Unilateral abdominal clonic jerking as an epileptic phenomenon.

Unilateral abdominal wall clonic seizures are a rare manifestation of epilepsy. We report three cases of focal aware seizures manifesting as unilateral abdominal clonic motor movements. Standard EEG for patients with focal motor abdominal seizures is often unrevealing, which can make the diagnosis difficult. We report the first case of intracranial EEG in the diagnosis of a patients with this type of semiology during a focal seizure. In the absence of an electroclinical seizure verified by video-EEG monitoring, caution should be made with the diagnosis. A careful history should be obtained to help differentiate between unilateral abdominal clonic jerking and other abdominal complaints.

Retention Rate and Efficacy of Perampanel with a Slow Titration Schedule in Adults.

RATIONALE: The manufacturer of perampanel (PER) suggests an initial adult dose of 2-4 mg/day and an upward dose titration of 2 mg at no more frequently than 1- or 2-week intervals when used with enzyme-enhancing antiepileptic drugs (AEDs) or nonenzyme-enhancing AEDs, respectively. The general practice in our clinic is an initial dose of PER 2 mg/day and titrated by 2 mg/4 weeks to an initial target of 6 mg/day. METHODS: Retrospective chart audit of patients starting PER in an adult epilepsy clinic between September 2013 and November 2016 with at least one 6-month follow-up visit was reviewed. Data collection included patient demographics, seizure characteristics, past and concurrent therapy, monthly seizure frequency before PER and at 6-month visit, and characteristics of PER discontinuation. Efficacy of treatment was assessed with the Engel classification and 50% responder rate. RESULTS: N = 102 patients; mean age = 40 years and 54% females. Focal onset seizures 85%, generalized 13%, and unknown 2%. Median prior AED exposure = 6 (range 3-20); median concomitant AED use = 2 (range 1-5). Follow-up range was 6-37 months. The median seizure frequency/month prePER treatment was 6 (range 0-30) for focal onset seizures and 1 (range 0-6) for generalized seizures. The retention rate amongst all patients at 6 months was 78.4%. At 6-month follow-up, 36% of all patients achieved Engel class I (seizure freedom) (30.7% of patients with focal onset seizures and 63.6% with generalized epilepsy). The 50% responder rate was 52% and 82% for focal and generalized epilepsy, respectively. CONCLUSION: PER has a good retention rate when titrated slowly and thus encouraging seizure freedom results in an otherwise medically refractory epilepsy population.

Divergence between functional magnetic resonance imaging and clinical indicators of language dominance in preoperative language mapping.

Accurate determination of hemispheric language dominance prior to epilepsy surgery is critically important to minimize cognitive morbidity. Functional MRI (fMRI) is a noninvasive method that is highly concordant with other clinical indicators of language laterality, and is now commonly used to confirm language dominance. However, there is also a high frequency of divergence between fMRI findings and other clinical indices that complicate determination of dominance and surgical decision-making in individual patients. Despite this, divergent cases are rarely published or discussed. This article provides three illustrative examples to demonstrate common scenarios where fMRI may produce conflicting or otherwise difficult-to-interpret findings. We will also discuss potential reasons for divergence and propose a flow-chart to aid clinical decision making in such situations.

Wilder Penfield, Sir Arthur Currie, and the Montreal Neurological Institute.

Wilder Penfield and the Montreal Neurological Institute (MNI) are inextricably linked. It was Penfield's unique idea to create a building with an academic atmosphere wherein basic neuroscience and clinical care of neurological patients would benefit from interaction and mutual support. It is clear that without Penfield that there would be no MNI; however, the role of another Canadian icon, Sir Arthur Currie, in the development of the MNI has heretofore been barely mentioned. The thesis of this paper is that Currie had a critical role in the gestation of the MNI that has generally been ignored.

Using learning analytics to evaluate a video-based lecture series.

