Guidelines on epilepsy monitoring unit (EMU) standards have been recently published. We aimed to survey Canadian EMUs to describe the landscape of safety practices and compare these to the recommendations from the new guidelines.

A 34-item survey was created by compiling questions on EMU structure, patient monitoring, equipment, personnel, standardized protocol use, and use of injury prevention tools. The questionnaire was distributed online to 24 Canadian hospital centers performing video-EEG monitoring (VEM) in EMUs. Responses were tabulated and descriptively summarized.

In total, 26 EMUs responded (100% response rate), 50% of which were adult EMUs. EMUs were on average active for 23.4 years and had on average 3.6 beds. About 81% of respondents reported having a dedicated area for VEM, and 65% reported having designated EMU beds. Although a video monitoring station was available in 96% of EMUs, only 48% of EMUs provided continuous observation of patients (video and/or physical). A total of 65% of EMUs employed continuous heart monitoring. The technologist-to-patient ratio was 1:1–2 in 52% of EMUs during the day. No technologist supervision was most often reported in the evening and at night. Nurse-to-EMU-patient ratio was mostly 1:1–4 independent of the time of day. Consent forms were required before admission in 27% of EMUs.

Canadian EMUs performed decently in terms of there being dedicated space for VEM, continuous heart monitoring, and adequate nurse-to-patient ratios. Other practices were quite variable, and adjustments should be made on a case-by-case basis to adhere to the latest guidelines.

There is limited data on the utility, yield, and cost efficiency of genetic testing in adults with epilepsy. We aimed to describe the yield and utility of genetic panels in our adult epilepsy clinic.

We performed a retrospective, cross-sectional study of all patients followed by an epileptologist at a Canadian tertiary care centre’s epilepsy clinic between January 2016 and August 2021 for whom a genetic panel was ordered. A panel was generally ordered when the etiology was unknown or in the presence of a malformation of cortical development. We determined the yield of panel positivity and of confirmed genetic diagnoses. We also estimated the proportion of these diagnoses that were clinically actionable.

In total, 164 panels were ordered in 164 patients. Most had refractory epilepsy (80%), and few had comorbid intellectual disability (10%) or a positive family history of epilepsy (11%). The yield of panel positivity was 11%. Panel results were uncertain 49% of the time and negative 40% of the time. Genetic diagnoses were confirmed in 7 (4.3%) patients. These genetic conditions involved the following genes: SCARB2, DEPDC5, PCDH19, LGI1, SCN1A, MT-TL1, and CHRNA7. Of the seven genetic diagnoses, 5 (71%) were evaluated to be clinically actionable.

We report a lower diagnostic yield for genetic panels in adults with epilepsy than what has so far been reported. Although the field of the genetics of epilepsy is a fast-moving one and more data is required, our findings suggest that guidelines for genetic testing in adults are warranted.

Caring for women with epilepsy (WWE) during pregnancy poses unique challenges. We conducted an audit of the care our epilepsy clinic provided to pregnant WWE.

We performed a retrospective study on all pregnancies followed by an epileptologist at a Canadian tertiary care centre’s epilepsy clinic between January 2003 and March 2021. Among 81 pregnancies in 53 patients, 72 pregnancies in 50 patients were analyzed to determine patient-related, follow-up-related, antiseizure-medication-related, and child-related pregnancy characteristics. Univariate analyses were performed to explore if these characteristics were associated with disabling seizure occurrence during pregnancy.

Most pregnancies were intended (72%) and occurred in women who used folic acid pre-pregnancy (76%) and who followed recommended blood tests for antiseizure medication (ASM) levels (71%). In 49% of pregnancies, ASM dosage was modified; 53% of these modifications were made in response to ASM blood levels. Most often used ASMs were lamotrigine (43%), followed by carbamazepine (32%) and levetiracetam (13%). One child was born with a thyroglossal duct cyst; our congenital malformation rate was thus 2%. Disabling seizures occurred in 24% of pregnancies. Exploratory analyses suggested that disabling seizure occurrence during pregnancy was associated with younger patient age (p = 0.018), higher number of ASMs used during pregnancy (p = 0.048), lamotrigine usage in polytherapy (p = 0.008), and disabling seizure occurrence pre-pregnancy (p = 0.027).

This Canadian audit provides an in-depth description of pregnancies benefiting from specialized epilepsy care. Our results suggest an association between disabling seizure occurrence during pregnancy and lamotrigine usage in polytherapy that warrants further evaluation.

Intervention time (IT) in response to seizures and adverse events (AEs) have emerged as key elements in epilepsy monitoring unit (EMU) management. We performed an audit of our EMU, focusing on IT and AEs.

We performed a retrospective study on all clinical seizures of admissions over a 1-year period at our Canadian academic tertiary care center’s EMU. This EMU was divided in two subunits: a daytime three-bed epilepsy department subunit (EDU) supervised by EEG technicians and a three-bed neurology ward subunit (NWU) equipped with video-EEG where patients were transferred to for nights and weekends, under nursing supervision. Among 124 admissions, 58 were analyzed. A total of 1293 seizures were reviewed to determine intervention occurrence, IT, and AE occurrence. Seizures occurring when the staff was present at bedside at seizure onset were analyzed separately.

