BACKGROUND: Refractory and super-refractory status epilepticus are medical emergencies that must be promptly treated in consideration of their high mortality and morbidity rate. Nevertheless, the available evidence of effective treatment of these conditions is scarce. Among novel antiseizure medications (ASMs), highly purified cannabidiol (hpCBD) has shown noteworthy efficacy in reducing seizures in Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS), and Tuberous Sclerosis Complex (TSC).
METHODS: Here, we present two cases of effective use of hpCBD in both refractory and super- refractory status epilepticus. The administration of the nasogastric tube permitted the resolution of status epilepticus without adverse events. At 6-month follow-up, both patients were on hpCBD treatment, which continued to be efficacious for treating seizures.
CONCLUSIONS: According to our experience, hpCBD should be taken into consideration as an add-on therapy of RSE and SRSE while also considering the possibility of maintaining this treatment during the follow-up of patients. However, more studies and real-world experiences are needed to better understand its effectiveness in this setting and the interaction with other ASMs.

BACKGROUND: Depending on the underlying etiology and epilepsy type, the burden of disease for patients with seizures can vary significantly. This analysis aimed to compare direct and indirect costs and quality of life (QoL) among adults with tuberous sclerosis complex (TSC) related with epilepsy, idiopathic generalized epilepsy (IGE), and focal epilepsy (FE) in Germany.
METHODS: Questionnaire responses from 92 patients with TSC and epilepsy were matched by age and gender, with responses from 92 patients with IGE and 92 patients with FE collected in independent studies. Comparisons were made across the main QoL components, direct costs (patient visits, medication usage, medical equipment, diagnostic procedures, ancillary treatments, and transport costs), indirect costs (employment, reduced working hours, missed days), and care level costs.
RESULTS: Across all three cohorts, mean total direct costs (TSC: €7602 [median €2620]; IGE: €1919 [median €446], P < 0.001; FE: €2598 [median €892], P < 0.001) and mean total indirect costs due to lost productivity over 3 months (TSC: €7185 [median €11,925]; IGE: €3599 [median €0], P < 0.001; FE: €5082 [median €2981], P = 0.03) were highest among patients with TSC. The proportion of patients with TSC who were unemployed (60%) was significantly larger than the proportions of patients with IGE (23%, P < 0.001) or FE (34%, P = P < 0.001) who were unemployed. Index scores for the EuroQuol Scale with 5 dimensions and 3 levels were significantly lower for patients with TSC (time-trade-off [TTO]: 0.705, visual analog scale [VAS]: 0.577) than for patients with IGE (TTO: 0.897, VAS: 0.813; P < 0.001) or FE (TTO: 0.879, VAS: 0.769; P < 0.001). Revised Epilepsy Stigma Scale scores were also significantly higher for patients with TSC (3.97) than for patients with IGE (1.48, P < 0.001) or FE (2.45, P < 0.001). Overall Quality of Life in Epilepsy Inventory-31 items scores was significantly lower among patients with TSC (57.7) and FE (57.6) than among patients with IGE (66.6, P = 0.004 in both comparisons). Significant differences between patients with TSC and IGE were also determined for Neurological Disorder Depression Inventory for Epilepsy (TSC: 13.1; IGE: 11.2, P = 0.009) and Liverpool Adverse Events Profile scores (TSC: 42.7; IGE: 37.5, P = 0.017) with higher score and worse results for TSC patients in both questionnaires.
CONCLUSIONS: This study is the first to compare patients with TSC, IGE, and FE in Germany and underlines the excessive QoL burden and both direct and indirect cost burdens experienced by patients with TSC.

In this case study, a 16-year-old male with Down syndrome (DS) faced persistent nocturnal seizures despite anti-seizure medications and treatment for concurrent hypothyroidism. Obstructive sleep apnea (OSA), a common issue in patients with Down syndrome, was revealed as a trigger of the seizures. The implementation of continuous positive airway pressure (CPAP) therapy along with medication adjustments led to a significant decrease in seizure frequency, highlighting the importance of a comprehensive approach to seizure management in patients with complex medical conditions.

Although more common than in the general population, seizures are an atypical manifestation of multiple sclerosis (MS) and their pathophysiology is not well understood. This study aims to examine the prevalence, clinical characteristics, brain imaging findings and course of epilepsy, presenting in patients with MS.
Observational retrospective study of MS patients evaluated at a single MS reference center in Buenos Aires, Argentina, between 2011 and 2022, focusing on those who developed epilepsy (EMS). Clinical, demographic, and prognostic factors were evaluated and compared to a control group of non-epileptic MS patients (NEMS). To analyze specific epilepsy characteristics, a second control group of patients with non-lesional focal epilepsy (FNLE) was also included.
Twenty-five patients (18 women), were diagnosed with epilepsy, corresponding to a prevalence of 1.95%. Comparison of brain imaging characteristics between EMS and NEMS patients showed brain atrophy (32% vs 6.1%, p<0.01), as well as cortical (26% vs 4%, p=0.03) and juxtacortical lesions (84% vs 55%, p=0.05), were more frequent in EMS patients. However, after multivariate analysis, cortical atrophy was the only variable linked to a significant increase in risk of epilepsy (OR 24, 95%CI=2.3-200, p<0.01). No significant differences in clinical characteristics, disease activity, disability levels, response to disease modified treatment (DMT) or lack of DMT efficacy were observed between MS patients with or without epilepsy. Most patients received anti-seizure medication (ASM), and seizure control was better in EMS than in FNLE patients (92% vs 58%, p=0.022) with no differences found in drug resistance. We did not find predictors of seizure recurrence in the population studied.
We observed a lower prevalence of epilepsy in this group of MS patients, compared to other reported cohorts. Although epilepsy seems to have a benign course in MS patients, cortical atrophy appears to be an important contributor to the development of secondary epilepsy in MS patients. Further investigations will be necessary to identify risk factors or biomarkers predicting increased epilepsy risk in MS.

