Epilepsy and Behavior Reports最新文献

{"title":"Gelastic spells in Angelman Syndrome, when laughter isn’t funny","authors":"Natasha Varughese ,&nbsp;Femke Horn ,&nbsp;Robert P. Carson","doi":"10.1016/j.ebr.2025.100751","DOIUrl":"10.1016/j.ebr.2025.100751","url":null,"abstract":"<div><div>Angelman syndrome (AS) is a neurodevelopmental syndrome characterized by people with a characteristic happy demeanor, impaired expressive communication, sleep disruptions, and a variety of paroxysmal events, including seizures and movement disorders. Herein, we report three cases, one child and two adults with AS, of gelastic (laugh-related) spells. The phenomenology in all three is most consistent with gelastic syncope. A fourth example demonstrating transient breath-holding while laughing is included for reference. We subsequently review the differential for gelastic spells in AS, including seizures and cataplexy, and expound on the importance of their identification in the context of risk factors for cardiac arrhythmia. This work adds gelastic syncope to the list of paroxysmal events in AS and may serve to inform providers and parents of this phenotype and provide guidance for subsequent evaluation and treatment if indicated.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100751"},"PeriodicalIF":1.8,"publicationDate":"2025-02-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143419189","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":"Ultrahigh-field imaging (7 Tesla) in DNET: Unmasking microstructural imaging characteristics – A case report","authors":"Marta Calvo-Imirizaldu ,&nbsp;Daniele Botta ,&nbsp;Margitta Seeck ,&nbsp;Jan Novy ,&nbsp;Jose Federico Ojeda Esparza ,&nbsp;Aikaterini Fitsiori ,&nbsp;Corrado Santarosa ,&nbsp;Kevin Battistini ,&nbsp;Karl-Olof Lövblad ,&nbsp;Felix T. Kurz","doi":"10.1016/j.ebr.2025.100749","DOIUrl":"10.1016/j.ebr.2025.100749","url":null,"abstract":"<div><div>Commercial ultrahigh-field 7 Tesla (T) MRI has been approved for clinical brain imaging, including applications in epilepsy and brain tumors. Increasing magnetic field strength offers significant advantages over lower-field MRI due to improved spatial resolution, signal-to-noise ratio, and contrast-to-noise ratio. These improvements provide better anatomical delineation and gray-white matter tissue-contrast differentiation.</div><div>We present a case of a presumed dysembryoplastic neuroepithelial tumor (DNET) imaged at 7 T MRI of the second generation, which revealed an unprecedented level of detail of the complex and intricate tumor architecture. Insights of its different components correlate closely with its known histopathological features. These tumors are unique among low-grade neoplasms due to their distinct clinical presentation, imaging features, and histopathological architecture. DNETs are rare, typically occurring in young patients with refractory epilepsy, and are classified by their well-defined histological subtypes. We review the various MRI patterns of DNET, which have been shown to correlate with histological subtypes and the extent of the epileptogenic zone.</div><div>Complete tumor resection is essential for long-term control and recurrence prevention, emphasizing the importance of precise preoperative visualization of the tumor and its surrounding tissue. In this case, 7 T images demonstrated superior lesion conspicuity and clearer boundaries, highlighting the advantages of ultrahigh-field MRI in defining the full extent of the lesion. Although 7 T MRI is not yet widely available, it has started to gain an important role in the management of epilepsy, particularly for cases requiring detailed structural analysis.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100749"},"PeriodicalIF":1.8,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143377715","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":"Myoclonic status epilepticus with dystonia-like symptoms in patients with dementia: Report of two cases","authors":"Rosario V. Rossi,&nbsp;Rosanna Melis,&nbsp;Noemi Murdeu,&nbsp;Sara Lizzos,&nbsp;Maria Luigia Piras,&nbsp;Loretta Racis,&nbsp;Silvia Serusi,&nbsp;Maria Valeria Saddi","doi":"10.1016/j.ebr.2025.100750","DOIUrl":"10.1016/j.ebr.2025.100750","url":null,"abstract":"<div><div>We report cases of two elderly women with dementia who presented with a new-onset seizure disorder characterized by subtle, rhythmic muscular contractions involving the buccolingual region and the four limbs, persistent jaw opening, and abnormal cervical posture that mimicked myoclonus-dystonia syndrome and oromandibular dystonia. The symptoms lasted several minutes to a few hours. Video-polygraphic recordings revealed an electromyographic (EMG) pattern of brief, shock-like muscular contractions consistent with myoclonus that correlated with a high-amplitude (70–90 µV), 11–14 