Christopher Saouda, Yamane Makke, Helen Edelberg, Mohamad Z. Koubeissi
{"title":"Rash and edema shortly after initial exposure to low dose cenobamate","authors":"Christopher Saouda, Yamane Makke, Helen Edelberg, Mohamad Z. Koubeissi","doi":"10.1016/j.ebr.2025.100800","DOIUrl":"10.1016/j.ebr.2025.100800","url":null,"abstract":"<div><div>Cenobamate (CNB) is an antiseizure medication (ASM) approved for the treatment of focal epilepsy. Rash and edema are uncommon adverse effects of this medication. We report two patients with medically refractory epilepsy (MRE) who received CNB and developed chest and facial rash and edema after the initial dose of 12.5 mg/d that prompted discontinuation of the medication with ensuing resolution of the rash and edema. Rechallenging with CNB resulted in similar reactions. Our report aims at increasing awareness of these reactions to CNB.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100800"},"PeriodicalIF":1.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144502037","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":"2025-09-01","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}
Andrew Zillgitt , David E Burdette , Atheel Yako , Revati Rashingkar , Ashleigh Terrell , Sydney Jacobs , Michael D Staudt
{"title":"Synergistic seizure reduction in patient with persistently elevated N-desmethylclobazam levels, CYP450 genetic polymorphism, and responsive neurostimulator targeting centromedian nuclei of bilateral thalami","authors":"Andrew Zillgitt , David E Burdette , Atheel Yako , Revati Rashingkar , Ashleigh Terrell , Sydney Jacobs , Michael D Staudt","doi":"10.1016/j.ebr.2025.100799","DOIUrl":"10.1016/j.ebr.2025.100799","url":null,"abstract":"<div><div>Clobazam (CLB) and cenobamate (CNB) are commonly used antiseizure medications (ASMs) in the treatment of patients with drug-resistant epilepsy (DRE). However, concomitant use of these two ASMs may lead to significant treatment-related adverse events (TRAE). Furthermore, these TRAE may be exacerbated in individuals with genetic polymorphisms involving the P450 system. In patients with DRE, epilepsy surgery, including neuromodulation, may lead to improved seizure control and a reduction in systemic TRAE from ASMs. This case report describes a patient with drug-resistant idiopathic generalized epilepsy (IGE) who experienced persistent excessive somnolence correlated with elevated N-desmethylclobazam (N-CLB) levels. Pharmacogenetic testing revealed poor metabolism of CYP2C19, and N-CLB levels remained elevated and detectable for nearly one year after the discontinuation of treatment with CLB and CNB. Responsive neurostimulator (RNS) implantation within the bilateral centromedian nuclei (CMN) of the thalamus resulted in seizure freedom until N-CLB levels fell, after which there was an 83–93 % reduction in the frequency of generalized tonic-clonic seizures (GTC).</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100799"},"PeriodicalIF":1.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144680422","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":"2025-09-01","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}
Rishi Katragadda , Kyung Eun Paik , D. Dilara Ertenu , Ahmad Marashly , Jay A. Salpekar , Aaron Hauptman
{"title":"Association between Dravet syndrome and Catatonia: a case report","authors":"Rishi Katragadda , Kyung Eun Paik , D. Dilara Ertenu , Ahmad Marashly , Jay A. Salpekar , Aaron Hauptman","doi":"10.1016/j.ebr.2025.100785","DOIUrl":"10.1016/j.ebr.2025.100785","url":null,"abstract":"<div><div>Catatonia is a neurobehavioral and motor syndrome that can occur in persons with epilepsy (PWE), though it is rarely described in individuals with developmental and genetic epilepsies such as Dravet syndrome. This case report describes a young adult with Dravet syndrome who developed catatonia after improving seizure control. In this report we explore forced normalization (FN) as a potential mediating mechanism. An 18-year-old male with Dravet syndrome experienced significant seizure reduction after zonisamide was added to his antiseizure regimen. Within two weeks, he developed catatonic features, including mutism, catalepsy, and psychomotor retardation. Bush Francis Catatonia Rating Scale (BFCRS) scores ranged from 17 to 22. Catatonia improved with lorazepam, though seizure frequency increased after zonisamide taper. He later experienced a decline in both neurological and psychiatric function following status epilepticus. EEG was not performed at the time of symptom onset, which limits the ability to diagnose this patient with FN. However, the clinical criteria for FN were partially met, and the timing of zonisamide initiation and catatonia emergence supports its consideration. Non-convulsive status epilepticus (NCSE) remains a plausible alternative mechanism for the catatonia seen in this patient, particularly in those with developmental encephalopathies. This case demonstrates an interesting temporal relationship between improved seizure control and emergence of catatonia in a patient with genetic epilepsy. Further research is required to clarify the relationship between catatonia and FN in epilepsy.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100785"},"PeriodicalIF":1.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144365606","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}
