N. Zalmay , G. Nune , C.N. Heck , K.T. Dao , B.T. Ly , J. Ipe , C.Y. Liu , H.P. Kunhi Veedu
{"title":"Successful treatment of super-refractory focal status epilepticus: Surgery, vagus nerve stimulation, and botox for epilepsia partialis continua","authors":"N. Zalmay , G. Nune , C.N. Heck , K.T. Dao , B.T. Ly , J. Ipe , C.Y. Liu , H.P. Kunhi Veedu","doi":"10.1016/j.ebr.2025.100775","DOIUrl":"10.1016/j.ebr.2025.100775","url":null,"abstract":"<div><div>Super-refractory status epilepticus (SRSE) is a life-threatening condition characterized by persistent seizures lasting beyond seven days despite anesthetic therapy. Managing SRSE is challenging, particularly in cases resistant to conventional treatments. This case report presents a 41-year-old female with prolonged SRSE secondary to focal epilepsy, successfully treated with surgical resection, vagus nerve stimulation (VNS), and botulinum toxin (BoNT) therapy. The patient initially presented with left focal motor status epilepticus and a right frontal intracerebral hematoma. Despite an emergency craniotomy, her seizures persisted, leading to prolonged SRSE. After multiple failed sedation weaning attempts, a stepwise intervention approach was implemented consisting of an Electrocorticography (ECoG)-guided surgical resection of the residual epileptic focus, VNS implantation for long-term seizure modulation, BoNT therapy for residual epilepsia partialis continua (EPC). The combined approach of surgical resection and VNS implantation successfully terminated the status epilepticus. Residual Epilepsia Partialis Continua (EPC) was effectively managed with botulinum toxin therapy (BoNT). This case underscores the potential efficacy of combining surgical resection and VNS implantation in treating SRSE and highlights the beneficial effects of BoNT in managing residual EPC, offering a potential treatment pathway for similar refractory cases.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100775"},"PeriodicalIF":1.8,"publicationDate":"2025-05-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143923931","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kati Rantanen , Jenna Mäntylä , Eeva Kettunen , Annina Saunajoki , Kai Eriksson
{"title":"Academic competence and special educational needs as outcomes of early onset epilepsy: A population-based prospective follow-up study","authors":"Kati Rantanen , Jenna Mäntylä , Eeva Kettunen , Annina Saunajoki , Kai Eriksson","doi":"10.1016/j.ebr.2025.100777","DOIUrl":"10.1016/j.ebr.2025.100777","url":null,"abstract":"<div><div>Epilepsy in children is associated with significant academic challenges, particularly among those with early-onset seizures and comorbid intellectual disabilities (ID). This study aims to bridge gaps in existing research by examining long-term academic outcomes and special educational needs (SEN) in a cohort of children with early-onset epilepsy, including those with complicated epilepsy (CE) and major comorbidities. The study followed a population-based cohort of 64 children with epilepsy (CWE) aged 3–6 years, identified from the Pediatric Neurology Unit at Tampere University Hospital. Six years later, 43 children (67 %) participated in the follow-up. Academic competence was assessed using the Teacher Report Form (TRF), which also provided data on SEN. The findings revealed that 67 % of participants had low academic competence, with a significant proportion requiring special educational support. Children with CE had notably lower academic performance compared to those with uncomplicated epilepsy (UE). The need for SEN was higher among children with CE (81 %) than those with UE (24 %), with epilepsy type emerging as a significant predictor for SEN. Despite the small sample size and limitations in generalizability, the study underscores the pervasive impact of early-onset epilepsy on academic outcomes and highlights the necessity for ongoing educational support. Future research should focus on larger cohorts and explore the efficacy of tailored educational interventions to improve the academic performance and quality of life for CWE. Regular screening and collaboration with educators are essential to address the unique needs of these children effectively.