BACKGROUND: The video-based lecture (VBL), an important component of the flipped classroom (FC) and massive open online course (MOOC) approaches to medical education, has primarily been evaluated through direct learner feedback. Evaluation may be enhanced through learner analytics (LA) - analysis of quantitative audience usage data generated by video-sharing platforms. METHODS AND RESULTS: We applied LA to an experimental series of ten VBLs on electroencephalography (EEG) interpretation, uploaded to YouTube in the model of a publicly accessible MOOC. Trends in view count; total percentage of video viewed and audience retention (AR) (percentage of viewers watching at a time point compared to the initial total) were examined. The pattern of average AR decline was characterized using regression analysis, revealing a uniform linear decline in viewership for each video, with no evidence of an optimal VBL length. Segments with transient increases in AR corresponded to those focused on core concepts, indicative of content requiring more detailed evaluation. We propose a model for applying LA at four levels: global, series, video, and feedback. DISCUSSION AND CONCLUSIONS: LA may be a useful tool in evaluating a VBL series. Our proposed model combines analytics data and learner self-report for comprehensive evaluation.

Orbitofrontal epilepsy: Case series and review of literature.

BACKGROUND: Orbitofrontal epilepsy (OFE) is less known and is poorly characterized in comparison with temporal lobe epilepsy, partly because it is rare and possibly because it is unrecognized and therefore underestimated. OBJECTIVE: This paper aimed to better characterize seizure semiology, presurgical findings, and surgical outcomes in patients with OFE. METHODS: We retrospectively reviewed all confidently established OFE cases from six Canadian epilepsy monitoring units between 1988 and 2014, and in the literature between 1972 and 2017. Inclusion criteria were identification of an epileptogenic lesion localized in the OFC or if the patient was seizure-free after surgical removal of the OFC in nonlesional cases. RESULTS: Sixteen cases were identified from our databases. Fifty percent had predominantly sleep-related seizures; 56% had no aura (the remaining had nonspecific or vegetative auras), and 62.5% featured hypermotor (mostly hyperkinetic) behaviors. Interictal epileptiform discharges over frontal and temporal derivations always allowed lateralization. Magnetic resonance imaging (MRI) identified an orbitofrontal lesion in 8/16, positron emission tomography (PET) identified a hypometabolism extending outside the orbital cortex in 4/9, ictal single-photon emission computed tomography (SPECT) identified an orbital hyperperfusion in 1/5, magnetoencephalography (MEG) identified lateral orbital sources in 2/4, and intracranial electroencephalography (EEG) identified an orbitofrontal onset in 9/10. Fourteen patients underwent surgery, all reaching a favorable outcome (71.4% Engel 1; 28.6% Engel 2; mean FU=5.6years). Pre- and postoperative neuropsychological assessments revealed heterogeneous findings. Our review of literature identified 71 possible cases of OFE, 32 with confident focus localization. Extracted data from these cumulated cases supported observations made from our case series. CONCLUSIONS: Orbitofrontal epilepsy should be suspected with sleep-related, hyperkinetic seizures with no specific aura, and frontotemporal interictal discharges. Several patients have nonmotor seizures with or without auras which may resemble temporal lobe seizures. Postoperative seizure outcome was favorable, but there is inherent bias as we only included patients with a seizure-free outcome if the MRI was negative. A larger study is required to address identified gaps in knowledge such as identifying discriminative features between medial and lateral OFE, evaluating the value of more recent diagnostic tools, and assessing the neuropsychological outcome of orbital epilepsy surgery.

A Video-Based Introductory EEG Curriculum for Neurology Residents and Other EEG Learners.