Michelle Zachman , Lixin Gong , Lingling An , Mark B. Halvorsen
{"title":"Dissolution and stability of vigabatrin powder in water, fruit juice, milk, and infant formula","authors":"Michelle Zachman , Lixin Gong , Lingling An , Mark B. Halvorsen","doi":"10.1016/j.ebr.2025.100816","DOIUrl":"10.1016/j.ebr.2025.100816","url":null,"abstract":"<div><div>The prescribing information for the antiseizure medication Vigadrone® (vigabatrin) Powder for Oral Solution advises only reconstituting the powder in water prior to administration and discarding unused solution. However, caregivers may seek to reconstitute vigabatrin in a pediatric patient’s preferred liquid rather than water to ease administration. We performed an in-use dissolution and stability study of vigabatrin powder reconstituted in 4 liquids—water, apple juice, whole milk, and infant formula—with testing performed 0, 24, and 48 h after preparation with storage at 2–8 °C in a capped, glass container. Vigabatrin reconstituted in each liquid was clear and free of visible particulates compared with matched drug-free liquid controls. In subsequent analyses using US Pharmacopeia (USP) procedures, high-performance liquid chromatography spectra and chromatograms for each sample solution matched the vigabatrin reference standard. At all time points, all sample solutions met USP-acceptance criteria of 95.0–105.0 % for product stability and USP-acceptance criteria for related substances/organic impurities testing for total impurities. Together, these results indicate that vigabatrin powder reconstituted in liquids other than water was stable for up to 48 h of refrigeration. This study provides useful information to help healthcare providers address real-world medication questions from caregivers.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100816"},"PeriodicalIF":1.5,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144829487","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}
Tal Benoliel , Oshrit Arviv , Diya Doufish , Netaniel Rein , Yuval Harpaz , Evgeny Tsizin , Michal Balberg , Sami Heymann , Zvi Israel , Mordekhay Medvedovsky , Dana Ekstein
{"title":"Depth-to-scalp spatiotemporal dynamics for stereo-EEG","authors":"Tal Benoliel , Oshrit Arviv , Diya Doufish , Netaniel Rein , Yuval Harpaz , Evgeny Tsizin , Michal Balberg , Sami Heymann , Zvi Israel , Mordekhay Medvedovsky , Dana Ekstein","doi":"10.1016/j.ebr.2025.100784","DOIUrl":"10.1016/j.ebr.2025.100784","url":null,"abstract":"<div><div>The data obtained from stereo-elecroencephalography (SEEG) in patients with focal epilepsy are crucial for defining the epileptogenic zone and achieving successful resection, but suboptimal electrode placement impairs SEEG results. We demonstrate an approach for concurrent scalp and depth EEG analysis from one patient with successful intracranial workup and one in whom the seizure onset zone was unsampled by SEEG. Intracranial epileptiform discharges were identified and clustered, their scalp correlates were averaged, and electric source imaging (ESI) was applied to the resulting averaged scalp potential – depth-to-scalp ESI (dsESI). We found temporal differences between intracranial and scalp peaks, as well as variations in averaged scalp spikes morphology and propagation, expressed by their amplitudes and width, and by their jitter across involved electrodes. Put together with the relative degree of focality and location of the averaged scalp spikes’ ESI on the cortex, these data could differentiate onset from propagation of interictal activity and identify unexplored nodes in the epileptic network. Our novel analysis highlights the importance of temporal, and not just spatial, spike dynamics within the epileptic network, may be used to validate depth electrode placement and aid in understanding the epileptic network.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100784"},"PeriodicalIF":1.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144470595","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}
Austin Sponaugle , Rebecca S. Stainman , Christopher M. Carosella
{"title":"Reduced VNS settings paradoxically decreases seizure burden in a patient following resolution of sleep disordered breathing","authors":"Austin Sponaugle , Rebecca S. Stainman , Christopher M. Carosella","doi":"10.1016/j.ebr.2025.100778","DOIUrl":"10.1016/j.ebr.2025.100778","url":null,"abstract":"<div><div>Vagus nerve stimulation (VNS) can be a highly effective treatment option for patients with drug resistant epilepsy. Notably, VNS has demonstrated side effects including a unique form of sleep disordered breathing known as vagus nerve stimulator associated sleep disordered breathing (VaS). However, the ways in which VaS interacts with seizure frequency is unknown. We report a case of a 28-year-old woman who presented to our department with complaints of worsening sleep quality 3 years following VNS implantation. Upon polysomnographic (PSG) evaluation, it was discovered that she suffered from VaS. The patient’s VNS output current was then down titrated during a subsequent PSG resulting in resolution of her VaS. Interestingly, despite downward titration of her nighttime VNS output current, the patient demonstrated a prolonged seizure free period, highlighting the complex interactions between VNS, VaS and seizure frequency. In cases where patients present with both epilepsy and VaS, physicians should be aware of this complex relationship. Further, this case highlights that down titration of VNS settings may result in not just improvement in VaS but may also result in improvement in seizure frequency.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100778"},"PeriodicalIF":1.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143929448","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}