Hertz, bilaterally symmetric electroencephalographic (EEG) rhythm over the frontocentral regions. A time-locked relationship between the frontocentral EEG activity and the EMG myoclonic potentials demonstrated the cortical origin of myoclonus and therefore the epileptic nature of the disorder, whereas the oromandibular and cervical dystonic-like postures suggested the pathogenic involvement of subcortical structures. The intravenous administration of diazepam suppressed the clinical symptoms and the EEG–EMG correlate of myoclonus. The clinical and neurophysiological findings illustrate a form of myoclonic status epilepticus (SE) with dystonia-like symptoms resulting from the functional involvement of cortical and subcortical structures. The manifestation of subtle, rhythmic myoclonus and dystonic-like postures in patients with atypical EEG patterns of SE may require challenging differential diagnoses with myoclonus-dystonia syndrome and oromandibular dystonia.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100750"},"PeriodicalIF":1.8,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143377713","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":"First experiences with multiple bilateral insertions of a newly developed microcatheter-compatible endovascular electroencephalogram electrode for humans with epilepsy","authors":"Yosuke Masuda ,&nbsp;Ayataka Fujimoto ,&nbsp;Hisayuki Hosoo ,&nbsp;Kota Araki ,&nbsp;Hiroki Ishida ,&nbsp;Mitsuyo Nishimura ,&nbsp;Aiki Marushima ,&nbsp;Eiichi Ishikawa ,&nbsp;Yuji Matsumaru","doi":"10.1016/j.ebr.2025.100748","DOIUrl":"10.1016/j.ebr.2025.100748","url":null,"abstract":"<div><div>Identifying epileptogenic zones non-invasively is challenging due to signal interference by the scalp and skull, necessitating invasive methods like subdural recordings and stereoelectroencephalography. Recent microcatheter advancements suggest that a microcatheter-compatible endovascular EEG (eEEG) device could overcome these barriers. We developed a thin, flexible eEEG electrode, the EP-01, for use with current microcatheters. The EP-01, comprising a platinum electrode and alloy wire coated with an electrically non-conductive polymer, was inserted via the jugular veins under local anesthesia. The EP-01 electrodes were planned to be placed in six locations: bilateral transverse sinuses, bilateral cavernous sinuses, and the anterior and posterior superior sagittal sinuses. We conducted a first-in-human study demonstrating the feasibility and efficacy of the EP-01electrodes in simultaneously recording intracranial EEG signals from multiple brain locations. The EP-01 electrodes were successfully placed as planned, except for one, without complications. Simultaneous eEEG and scalp EEG recordings were performed during a Wada test to evaluate efficacy and safety. The eEEG recorded alpha waves and slow-wave activity during propofol administration, corresponding to scalp EEG findings, with amplitudes 3–4 times higher. Post-procedural assessments confirmed cranial vessels’ patency and absence of complications. The EP-01 successfully recorded EEG signals at multiple locations in the human brain using an endovascular approach. Compared to scalp EEG, the present approach seems to have the potential to record higher-amplitude EEG. However, the study was limited to short-term recordings without epileptic discharges. Further investigations, including long-term placement, are thus needed for seizure recordings and safety evaluations.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100748"},"PeriodicalIF":1.8,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143444727","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":"Painful Todd’s: Post-ictal painful hemiparesis as an identifier of insular epilepsy","authors":"Julian Larkin ,&nbsp;Tudor Munteanu ,&nbsp;Emma Dolan ,&nbsp;Daniel J. Costello ,&nbsp;Kieron Sweeney ,&nbsp;Ronan Kilbride ,&nbsp;Peter Widdess-Walsh","doi":"10.1016/j.ebr.2025.100747","DOIUrl":"10.1016/j.ebr.2025.100747","url":null,"abstract":"<div><div>The insula can generate seizures which mimic frontal, temporal and parietal epilepsies making electroclinical localization difficult. We report the case of a twenty-one-year-old woman who presented with seizure semiology of a left-sided painful somatosensory aura, progressing to bilateral tonic posturing and complex manual automatisms. She described a painful sensation and weakness affecting her left side following the offset of a seizure, with the pain consistenly outlasting the weakness. This would last from hours to days depending on the severity and duration of the seizure. Stereo-electroencephalography (SEEG) demonstrated seizure onset in the limen of the right insula. Extra-operative stimulation of the insula reproduced the clinical symptoms. She underwent radiofrequency thermocoagulation (RFTC) which has resulted in a significant reduction in seizure frequency. This case report describes a lateralized painful Todd’s phenomenon as a feature of insular epilepsy confirmed by SEEG and extra-operative stimulation.