INTRODUCTION: It is difficult to provide standardized formal education in EEG because of time limitations and the availability of expert teachers. Video-based miniature lectures are a useful way to standardize the foundational principles of EEG and support learning during EEG/epilepsy rotations. METHODS: A curriculum of 10 EEG teaching videos was developed based on concepts outlined in the Accreditation Council for Graduate Medical Education Neurology Milestones. The videos were short (6-17 minutes) and made available to residents rotating through an EEG/epilepsy rotation in two neurology residency programs. Residents were instructed to review the videos and then apply their newly learned skills during EEG reading sessions. A survey about the process was completed at the end of the year. RESULTS: Twenty-one residents participated in the curriculum, and 15 (71%) responded to the survey. Two-thirds of respondents (10/15) said that they watched all of the videos, and 87% (13/15) watched at least half of the videos. All of the respondents used the videos as introductions to EEG concepts, and approximately half of respondents returned to the videos as a refresher after the rotation was over. Nearly all respondents either agreed or strongly agreed that the curriculum was a useful component of the rotation and helped them to understand difficult concepts. All strongly agreed that they would recommend the curriculum to other residents. DISCUSSION: A video-based approach to EEG teaching could complement existing curricula and ensure that learners have access to foundational miniature lectures when and where they need them.

Language lateralization of a bilingual person with epilepsy using a combination of fMRI and neuropsychological assessment findings.

This report describes the findings of language functional magnetic resonance imaging (fMRI) in a left-handed Urdu and English speaker with right hemisphere-originating epilepsy and unclear language dominance. fMRI is a reliable method for determining hemispheric language dominance in presurgical planning. However, the effects of bilingualism on language activation depend on many factors including age of acquisition and proficiency in the tested language, and morphological properties of the language itself. This case demonstrates that completing fMRI in both spoken languages and interpreting the results within the context of a neuropsychological assessment are essential in arriving at accurate conclusions about language distribution in bilingual patients.

Canadian epileptologists' counseling of drivers amidst guideline inconsistencies.

BACKGROUND: Epilepsy is a common medical condition for which physicians perform driver fitness assessments. The Canadian Medical association (CMA) and the Canadian Council of Motor transportation administrators (CCMTA) publish documents to guide Canadian physicians' driver fitness assessments. OBJECTIVES: We aimed to measure the consistency of driver fitness counseling among epileptologists in Canada, and to determine whether inconsistencies between national guidelines are associated with greater variability in counseling instructions. METHODS: We surveyed 35 epileptologists in Canada (response rate 71%) using a questionnaire that explored physicians' philosophies about driver fitness assessments and counseling practices of seizure patients in common clinical scenarios. Of the nine scenarios, CCMTA and CMA recommendations were concordant for only two. Cumulative agreement for all scenarios was calculated using Kappa statistic. Agreement for concordant (two) vs. discordant (seven) scenarios were split at the median and analyzed using the Wilcoxon signed rank sum test. RESULTS: Overall the agreement between respondents for the clinical scenarios was not acceptable (Kappa=0.28). For the two scenarios where CMa and CCMta guidelines were concordant, specialists had high levels of agreement with recommendations (89% each). A majority of specialists disagreed with CMa recommendations in three of seven discordant scenarios. The lack of consistency in respondents' agreement attained statistical significance (p<0.001). CONCLUSIONS: Canadian epileptologists have variable counseling practices about driving, and this may be attributable to inconsistencies between CMa and CCMta medical fitness guidelines. This study highlights the need to harmonize driving recommendations in order to prevent physician and patient confusion about driving fitness in Canada.

Systematic review and meta-analysis of standard vs selective temporal lobe epilepsy surgery.