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100777"},"PeriodicalIF":1.8,"publicationDate":"2025-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143918374","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Investigation of the relationship between 0.5–1200 Hz signal characteristics of cortical high-frequency oscillations and epileptogenicity through multivariate analysis","authors":"Takashi Shibata , Hiroki Tsuchiya , Mari Akiyama , Tomoyuki Akiyama , Masao Matsuhashi , Katsuhiro Kobayashi","doi":"10.1016/j.ebr.2025.100776","DOIUrl":"10.1016/j.ebr.2025.100776","url":null,"abstract":"<div><div>Fast ripples (FRs) (250–500 Hz) on the electroencephalogram (EEG) are closely related to epileptogenicity and are important to determine cortical regions resected in epilepsy surgery. However, FR-related epileptogenicity may be variable, and may depend on information associated with FRs. We enrolled nine epilepsy patients who had undergone intracranial 5 kHz-sampling-rate EEG for surgical treatment and had final Engel class I outcomes. Three electrodes were selected from each epileptogenic area (EA) and the unlikely EA (the region outside the EA) in each patient. Up to 100 candidate FRs were automatically detected from interictal nocturnal EEG at each of the selected electrodes and were visually reviewed independently by two researchers. Multivariate logistic regression analysis was performed using the frequency and log-power value of the corresponding FRs, presence of concurrent spike, ripple, very-high-frequency oscillations (vHFO)1 (500–600 Hz), and vHFO2 (600–1200 Hz), and whether the timing of the spectral peak of corresponding FRs was in the peak–trough or trough–peak transition of each slow activity (0.5–1, 1–2, 2–3, 3–4, and 4–8 Hz) as independent variables. Factors significantly related to epileptogenicity were FR power, the concurrent presence of spike and vHFO2, coupling with 0.5–1 and 1–2 Hz slow waves in the peak–trough transition, and coupling with 3–4 and 4–8 Hz slow waves in the trough–peak transition. Multifactorial analysis of FRs may increase their usefulness, potentially leading to improved treatment outcomes in epilepsy surgery.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100776"},"PeriodicalIF":1.8,"publicationDate":"2025-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143877527","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Ictal cold shiver caused by autoimmune limbic encephalitis: A case report and literature review","authors":"Yosuke Takeuchi , Teruaki Masuda , Hiroyuki Matsuta , Kenichi Yabuuchi , Nobuhiro Hata , Minoru Fujiki , Konen Obayashi , Etsuro Matsubara , Noriyuki Kimura","doi":"10.1016/j.ebr.2025.100773","DOIUrl":"10.1016/j.ebr.2025.100773","url":null,"abstract":"<div><div>Ictal cold shiver is a rare manifestation of seizures that originate in the temporal lobes; it is characterized by body trembling and sensations of cold. This study aims to investigate the clinical manifestations and etiological factors in patients with ictal cold shiver. We reviewed 59 cases of ictal cold shiver by using various keywords to search published original articles and case reports, and we described a new case of autoimmune limbic encephalitis. The median age at diagnosis was higher in patients with autoimmune encephalitis than in those with other etiologies (p = 0.007). Autoimmune encephalitis showed more frequent bilateral temporal lobe abnormalities on magnetic resonance imaging (MRI) (p = 0.027) and generalized or bilateral independent epileptiform discharges on electroencephalography (EEG) (p = 0.047). Anti-leucine-rich glioma-inactivated 1 antibodies were most commonly detected in patients with autoimmune encephalitis with ictal cold shiver (85.7 %). This study highlights the importance of considering autoimmune mechanisms in the presence of ictal cold shiver, especially in patients with bilateral temporal lobe lesions on MRI, generalized or bilateral independent epileptiform discharges on EEG, and older onset.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"30 ","pages":"Article 100773"},"PeriodicalIF":1.8,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143870192","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Eshita Sharma , Beatriz Westphalen Pomianoski , Rabbia Jabbar , Ayesha Ayesha , Yasmin Picanco Silva , Paweł Łajczak , Aisha Rizwan Ahmed , Oguz Kagan Sahin , Mir Wajid Majeed , Mohammed Raake , Walter Fagundes , Giovani Noll