UNASSIGNED: Preclinical studies in a mouse model have shown that SYNGAP1 haploinsufficiency results in an epilepsy phenotype with excessive GluA2-AMPA insertion specifically on the soma of fast-spiking parvalbumin-positive interneurons associated with significant dysfunction of cortical gamma homeostasis that was rescued by perampanel (PER), an AMPA receptor blocker. In this single case, we aimed to investigate the presence of dysregulated cortical gamma in a toddler with a pathogenic SYNGAP1 variant and report on the effect of low-dose PER on electroencephalogram (EEG) and clinical profile.
UNASSIGNED: Clinical data from physician\'s clinic notes; genetic testing reports; developmental scores from occupational therapy, physical therapy, speech and language therapy evaluations; and applied behavioral analysis reports were reviewed. Developmental assessments and EEG analysis were done pre- and post-PER.
UNASSIGNED: Clinically, the patient showed improvements in the developmental profile and sleep quality post-PER. EEG spectral power analysis in our patient revealed a loss of gamma power modulation with behavioral-state transitions similar to what was observed in Syngap1+/- mice. Furthermore, the administration of low-dose PER rescued the dysfunctional cortical gamma homeostasis, similar to the preclinical study. However, as in the epileptic mice, PER did not curb epileptiform discharges or clinical seizures.
UNASSIGNED: Similar to the Syngap1+/- mice, cortical gamma homeostasis was dysregulated in the patient. This dysfunction was rescued by PER. These encouraging results necessitate further validation of gamma dysregulation as a potential translational EEG biomarker in SYNAP1-DEE. Low-dose PER can be explored as a therapeutic option through clinical trials.

UNASSIGNED: Bilateral femoral neck fractures are rare among children. Although several case reports have been published, fractures caused by epilepsy attacks in children have not been reported in the literature. This is the first report of simultaneous bilateral femoral neck fractures in a pediatric patient with epilepsy convulsions.
UNASSIGNED: This is a case of a child with bilateral femoral neck fractures caused by epileptic seizures. A 13-year-old Japanese boy had an epileptic seizure and was admitted to our hospital. The patient complained of bilateral thigh pain. Plain radiography revealed a bilateral femoral neck fracture.
UNASSIGNED: The patient\'s simultaneous bilateral femoral neck fractures were successfully managed with closed reduction and internal fixation, a careful postoperative course, and ultrasound fracture therapy. Despite the delay in diagnosis, bone union was confirmed 6 months postoperatively. Pediatric bilateral femoral neck fractures without a history of trauma are rare and likely to be missed. This case was a teachable experience highlighting the importance of being vigilant about fractures in children with postepileptic seizures.

BACKGROUND: There is a bi-directional relationship between seizures and substance misuse, i.e., alcohol and recreational drugs. Seizures and substance misuse are recognised separately to influence increased emergency department (ED) admissions and early death. There is however no understanding of the cumulative influence of these matters on repeat ED attenders for seizures esp. as a third are likely to re-attend within the year. This case-control study compares the characteristics of people with substance misuse to those without substance misuse presenting recurrently with seizures to the ED.
METHODS: From a single ED serving a rural population in the Southwest of England, data of all people presenting more than once with a seizure over a 4-year period were examined. The diagnosis of alcohol or drug misuse, deaths, demographic characteristics, and service use were captured.
RESULTS: Of 450 repeat attenders, 95 had a recorded history of alcohol and/or drug problems. Those with substance misuse had double the mortality when adjusted for age and gender compared to those without. They were also more likely to be male, younger in age, have mental health issues, live in socially deprived neighborhoods, not take anti-seizure medications and not have had a specialist review in epilepsy services in the previous year. Nearly a quarter of those with addiction issues died in the 4-year period.
CONCLUSIONS: Service provision for this vulnerable group may need to be modelled along different lines to traditional approaches, such as an assertive outreach community-based service as provided for chronic psychiatric and addiction management.

Seizures triggered by skin application, inhalation or ingestion of over-the-counter medications containing eucalyptus oil are known. We report five children who suffered likewise. We made a systematic search for all reported cases and performed a pooled analysis to provide a comprehensive estimate of the type of seizures, their management and outcome. In 110 cases (49 children), inhalational use was the most predominant, generalised tonic-clonic (the commonest semiology) and levetiracetam was the most common anti-convulsant treatment used. Most cases had an uneventful recovery. Adults were less likely to have prolonged and multiple seizures, requiring intensive care or mechanical ventilation.