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100747"},"PeriodicalIF":1.8,"publicationDate":"2025-02-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143369827","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":"Antiseizure medications and their differing effects on cardiovascular risk","authors":"Aleena Abbasi ,&nbsp;Bassil Abbasi ,&nbsp;Scott Mintzer ,&nbsp;Carla LoPinto-Khoury","doi":"10.1016/j.ebr.2025.100746","DOIUrl":"10.1016/j.ebr.2025.100746","url":null,"abstract":"<div><div>This review discusses the differing effects of enzyme-inducing and non-inducing antiseizure medications on cardiovascular risk and their implications for the management strategies of epilepsy patients. Traditional risk markers, including low density lipoprotein, high density lipoprotein and triglycerides, can be altered by both enzyme induction and inhibition. Other markers of vascular risk, including c-reactive protein, non-high-density lipoprotein and homocysteine, are affected by antiseizure medications, although adults and children may have different responses. The overall atherosclerotic risk picture is more complex due to indirect effects such as neuroendocrine function and the metabolic syndrome. Large scale data shows an evolving understanding of cardiovascular risk. Long term risks of enzyme inducing antiseizure medications and valproic acid are apparent when studies examine medications individually. Finally, effects of antiseizure medications on cardiac rhythm and possibly autonomic control are discussed with respect to their clinical relevance to the practicing clinician.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100746"},"PeriodicalIF":1.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143350635","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":"Usefulness of perampanel as initial monotherapy in children with non-lesional focal epilepsy","authors":"Hideaki Kanemura ,&nbsp;Yoshihiro Miyasato ,&nbsp;Yutaro Tomi ,&nbsp;Fumikazu Sano","doi":"10.1016/j.ebr.2025.100743","DOIUrl":"10.1016/j.ebr.2025.100743","url":null,"abstract":"<div><div>This study aimed to compare carbamazepine (CBZ) and perampanel (PER) in terms of the efficacy against clinical seizures and electroencephalographic abnormalities such as interictal epileptiform discharges (IEDs) and safety as initial monotherapy in children with non-lesional focal epilepsies. This retrospective review included participants recruited from among epilepsy outpatients treated at the authors’ hospital between January 01, 2000, and December 31, 2019 in the CBZ group and between January 01, 2020, and December 31, 2022 in the PER group. The inclusion criterion in both groups was ≥12 months of follow-up. Responders were identified as participants demonstrating complete disappearance (100 % reduction in seizures or IEDs) or response (&gt;50 % reduction in seizure frequency or IEDs). Safety primary outcome was defined as appearance rate of AEs. The study group comprised 247 participants treated with CBZ and 46 participants treated with PER. Total efficacy rate for clinical seizures was significantly higher with PER than with CBZ (p = 0.0148). Moreover, the rate of complete disappearance was significantly higher with PER than with CBZ (p = 0.0133). Total efficacy rate for IED was again significantly higher with PER than with CBZ (p &lt; 0.0001). The appearance of adverse events was significantly lower with PER than with CBZ (p = 0.023). PER may be useful as initial monotherapy in children with non-lesional focal epilepsies.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100743"},"PeriodicalIF":1.8,"publicationDate":"2025-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143129950","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":"Utility and safety of epilepsy monitoring unit in an inpatient psychiatric setting in Japan","authors":"Go Taniguchi ,&nbsp;Mao Fujioka ,&nbsp;Yumiko Okamura ,&nbsp;Minako Miyagi ,&nbsp;Kenichi Yano ,&nbsp;Shinsuke Kondo ,&nbsp;Kiyoto Kasai","doi":"10.1016/j.ebr.2025.100744","DOIUrl":"10.1016/j.ebr.2025.100744","url":null,"abstract":"<div><div>An epilepsy monitoring unit (EMU) with long-term video electroencephalographic monitoring (LTVEM) was launched in our psychiatric ward to facilitate close examination of patients with epilepsy and offer hospital experience to psychiatrists unfamiliar with epilepsy care in Japan. This study aimed to examine the usefulness and safety of the EMU in an inpatient psychiatric setting in Japan by retrospectively reviewing the clinical notes of EMU admission cases in the psychiatric ward of the University of Tokyo Hospital