OBJECTIVE: To compare standard anterior temporal lobectomy (ATL) with selective amygdalohippocampectomy (SAH) for postoperative seizure control in temporal lobe epilepsy (TLE). METHODS: We searched MEDLINE and Embase using Medical Subject Headings and keywords related to ATL and SAH. We included original research that directly compared seizure outcomes in patients undergoing SAH or ATL for TLE. A fixed-effect model was used to derive a pooled risk ratio (RR) for either an Engel Class I (free of disabling seizures) or a composite of an Engel Class I and II (rare disabling seizures) outcome. RESULTS: Of 4,675 abstracts initially identified by the search, 65 were reviewed as full text. Thirteen studies containing data from 8 countries (5 continents) met our inclusion criteria. Eleven studies comprising 1,203 patients demonstrated that participants were statistically more likely to achieve an Engel Class I outcome after ATL compared with SAH (risk ratio 1.32, 95% confidence interval [CI] 1.12-1.57; p < 0.01). The summary risk difference of 8% (95% CI 3%-14%) translates to a number needed to treat of 13 (95% CI 7-33) for 1 additional patient to achieve an Engel Class I outcome after ATL. The result remained significant when 2 studies that contained fewer than 15 participants in at least 1 arm were excluded and in analyses restricted to hippocampal sclerosis. CONCLUSIONS: Standard ATL confers an improved chance of achieving freedom from disabling seizures in patients with TLE. Improved seizure freedom must be balanced against the neuropsychological impact of each procedure. A randomized controlled trial is justified.

Spatial MEG laterality maps for language: clinical applications in epilepsy.

Functional imaging is increasingly being used to provide a noninvasive alternative to intracarotid sodium amobarbitol testing (i.e., the Wada test). Although magnetoencephalography (MEG) has shown significant potential in this regard, the resultant output is often reduced to a simplified estimate of laterality. Such estimates belie the richness of functional imaging data and consequently limit the potential value. We present a novel approach that utilizes MEG data to compute "complex laterality vectors" and consequently "laterality maps" for a given function. Language function was examined in healthy controls and in people with epilepsy. When compared with traditional laterality index (LI) approaches, the resultant maps provided critical information about the magnitude and spatial characteristics of lateralized function. Specifically, it was possible to more clearly define low LI scores resulting from strong bilateral activation, high LI scores resulting from weak unilateral activation, and most importantly, the spatial distribution of lateralized activation. We argue that the laterality concept is better presented with the inherent spatial sensitivity of activation maps, rather than being collapsed into a one-dimensional index.

Depiction of seizure first aid management in medical television dramas.

OBJECTIVES: The objective of this study was to investigate whether medical television dramas portray proper seizure first aid. METHODS: Episodes of the four highest-rated US medical dramas ("Grey's Anatomy," "House M.D.", "Private Practice," and "ER") were screened for the presence of seizures. "Patient" age, sex, semiology, and etiology were recorded. The appropriateness of seizure first aid management was determined by comparison to the Epilepsy Foundation of America (EFA) guidelines. RESULTS: Among 364 television programs, 65 seizures (in 59 individuals) were identified (30 males; 29 females). Seizures were primary or secondarily generalized tonic-clonic in 53 (81.5%) cases. Other seizure types included complex partial (5, 7.7%), simple partial (1, 1.5%), myoclonic (1, 1.5%), absence (1, 1.5%), and psychogenic (1, 1.5%). On 63 occasions (96.9%), first aid was performed by a health care professional. First aid management was judged appropriate in 21 (32.3%) seizures, inappropriate in 28 (43.1%), and indeterminate in 16 (24.6%). Inappropriate practices included holding the person down (17, 26.2%), trying to stop the involuntary movements (10, 15.4%) and putting something in the person's mouth (11, 16.9%). CONCLUSIONS: The first aid management of seizures performed by actors portraying health care professionals was inappropriate in nearly half of all cases. Inaccurate depiction of seizure first aid management may contribute to misinformation of the general public. The television industry could easily incorporate the simple first aid EFA seizure guidelines as a public service without sacrificing dramatic impact.

Lamotrigine-valproic acid combination therapy for medically refractory epilepsy.