Samwel Sylvester Msigwa , Suluma Aslan , Elizabeth Mareale , Mercy Bingileki
{"title":"Coexistence of epilepsy with eyelid myoclonia, schizoaffective disorder, and cavum septi pellucidi et vergae: A case report","authors":"Samwel Sylvester Msigwa , Suluma Aslan , Elizabeth Mareale , Mercy Bingileki","doi":"10.1016/j.ebr.2025.100786","DOIUrl":"10.1016/j.ebr.2025.100786","url":null,"abstract":"<div><div>We report the first case of a 14-year-old boy presenting with the rare co-occurrence of cavum septi pellucidi et vergae (CSPV), epilepsy with eyelid myoclonia (EEM), and schizoaffective disorder (SAD). The patient initially presented with a one-month history of abnormal eye movements, including continuous blinking and upward rolling of the eyeballs, which occurred predominantly at night. There was no alteration in consciousness. A detailed history revealed that the patient had experienced mood disturbances, delusional beliefs, auditory and visual hallucinations, and significant behavioral dysregulation for one year. These symptoms had been partially managed with haloperidol. Magnetic resonance imaging (MRI) confirmed the presence of CSPV. At the same time, electroencephalography (EEG) during intermittent photic stimulation demonstrated brief generalized epileptiform discharges triggered by eye closure, consistent with a diagnosis of EEM. The simultaneous presence of psychotic and affective symptoms met the diagnostic criteria for SAD. The patient was treated with sodium valproate in addition to his existing low-dose haloperidol regimen. This led to the complete resolution of seizures and psycho-affective symptoms at one- and three-month follow-ups. However, a decline in academic performance was noted at the one-year follow-up. In resource-limited settings without access to genetic or autoimmune tests, care was guided by practical adaptations rather than standard protocols. This case highlights a potential neurodevelopmental link between CSPV and epileptic and psychiatric manifestations, underscores the value of neuroimaging and EEG in pediatric neuropsychiatric overlap, and calls for research into mechanisms connecting midline brain anomalies with complex neuropsychiatric disorders.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100786"},"PeriodicalIF":1.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144320785","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}
Rowan Pentz , Kevin Jones , Ronit Mesterman , Rajesh RamachandranNair , Hiroshi Otsubo , Ayako Ochi , Ivanna Yau , Elizabeth J Donner , George M Ibrahim , Puneet Jain , Robyn Whitney
{"title":"Brain tumours as an unrecognized etiology of infantile epileptic spasms syndrome (IESS): The role of resective epilepsy surgery","authors":"Rowan Pentz , Kevin Jones , Ronit Mesterman , Rajesh RamachandranNair , Hiroshi Otsubo , Ayako Ochi , Ivanna Yau , Elizabeth J Donner , George M Ibrahim , Puneet Jain , Robyn Whitney","doi":"10.1016/j.ebr.2025.100802","DOIUrl":"10.1016/j.ebr.2025.100802","url":null,"abstract":"<div><div>Brain tumours are a rare cause of infantile epileptic spasm syndrome (IESS). Epilepsy surgery may be utilized in refractory IESS due to tumours, although reports are limited. We report an illustrative case of IESS which resolved after tumour resection and performed a literature review of all reported cases of brain tumours causing IESS with hypsarrhythmia. We present a 10-month-old girl with drug-resistant symmetric epileptic spasms (ES). Initial EEG showed symmetric hypsarrhythmia and brain MRI demonstrated a left temporal lobe tumour. Despite non-focal clinical and EEG findings, she underwent tumour resection at 21 months (ganglioglioma). She subsequently had resolution of ES and hypsarrhythmia and developmental gains. Twenty-seven published cases of brain tumours and IESS were reviewed. ES were refractory to medical management in 74 % of reported cases (17/23). ES were symmetric in 17/21 (81 %) and asymmetric in 4/21 (19 %) specified cases. Hypsarrhythmia pattern was asymmetric in 10/20 (50 %) specified cases. Of 21 surviving patients who underwent surgery, 13 (62 %) were seizure free at follow-up and only 1 (5 %) had no improvement. Developmental outcomes were available in 19/21 (90 %) surviving surgical patients and 10/19 had improvements (52 %); 6/19 had normalized development (32 %). Various tumour types were reported and a temporal or frontal location was most common. Brain tumours can cause IESS with or without clinical or EEG focality and are amenable to surgical treatment. We emphasize that the absence of focal signs should not delay surgical assessment for patients with refractory IESS and brain tumours.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"31 ","pages":"Article 100802"},"PeriodicalIF":1.8,"publicationDate":"2025-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144623711","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}