between August 2014 and March 2020. The usefulness of the EMU was evaluated in terms of 1) habitual seizure recordings, 2) change in diagnosis, 3) frequency of epileptic seizures, and 4) surgical cases after EMU admission, and 5) EMU safety. Habitual events were recorded during LTVEM in 69 % of patients. The diagnosis was changed post-EMU evaluation in 28 % of the patients admitted for differential diagnosis. Among 41 patients who received anti-seizure medications, seizure frequency improved in 22 (53 %), whereas 10 (24 %) became seizure-free, and seizures were reduced by &gt;50 % in 12 (29 %). Eleven patients eventually underwent surgery for epilepsy. Among the total 134 patients, 13 (10 %) experienced adverse events during EMU stay and 6 experienced falls, but none required prolonged hospitalization. Furthermore, three patients had panic attacks, but no post-ictal psychiatric symptoms were observed. Admission to an EMU in the psychiatric setting can facilitate accurate diagnosis and improve epilepsy management. Fall prevention measures should be implemented during and after LTVEM for safer EMU evaluations.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100744"},"PeriodicalIF":1.8,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143387209","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":"Alice in Wonderland Syndrome: Localising insights from right visual cortex stroke complicated by epilepsia partialis continua","authors":"Gashirai K. Mbizvo ,&nbsp;Viraj Bharambe ,&nbsp;Brython Hywel ,&nbsp;Shubhabrata Biswas ,&nbsp;Andrew J. Larner","doi":"10.1016/j.ebr.2025.100745","DOIUrl":"10.1016/j.ebr.2025.100745","url":null,"abstract":"<div><div>The Alice in Wonderland syndrome comprises visual and/or somaesthetic distortions, most often migrainous in origin. We present a case of Alice in Wonderland syndrome in association with a right posterior/visual cortex infarction with subsequent seizures including focal motor status (epilepsia partialis continua), the latter a hitherto unreported association. These pathophysiological changes were of right hemisphere origin, suggesting Alice in Wonderland syndrome, if present, might have localising value for seizure focus in the right hemisphere.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100745"},"PeriodicalIF":1.8,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143129968","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":"Effect of deep brain stimulation on the severity of seizures and the quality of life in patients with multifocal drug-resistant epilepsy in Iran: A pilot review of local experience","authors":"Amir Reza Bahadori ,&nbsp;Parisa Javadnia ,&nbsp;Afshan Davari ,&nbsp;Sajad Shafiee ,&nbsp;Sara Ranji ,&nbsp;Mehrdad Sheikhvatan ,&nbsp;Abbas Tafakhori","doi":"10.1016/j.ebr.2025.100742","DOIUrl":"10.1016/j.ebr.2025.100742","url":null,"abstract":"<div><div>This study investigates the impact of the anterior nucleus of the thalamus deep brain stimulation (ANT-DBS) on patients with drug-resistant epilepsy (DRE) in Iran, specifically focusing on its effects on seizure metrics, severity and its influence on quality of life over time. A cohort of eight patients with DRE in Iran who underwent ANT-DBS was evaluated. Pre-operative assessments included comprehensive documentation of seizure frequency, duration, severity scores, and the Quality of Life in Epilepsy Inventory (QOLIE-13). Each patient also underwent high-resolution imaging using a 1.5 Tesla MRI, with targeted electrode placement in the anterior thalamic area. Post-operative evaluations measured changes in seizure frequency, severity scores, duration, and quality of life indicators. All subjects presented with DRE, and the mean age of participants was 24.62 years. Post-operative data revealed significantly reduced seizure frequency, duration, and severity scores. Notably, this reduction was more pronounced at the 6-month follow-up than the 3-month assessment, indicating a progressive therapeutic effect. All patients demonstrated a response to ANT-DBS, with two individuals achieving seizure freedom. Additionally, there was a marked improvement in quality of life, particularly in the domains of energy/fatigue and social functioning. ANT-DBS has been established as a promising and safe therapeutic intervention for patients with DRE. In a cohort of DRE patients in Iran, the treatment demonstrated comparable efficacy in decreasing seizure frequency and severity and enhancing self-reported quality of life, consistent with findings reported in the existing literature. The therapeutic benefits of ANT-DBS appear to augment over time.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100742"},"PeriodicalIF":1.8,"publicationDate":"2025-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143129967","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}