PURPOSE: A retrospective study of lamotrigine (LTG)-valproic acid (VPA) combination therapy in medically refractory epilepsy. METHODS: Patients were identified with an adult epilepsy clinic database and were included if they had been on LTG-VPA combination therapy for at least 6 months. Patient demographics and information about epilepsy type, severity, and degree of medical intractability were obtained by retrospective chart review. The primary outcome measure was change in baseline seizure frequency, and patients were stratified into three groups: (i) seizure-free, (ii) improved (at least 50% reduction in baseline seizure frequency), and (iii) not improved. RESULTS: Thirty-five patients met all inclusion-exclusion criteria. Epilepsy type was generalized in 25 patients (71%) and partial in 10 patients (29%). Before LTG-VPA treatment, 27 of 35 (77%) experienced disabling seizures on a monthly basis, and 17 of 35 (49%) of patients had at least one disabling seizure per week. Patients had previously failed treatment with a median of five antiepileptic drugs (AEDs), alone or in combination. With LTG-VPA therapy, 18 (51.4%) remained completely seizure-free, four (11.4%) were improved, and 13 (37.1%) were unimproved. Median follow-up was 42 months. Of the 22 patients who improved, 11 had previously failed LTG and VPA monotherapy. There was no significant difference between improved and unimproved patients with respect to demographics, epilepsy type or severity, or number of previously failed AEDs. DISCUSSION: The combination of LTG and VPA should be considered in patients with medically refractory epilepsy. The effectiveness of this combination appears to be independent of epilepsy type or patient demographics.

Quality of life and memory after vagus nerve stimulator implantation for epilepsy.

OBJECTIVE: This prospective, case control study evaluates quality of life (QOL), depressive affect, and memory outcomes of epilepsy patients implanted with a vagus nerve stimulator (VNS). METHODS: Three groups of patients with epilepsy underwent assessment on two occasions: 1) patients with a VNS were tested before and 12 months after implantation (n = 16); 2) patients who underwent cerebral resective surgery were tested pre- and post-operatively (n = 10); and 3) patients under medical management (n = 9). Group means were compared on the QOLIE-89, Geriatric Depression Scale, Wechsler Memory Scale - III, and the Memory Observation Questionnaire. Secondary analyses calculated the reliable change index, providing information on change beyond measurement error and chance. RESULTS: Mean ratings of QOL, depression, and memory complaints and objective memory scores remained stable or improved in all the groups. The QOL improved more after cerebral resective surgery than VNS or medication controls, but the VNS and medication control groups did not differ. In the VNS group, QOL was not related to seizure reduction. The percentage of cases showing real change in memory was equivalent across groups, except in one of eight indices (i.e., verbal recognition memory). CONCLUSIONS: This first case controlled design found that vagus nerve stimulation as an adjunctive therapy for seizure control did not change QOL, depressive affect, or objective memory scores over one-year more so than medical management alone. We point out the need for larger case control, non-industry funded investigations.

Neurocardiogenic syncope: frequency and consequences of its misdiagnosis as epilepsy.

BACKGROUND: Neurocardiogenic syncope (NCS) can be mistaken as a seizure. We reviewed the frequency and diagnostic consequences of this misdiagnosis. METHODS: A retrospective review of outpatient adult epilepsy clinic charts (QEII Health Sciences Centre, Halifax, NS) was conducted to identify NCS patients referred with a provisional diagnosis of seizures. Charts were reviewed in detail with an emphasis on the consequences of misdiagnosis. RESULTS: Of 1506 consecutive referrals to the epilepsy clinic, 194 (12.9%) ultimately had a clinical diagnosis of NCS. Mean age was 38 +/- 16 years (mean age of syncopal onset was 28 +/- 16 years). Two-thirds of referrals were from primary care physicians (including emergency departments) and 18% from neurologists. Thirty-five percent were prescribed antiepileptic drugs (AEDs) prior to referral with eight patients (4.1%) experiencing hypersensitivity reactions. Three of five women had adverse pregnancy outcomes while taking AEDs. One-third of patients had restrictions placed on their driving privileges while 11 patients (5.7%) had their employment interrupted. Diagnostic modalities used in the work-up included EEG (90%), CT head (51%), and MRI head (15%). CONCLUSIONS: NCS is commonly misdiagnosed as epilepsy. Some patients had an incorrect diagnosis for > 10 years. Patients with this misdiagnosis are often excessively investigated, inappropriately treated, and have unnecessary restrictions placed on driving and employment.