Median IT was 21.0 (11.0–40.8) s. The EDU, bilateral tonic–clonic seizures (BTCS), and the presence of a warning signal were associated with increased odds of an intervention taking place. The NWU, BTCS, and seizure rank (seizures were chronologically ordered by the patient for each subunit) were associated with longer ITs. Bedside staff presence rate was higher in the EDU than in the NWU (p < 0.001). AEs occurred in 19% of admissions, with no difference between subunits. AEs were more frequent in BTCS than in other seizure types (p = 0.001).

This study suggests that close monitoring by trained staff members dedicated to EMU patients is key to optimize safety. AE rate was high, warranting corrective measures.

Our purpose was to determine the role of CHRNA4 and CHRNB2 in insular epilepsy.

We identified two patients with drug-resistant predominantly sleep-related hypermotor seizures, one harboring a heterozygous missense variant (c.77C>T; p. Thr26Met) in the CHRNB2 gene and the other a heterozygous missense variant (c.1079G>A; p. Arg360Gln) in the CHRNA4 gene. The patients underwent electrophysiological and neuroimaging studies, and we performed functional characterization of the p. Thr26Met (c.77C>T) in the CHRNB2 gene.

We localized the epileptic foci to the left insula in the first case (now seizure-free following epilepsy surgery) and to both insulae in the second case. Based on tools predicting the possible impact of amino acid substitutions on the structure and function of proteins (sorting intolerant from tolerant and PolyPhen-2), variants identified in this report could be deleterious. Functional expression in human cell lines of α4β2 (wild-type), α4β2-Thr26Met (homozygote), and α4β2/β2-Thr26Met (heterozygote) nicotinic acetylcholine receptors revealed that the mutant subunit led to significantly higher whole-cell nicotinic currents. This feature was observed in both homo- and heterozygous conditions and was not accompanied by major alterations of the current reversal potential or the shape of the concentration-response relation.

This study suggests that variants in CHRNB2 and CHRNA4, initially linked to autosomal dominant nocturnal frontal lobe epilepsy, are also found in patients with predominantly sleep-related insular epilepsy. Although the reported variants should be considered of unknown clinical significance for the moment, identification of additional similar cases and further functional studies could eventually strengthen this association.

To ensure the overall quality of our electroencephalogram (EEG) laboratory, we decided to perform an audit of EEGs interpreted at our institution, focusing initially on EEGs reporting temporal abnormalities.

Reports of all EEGs performed between January 1st and June 30th, 2006 were reviewed in order to identify tracings mentioning abnormalities in the temporal regions. These records were then independently reviewed by two epileptologists on two distinct occasions, separated by an interval of at least six months. If the recording was considered normal after this process, the cause for misinterpretation was identified and the patient's chart was reviewed to determine if he was epileptic or not based on available evidence until June 2009.

In the first half of 2006,143 out of 773 EEGs mentioned temporal abnormalities (18.5%). In general, intra- and interrater agreement ratios between our two epileptologists were moderate to substantial for normality, presence of epileptic activity and presence of slowing. Forty-five recordings (31.5%) were reported as normal independently by them on two distinct sittings six months apart. The most common causes for misinterpretation were the presence of benign epileptiform variants, normal sharply contoured patterns of somnolence or hyperventilation. Chart review confirmed that most were non-epileptic patients (60% non-epileptic, 27% epileptic, 13% unknown).

Moderate to substantial intra- and interrater agreement as well as frequent misinterpretation of physiological variants indicate that some corrective measures need to be implemented to improve the consistency of EEG interpretation amongst our group of electroencephalographers.

Retrospective study assessing the efficacy and tolerability of vagus nerve stimulation (VNS) for the treatment of refractory epilepsy at Notre-Dame Hospital.

Chart review of all adult epileptic patients treated by VNS with ≥ 1 year follow-up. Responders were defined as patients with ≥ 50% reduction of baseline seizure frequency.

Thirty-four patients (14M; mean age = 29.9 yrs) received a VNS. Sub-pectoralis implantation (n = 25) was more frequent than subcutaneous (n = 9). Most patients suffered from intractable partial epilepsy. After 6 months, 12 months, 24 months, and 36 months, 14/34 patients (41%), 16/34 patients (47%), 17/30 patients (57%) and 12/20 patients (60%) respectively were responders. Two patients (6%) became seizure-free. Complications related to implantation were minor: eight cases of limited cervical hypoesthesia, two minor scar infections and one Horner syndrome. Adverse events (voice hoarseness, throat paresthesia, coughing) related to stimulation were generally mild and tended to wane over time. However, a reduction in seizure frequency did not translate into a reduction in medication, as only 9% of responders had less antiepileptic medication at last follow-up compared to baseline.

VNS as practiced at Notre-Dame hospital is an efficacious and safe treatment for refractory epilepsy. Quotas allotted to epilepsy centers in the province of Quebec should be lifted or increased to allow more patients to benefit from this therapeutic device.