</p></div><div><h3>Materials and methods</h3><p>In a patient with RNS/NeuroPace implanted bi-temporally and type 1 diabetes mellitus, seizure event times and onset locations were matched to continuous tissue glucose.</p></div><div><h3>Results</h3><p>Left focal seizure (LFS, n<!--> <!-->=<!--> <!-->22) glucoses averaged 169<!--> <!-->mg/dL, while right focal seizure (RFS, n<!--> <!-->=<!--> <!-->23) glucoses averaged 131<!--> <!-->mg/dL (p<!--> <!-->=<!--> <!-->0.03). LFS occurred at mean time 17:02 while RFS occurred at 04:23. LFS spread to the contralateral side (n<!--> <!-->=<!--> <!-->19) more than RFS (n<!--> <!-->=<!--> <!-->2).</p></div><div><h3>Conclusion</h3><p>Seizure onset laterality and spread vary with glucose and time of seizure.</p></div>","PeriodicalId":56365,"journal":{"name":"Epilepsy and Behavior Case Reports","volume":"10 ","pages":"Pages 65-70"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.ebcr.2018.03.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36367214","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nilika S. Singhal , Adam L. Numis , Morgan B. Lee , Edward F. Chang , Joseph E. Sullivan , Kurtis I. Auguste , Vikram R. Rao
{"title":"Responsive neurostimulation for treatment of pediatric drug-resistant epilepsy","authors":"Nilika S. Singhal , Adam L. Numis , Morgan B. Lee , Edward F. Chang , Joseph E. Sullivan , Kurtis I. Auguste , Vikram R. Rao","doi":"10.1016/j.ebcr.2018.02.002","DOIUrl":"10.1016/j.ebcr.2018.02.002","url":null,"abstract":"<div><p>Responsive neurostimulation for epilepsy involves an implanted device that delivers direct electrical brain stimulation in response to detection of incipient seizures. Responsive neurostimulation is a safe and effective treatment for adults with drug-resistant epilepsy, but although novel treatments are critically needed for younger patients, responsive neurostimulation is currently not approved for children with drug-resistant epilepsy. Here, we report a 16-year-old patient with seizures arising from eloquent cortex, who was successfully treated with responsive neurostimulation. This case highlights the potential utility of this therapy for pediatric patients and underscores the need for larger studies.</p></div>","PeriodicalId":56365,"journal":{"name":"Epilepsy and Behavior Case Reports","volume":"10 ","pages":"Pages 21-24"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.ebcr.2018.02.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36317160","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Epilepsy and concomitant obsessive–compulsive disorder","authors":"Jacob S. Bird , Emiy Shah , Paul Shotbolt","doi":"10.1016/j.ebcr.2018.07.001","DOIUrl":"10.1016/j.ebcr.2018.07.001","url":null,"abstract":"<div><p>People with epilepsy (PWE) often suffer psychiatric symptoms which can impact them more than seizures. Affective and psychotic disorders are well recognized as occurring more frequently in PWE than the general population. Less is known about obsessive–compulsive disorder (OCD) in PWE, despite it being as disabling and distressing. We sought to explore the association between epilepsy and OCD with casereports by identifying ten PWE and concomitant OCD. Demographics, seizure classification, neurological, surgical, psychiatric and psychological treatment as well as quality of life were examined. A detailed analysis was performed for three of them, to explore the lived-experience of patients with the two conditions. This is followed by a discussion of how treatment for co-morbid epilepsy and OCD can be appropriately tailored to be patient specific and provide the greatest potential for improvement.</p></div>","PeriodicalId":56365,"journal":{"name":"Epilepsy and Behavior Case Reports","volume":"10 ","pages":"Pages 106-110"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.ebcr.2018.07.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36538740","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Patrick Kwan , Scott Mintzer , Antonio Laurenza , Anna Patten , Karen Cartwright
{"title":"Evaluation of perampanel as monotherapy for focal seizures: Experience from open-label extension studies","authors":"Patrick Kwan , Scott Mintzer , Antonio Laurenza , Anna Patten , Karen Cartwright","doi":"10.1016/j.ebcr.2017.11.001","DOIUrl":"10.1016/j.ebcr.2017.11.001","url":null,"abstract":"<div><p>Perampanel, a selective, non-competitive α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antagonist, is approved for adjunctive treatment of focal seizures, with or without secondarily generalized seizures, and for primary generalized tonic–clonic seizures in patients with epilepsy aged ≥<!--> <!-->12<!--> <!-->years. Perampanel was recently approved for monotherapy use for focal seizures in the U.S.A. Anti-seizure drug monotherapy may be preferable to polytherapy, which is generally associated with increased toxicity, non-compliance, and cost. Here, we report cases where patients had converted to perampanel monotherapy during open-label extension (OLEx) portions of 9 Phase II and III studies.</p><p>Of 2245 patients who enrolled in the OLEx studies, we identified 7 patients with drug-resistant focal seizures who discontinued all non-perampanel anti-seizure drugs and were maintained on perampanel monotherapy for ≥<!--> <!-->91<!--> <!-->days until the end of data cut-off. Patients received perampanel monotherapy for up to 1099<!--> <!-->days (157<!--> <!-->weeks), most at a modal dose of 12<!--> <!-->mg. Seizure data were available for 6 patients, of whom 5 had a ≥<!--> <!-->90% reduction in overall seizure frequency between baseline and their last 13-week period of monotherapy (3 were seizure-free). Perampanel monotherapy was generally well tolerated and the safety profile during perampanel monotherapy was consistent with clinical and post-marketing experience in the adjunctive setting.</p><p>This analysis included a small proportion of patients with highly drug-resistant focal seizures who converted to monotherapy during OLEx studies. While these limited data are encouraging in suggesting that perampanel might be useful as a monotherapy, further studies are required to explore outcomes in a less drug-resistant population, where a larger proportion of patients might benefit from monotherapy.</p></div>","PeriodicalId":56365,"journal":{"name":"Epilepsy and Behavior Case Reports","volume":"9 ","pages":"Pages 1-5"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.ebcr.2017.11.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36054493","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Su-Ching Hu , Kun-Long Hung , Hui-Ju Chen , Wang-Tso Lee
{"title":"Seizure remission and improvement of neurological function in sialidosis with perampanel therapy","authors":"Su-Ching Hu , Kun-Long Hung , Hui-Ju Chen , Wang-Tso Lee","doi":"10.1016/j.ebcr.2018.02.005","DOIUrl":"10.1016/j.ebcr.2018.02.005","url":null,"abstract":"<div><p>A 15-year-old boy experienced myoclonic seizures for 3<!--> <!-->years. He initially had occasional myoclonus, gradually progressive ataxia, tremors, and psychomotor and speech regression developed. Eventually, he exhibited nearly continuous myoclonus. He received treatment of sodium valproate, levetiracetam, clobazam, and phenobarbital, without efficacy. A ketogenic diet also proved ineffective. Adjunctive therapy with 4<!--> <!-->mg/day of perampanel was started and was gradually titrated to 10<!--> <!-->mg/day. The remission of myoclonic seizures was achieved within one month. The patient's neurological and cognitive functions improved to a certain degree during the following 20<!--> <!-->months. Sialidosis was confirmed by the mutations of <em>NEU1</em> gene.</p></div>","PeriodicalId":56365,"journal":{"name":"Epilepsy and Behavior Case Reports","volume":"10 ","pages":"Pages 32-34"},"PeriodicalIF":0.0,"publicationDate":"2018-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.ebcr.2018.02.005","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36288343","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}