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Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) with high-frequency oscillations on scalp EEG: A case report
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-13 DOI: 10.1016/j.ebr.2025.100754
Keisuke Maeda , Himari Tsuboi , Nami Hosoda , Junichi Fukumoto , Shiho Fujita , Naohiro Ichino , Keisuke Osakabe , Keiko Sugimoto , Gen Furukawa , Naoko Ishihara
Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a rare mitochondrial disease with a prevalence of 16–18 per 100,000 persons. Most patients with MELAS develop epilepsy and require effective control of recurrent attacks. High-frequency oscillations (HFOs) on scalp EEG are transient bursts of EEG activity with frequencies beyond 80 Hz and are a promising biomarker for seizure control in epilepsy. However, reports on scalp HFOs are limited to some epilepsy syndromes. Herein, we report on the appearance of scalp HFOs in a pediatric patient with MELAS. The patient, a 13-year-old boy, presented with a stroke-like episode at age 9 years and was diagnosed with MELAS. The main symptom was visual disturbances, but epilepsia partialis continua (EPC) was also observed. Scalp EEG recordings were made six times: 8 days before the stroke-like episode (day –8 EEG), the day of appearance (day 1 EEG), and 4, 8, 10, and 100 days after the episode. Analysis of scalp HFOs showed that no scalp HFOs were detected in the day –8 EEG, whereas 1.20 scalp HFOs per minute were detected in the day 1 EEG at the appearance of the stroke-like episode with EPC. The scalp HFO detection rate decreased with the loss of EPC, and no scalp HFOs appeared on EEG, although visual disturbances continued to be observed. By contrast, epileptic discharges remained on EEG after EPC disappearance. Scalp HFOs have the potential to be a useful biomarker for reflecting epileptic seizure in patients with MELAS.
{"title":"Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) with high-frequency oscillations on scalp EEG: A case report","authors":"Keisuke Maeda ,&nbsp;Himari Tsuboi ,&nbsp;Nami Hosoda ,&nbsp;Junichi Fukumoto ,&nbsp;Shiho Fujita ,&nbsp;Naohiro Ichino ,&nbsp;Keisuke Osakabe ,&nbsp;Keiko Sugimoto ,&nbsp;Gen Furukawa ,&nbsp;Naoko Ishihara","doi":"10.1016/j.ebr.2025.100754","DOIUrl":"10.1016/j.ebr.2025.100754","url":null,"abstract":"<div><div>Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a rare mitochondrial disease with a prevalence of 16–18 per 100,000 persons. Most patients with MELAS develop epilepsy and require effective control of recurrent attacks. High-frequency oscillations (HFOs) on scalp EEG are transient bursts of EEG activity with frequencies beyond 80 Hz and are a promising biomarker for seizure control in epilepsy. However, reports on scalp HFOs are limited to some epilepsy syndromes. Herein, we report on the appearance of scalp HFOs in a pediatric patient with MELAS. The patient, a 13-year-old boy, presented with a stroke-like episode at age 9 years and was diagnosed with MELAS. The main symptom was visual disturbances, but epilepsia partialis continua (EPC) was also observed. Scalp EEG recordings were made six times: 8 days before the stroke-like episode (day –8 EEG), the day of appearance (day 1 EEG), and 4, 8, 10, and 100 days after the episode. Analysis of scalp HFOs showed that no scalp HFOs were detected in the day –8 EEG, whereas 1.20 scalp HFOs per minute were detected in the day 1 EEG at the appearance of the stroke-like episode with EPC. The scalp HFO detection rate decreased with the loss of EPC, and no scalp HFOs appeared on EEG, although visual disturbances continued to be observed. By contrast, epileptic discharges remained on EEG after EPC disappearance. Scalp HFOs have the potential to be a useful biomarker for reflecting epileptic seizure in patients with MELAS.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100754"},"PeriodicalIF":1.8,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143437586","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}
引用次数: 0
Encephalocele-associated temporal lobe refractory epilepsy: Report of two cases
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-08 DOI: 10.1016/j.ebr.2025.100752
María Zuluaga , Valeria Valencia-Cifuentes , María Alejandra Recio , Juan Camilo Márquez , Juan P. Fernández-Cubillos , Yuri Takeuchi
Encephaloceles are abnormal protrusions of brain tissue, meninges, and cerebrospinal fluid that result from defects in the skull base or cranial vault. These abnormalities can lead to seizure disorders and focal pharmacoresistant epilepsies. However, clinical suspicion and diagnosis are frequently delayed due to the significant challenges in interpreting initial imaging. Interpretation omissions can contribute to false diagnosis as non-lesional epilepsy in patients with encephalocele. This warrants appropriate imaging paradigms and careful interpretation. When clinical suspicion remains, further surgical exploration should be considered. Different treatment approaches, such as lesionectomy, temporal lobectomy, or invasive studies such as stereoelectroencephalography, may be employed. We describe two cases of adult-onset epilepsy associated with encephaloceles: a 28-year-old woman with drug-resistant epilepsy secondary to a right temporal encephalocele that was not initially diagnosed through neuroimaging, and a 43-year-old woman who presented with de novo focal status epilepticus attributed to a left temporal encephalocele, diagnosed during surgical exploration for a misdiagnosed neoplasm. Both cases were successfully treated surgically, with one-year follow-up free of new seizures.