{"title":"Responsive neurostimulation for patients with refractory mesial temporal lobe epilepsy: A systematic review and meta-analysis","authors":"Eshita Sharma , Beatriz Westphalen Pomianoski , Rabbia Jabbar , Ayesha Ayesha , Yasmin Picanco Silva , Paweł Łajczak , Aisha Rizwan Ahmed , Oguz Kagan Sahin , Mir Wajid Majeed , Mohammed Raake , Walter Fagundes , Giovani Noll","doi":"10.1016/j.ebr.2025.100774","DOIUrl":"10.1016/j.ebr.2025.100774","url":null,"abstract":"<div><div>Responsive neurostimulation (RNS) is a well-established adjuvant therapy for reducing seizure frequency in adults with medically refractory partial-onset seizures, particularly in individuals who are either not candidates for surgical resection or remain seizure-prone post-surgery. However, its effectiveness in patients with mesial temporal lobe epilepsy (MTLE) remains unclear. This meta-analysis evaluates the efficacy of RNS therapy in individuals with medically refractory MTLE. A systematic search of PubMed, Cochrane, and Scopus databases was conducted to identify eligible studies. Outcomes assessed included mean seizure frequency reduction, responder rate (proportion of patients achieving ≥50 % reduction in seizure frequency), and the proportion of patients achieving seizure freedom within six months of follow-up. Statistical analyses were performed using STATA. Seven observational studies involving 207 patients were included. RNS was associated with a mean seizure frequency reduction of 68.76 % (95 % CI 57.16–80.37 %; I<sup>2</sup> = 81.68 %), a responder rate of 67.58 % (95 % CI 46.51–88.66 %; I<sup>2</sup> = 94 %), and seizure freedom within six months in 28.94 % of patients (95 % CI 3.03–54.86 %; I<sup>2</sup> = 88 %). Moderate to high heterogeneity was observed across the studies. RNS may represent a viable therapeutic option for patients with MTLE, demonstrating substantial reductions in seizure frequency and a notable proportion of patients achieving seizure freedom. Additional studies are needed to confirm these findings and to explore the comparative efficacy and safety of RNS therapy in relation to other treatment options.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"30 ","pages":"Article 100774"},"PeriodicalIF":1.8,"publicationDate":"2025-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143870193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zachary Mills, James Thomas Houston, Ashley Thomas, Kelsey Shoenmeyer
{"title":"Cerebrotendinous Xanthomatosis: Novel EEG finding of Fixation-Off Sensitivity","authors":"Zachary Mills, James Thomas Houston, Ashley Thomas, Kelsey Shoenmeyer","doi":"10.1016/j.ebr.2025.100770","DOIUrl":"10.1016/j.ebr.2025.100770","url":null,"abstract":"<div><div>Cerebrotendinous Xanthomatosis (CTX) is a rare autosomal recessive condition resulting in accumulation of cholesterol and cholestanol due to disrupted bile synthesis. Affected tissues include brain, tendons, skin, bone, lungs, and eyes. We report a clinical case presenting with epilepsy, which has been described, however with a particular EEG appearance that appears novel with Fixation-Off Sensitivity (FOS). The patient’s EEG showed significant buildup of abnormal slowing and frontally predominant generalized epileptiform discharges when her eyes were closed, and in contrast essentially normal tracings while eyes were open, eventually showing electrographic evolution and generating a bilateral tonic-clonic seizure. Genetic testing confirmed the diagnosis of CTX, and CTX-specific treatment with chonodeoxycholic acid was initiated in addition to anti-seizure medication.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"30 ","pages":"Article 100770"},"PeriodicalIF":1.8,"publicationDate":"2025-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143859243","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Successful application of vagus nerve stimulation in super refractory status epilepticus associated with MERRF syndrome","authors":"Leyla Baysal , Sabrina Jobi , Simone Zimmermann , Ann-Kristin Helmers , Nils Gerd Margraf","doi":"10.1016/j.ebr.2025.100769","DOIUrl":"10.1016/j.ebr.2025.100769","url":null,"abstract":"<div><div>Myoclonic epilepsy with ragged red fibers (MERRF) syndrome is a rare mitochondrial disorder marked by myoclonus, ataxia, cognitive impairment, myopathy, and sensorineural hearing loss. Seizures in MERRF syndrome are often resistant to standard antiseizure medication. We present a unique case of an adult patient with MERRF syndrome, caused by the m.8344A > G point mutation, who developed super-refractory status epilepticus (SRSE), successfully managed by the initiation and rapid escalation of vagus nerve stimulation (VNS) therapy. Over a two-year follow-up period, the VNS therapy enabled a long-term control of generalized tonic-clonic seizures. A further status epilepticus has not yet occurred. VNS may therefore be a promising therapeutic option for managing SRSE in patients with mitochondrial epilepsy syndromes.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"30 ","pages":"Article 100769"},"PeriodicalIF":1.8,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143847441","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Evangeline A. Huis in 't Veld , Olga Braams , Willem M. Otte , Peter van Rijen , Kees P.J. Braun , Renske Schappin