{"title":"Encephalocele-associated temporal lobe refractory epilepsy: Report of two cases","authors":"María Zuluaga ,&nbsp;Valeria Valencia-Cifuentes ,&nbsp;María Alejandra Recio ,&nbsp;Juan Camilo Márquez ,&nbsp;Juan P. Fernández-Cubillos ,&nbsp;Yuri Takeuchi","doi":"10.1016/j.ebr.2025.100752","DOIUrl":"10.1016/j.ebr.2025.100752","url":null,"abstract":"<div><div>Encephaloceles are abnormal protrusions of brain tissue, meninges, and cerebrospinal fluid that result from defects in the skull base or cranial vault. These abnormalities can lead to seizure disorders and focal pharmacoresistant epilepsies. However, clinical suspicion and diagnosis are frequently delayed due to the significant challenges in interpreting initial imaging. Interpretation omissions can contribute to false diagnosis as non-lesional epilepsy in patients with encephalocele. This warrants appropriate imaging paradigms and careful interpretation. When clinical suspicion remains, further surgical exploration should be considered. Different treatment approaches, such as lesionectomy, temporal lobectomy, or invasive studies such as stereoelectroencephalography, may be employed. We describe two cases of adult-onset epilepsy associated with encephaloceles: a 28-year-old woman with drug-resistant epilepsy secondary to a right temporal encephalocele that was not initially diagnosed through neuroimaging, and a 43-year-old woman who presented with de novo focal status epilepticus attributed to a left temporal encephalocele, diagnosed during surgical exploration for a misdiagnosed neoplasm. Both cases were successfully treated surgically, with one-year follow-up free of new seizures.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100752"},"PeriodicalIF":1.8,"publicationDate":"2025-02-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143387210","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}
引用次数: 0
Gelastic spells in Angelman Syndrome, when laughter isn’t funny
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-08 DOI: 10.1016/j.ebr.2025.100751
Natasha Varughese , Femke Horn , Robert P. Carson
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.
{"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}
引用次数: 0
Ultrahigh-field imaging (7 Tesla) in DNET: Unmasking microstructural imaging characteristics – A case report
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-05 DOI: 10.1016/j.ebr.2025.100749
Marta Calvo-Imirizaldu , Daniele Botta , Margitta Seeck , Jan Novy , Jose Federico Ojeda Esparza , Aikaterini Fitsiori , Corrado Santarosa , Kevin Battistini , Karl-Olof Lövblad , Felix T. Kurz
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.
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.
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.
{"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}
引用次数: 0
Myoclonic status epilepticus with dystonia-like symptoms in patients with dementia: Report of two cases
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-04 DOI: 10.1016/j.ebr.2025.100750
Rosario V. Rossi, Rosanna Melis, Noemi Murdeu, Sara Lizzos, Maria Luigia Piras, Loretta Racis, Silvia Serusi, Maria Valeria Saddi
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.
{"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}
引用次数: 0
First experiences with multiple bilateral insertions of a newly developed microcatheter-compatible endovascular electroencephalogram electrode for humans with epilepsy
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-04 DOI: 10.1016/j.ebr.2025.100748
Yosuke Masuda , Ayataka Fujimoto , Hisayuki Hosoo , Kota Araki , Hiroki Ishida , Mitsuyo Nishimura , Aiki Marushima , Eiichi Ishikawa , Yuji Matsumaru
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.
{"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}
引用次数: 0
Painful Todd’s: Post-ictal painful hemiparesis as an identifier of insular epilepsy
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-02 DOI: 10.1016/j.ebr.2025.100747
Julian Larkin , Tudor Munteanu , Emma Dolan , Daniel J. Costello , Kieron Sweeney , Ronan Kilbride , Peter Widdess-Walsh
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.
{"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}
引用次数: 0
Antiseizure medications and their differing effects on cardiovascular risk
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-01 DOI: 10.1016/j.ebr.2025.100746
Aleena Abbasi , Bassil Abbasi , Scott Mintzer , Carla LoPinto-Khoury
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.
{"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}
引用次数: 0
Usefulness of perampanel as initial monotherapy in children with non-lesional focal epilepsy
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-01-30 DOI: 10.1016/j.ebr.2025.100743
Hideaki Kanemura , Yoshihiro Miyasato , Yutaro Tomi , Fumikazu Sano
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 (>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 < 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.
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引用次数: 0
Utility and safety of epilepsy monitoring unit in an inpatient psychiatric setting in Japan
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-01-29 DOI: 10.1016/j.ebr.2025.100744
Go Taniguchi , Mao Fujioka , Yumiko Okamura , Minako Miyagi , Kenichi Yano , Shinsuke Kondo , Kiyoto Kasai
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 >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.
{"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}
引用次数: 0
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Epilepsy and Behavior Reports
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