{"title":"Exploring determinants of time to school re-entry after pediatric epilepsy surgery","authors":"Evangeline A. Huis in 't Veld , Olga Braams , Willem M. Otte , Peter van Rijen , Kees P.J. Braun , Renske Schappin","doi":"10.1016/j.ebr.2025.100771","DOIUrl":"10.1016/j.ebr.2025.100771","url":null,"abstract":"<div><div>After epilepsy surgery, it varies when children re-enter school. The aim of this study was to identify determinants for this variation. Parents of 21 school-attending children participated in semi-structured interviews during their child’s hospitalization for epilepsy surgery and one year afterward (based on the standard neuropsychological post-surgical follow-up). The mean time to school re-entry was 10.7 weeks (SD = 6.3). One child did not attend school after one year, whilst the fastest child resumed school 2 weeks after surgery. We performed univariable linear regression models with bootstrapped R<sup>2</sup> for all variables deemed theoretically or clinically relevant to school re-entry. We found that temporal surgery was significantly related to shorter time to school re-entry; and that longer hospitalization, and presurgical outpatient educational counseling were significantly related to longer time to school re-entry. In multivariable linear regression, these variables together predicted 57% of variance (bootstrapped) in time to school re-entry. In conclusion, our findings indicate that school re-entry varies considerably among children but can partly be explained by surgery related variables and the presence of counseling. Given the importance of school in children’s daily lives, we argue that school attendance should be stimulated by increasing professionals’ awareness of children’s school re-entry process.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"30 ","pages":"Article 100771"},"PeriodicalIF":1.8,"publicationDate":"2025-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143838023","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Before diagnosing stroke-like episodes in OPA1-related mitochondrial disorder, its imaging criteria must be met","authors":"Josef Finsterer","doi":"10.1016/j.ebr.2025.100772","DOIUrl":"10.1016/j.ebr.2025.100772","url":null,"abstract":"","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100772"},"PeriodicalIF":1.8,"publicationDate":"2025-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144491169","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lucas D'Andrea, Raphael Mosqueira, Alcenor C. Miranda Filho, Renato L. Marchetti
{"title":"A functional seizure case in Wilson’s disease","authors":"Lucas D'Andrea, Raphael Mosqueira, Alcenor C. Miranda Filho, Renato L. Marchetti","doi":"10.1016/j.ebr.2025.100768","DOIUrl":"10.1016/j.ebr.2025.100768","url":null,"abstract":"<div><div>Wilson’s disease (WD) is a rare disorder characterized by abnormal copper metabolism, leading to its accumulation in various tissues, particularly the brain and the liver. Psychiatric and neurological symptoms are common manifestations of WD. We present a case of a 22-year-old woman diagnosed with WD who exhibited neurological symptoms and experienced functional seizures (FS) that were misdiagnosed as epilepsy secondary to WD for almost two years. The patient’s history of childhood trauma and interpersonal difficulties underscored the complex interplay between organic and psychogenic factors contributing to FS development. This case highlights the diagnostic challenges associated with the neuropsychiatric manifestations of Wilson’s disease, as well as the complexities in differentiating functional seizures from epilepsy. It emphasizes the importance of comprehensive assessment and multidisciplinary care in optimizing patient outcomes.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"30 ","pages":"Article 100768"},"PeriodicalIF":1.8,"publicationDate":"2025-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143851671","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}