Top of Basilar Syndrome Presenting With Hyperekplexia Initially Diagnosed as a Convulsive Status Epilepticus.

BACKGROUND: Hyperekplexia is a rare neurologic disorder characterized by pronounced startle responses to tactile or acoustic stimuli and increase tone. Acquired hyperekplexia is usually seen in brainstem pathologies and when it develops acutely it can be easily misdiagnosed as a convulsive seizure. CASE REPORT: A 38-year-old man presented with acute onset generalized brief involuntary jerky movements and a decreased level of consciousness. He was initially diagnosed with convulsive status epilepticus for which he received multiple antiseizure medications without any improvement. Further investigations revealed abnormal oculocephalic reflex response and that his movements were in fact hyperkeplexia caused by brainstem infarction with basilar artery thrombus secondary to right vertebral artery dissection. Emergent thrombectomy was performed and he was eventually discharged to a rehabilitation facility. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Emergency physicians should be aware of hyperekplexia and how to differentiate it from convulsive stats epilepticus because the pathology and the emergent treatment of these 2 serious conditions are different. An underlying acquired brainstem pathology (especially basilar artery thromboembolism) should be suspected in any patient with untypical convulsive like movements along with focal neurologic signs compatible with brain stem pathology even when computed tomography imaging is normal. © 2020 Elsevier Inc.

The online educational tool "Roadmap to EEGs" significantly improved trainee performance in recognizing EEG patterns.

OBJECTIVE: We created a framework to assess the competency-based EEG curriculum, outlined by the International League Against Epilepsy (ILAE) through a video-based online educational resource ("Roadmap to EEGs") and assessed its effectiveness and feasibility in improving trainees' knowledge. METHODS: Ten video-based e-learning modules addressed seven key topics in EEG and epileptology (normal EEG, normal variants, EEG artifacts, interictal epileptiform discharges (IED), focal seizures, idiopathic generalized epilepsy (IGE), and developmental and epileptic encephalopathies (DEE)). We posted the educational videos on YouTube for free access. Pre- and post-tests, each comprising 20 multiple-choice questions, were distributed to institution leadership and advertised on social media platforms to reach a global audience. The tests were administered online to assess the participants' knowledge. Pre- and post-test questions showed different EEG samples to avoid memorization and immediate recall. After completing the post-test, participants were asked to respond to 7 additional questions assessing their confidence levels and recommendations for improvement. RESULTS: A total of 52 complete and matched pre- and post-test responses were collected. The probability of a correct response was 73% before teaching (95% CI: 70%-77%) and 81% after teaching (95% CI: 78%-84%). The odds of a correct response increased significantly by 59% (95% CI: 28%-98%, p < .001). For participants having >4 weeks of EEG training, the probability of a correct response was 76% (95% CI: .72-.79) and 81% after teaching (95% CI: .78-.84). The odds of answering correctly increased by 44% (95% CI: 15%-80%, p = .001). Participants felt completely confident in independently interpreting and identifying EEG findings after completing the teaching modules (17.1% before vs. 37.8% after, p-value < .0001). 86.5% of participants expressed a high likelihood of recommending the module to other trainees. SIGNIFICANCE: The video-based online educational resource allows participants to acquire foundational knowledge in EEG/epilepsy, and participants to review previously learned EEG/epilepsy information.

Expert accuracy and inter-rater agreement of "must-know" EEG findings for adult and child neurology residents.

OBJECTIVE: We published a list of "must-know" routine EEG (rEEG) findings for trainees based on expert opinion. Here, we studied the accuracy and inter-rater agreement (IRA) of these "must-know" rEEG findings among international experts. METHODS: A previously validated online rEEG examination was disseminated to EEG experts. It consisted of a survey and 30 multiple-choice questions predicated on the previously published "must-know" rEEG findings divided into four domains: normal, abnormal, normal variants, and artifacts. Questions contained de-identified 10-20-s epochs of EEG that were considered unequivocal examples by five EEG experts. RESULTS: The examination was completed by 258 international EEG experts. Overall mean accuracy and IRA (AC1) were 81% and substantial (0.632), respectively. The domain-specific mean accuracies and IRA were: 76%, moderate (0.558) (normal); 78%, moderate (0.575) (abnormal); 85%, substantial (0.678) (normal variants); 85%, substantial (0.740) (artifacts). Academic experts had a higher accuracy than private practice experts (82% vs. 77%; p = .035). Country-specific overall mean accuracies and IRA were: 92%, almost perfect (0.836) (U.S.); 86%, substantial (0.762) (Brazil); 79%, substantial (0.646) (Italy); and 72%, moderate (0.496) (India). In conclusion, collective expert accuracy and IRA of "must-know" rEEG findings are suboptimal and heterogeneous. SIGNIFICANCE: We recommend the development and implementation of pragmatic, accessible, country-specific ways to measure and improve the expert accuracy and IRA.

Education Research: EEG Education in Child Neurology and Neurodevelopmental Disabilities Residencies: A Survey of US and Canadian Program Directors.

Background and Objectives: In the United States, many child neurologists (CNs) and neurodevelopmental disability (NDD) specialists who read EEGs in clinical practice had no additional EEG training other than what was received during residency. This practice highlights the importance of ensuring that CN/NDD residents achieve EEG competence before graduation. However, prior survey-based evidence showed that roughly a third of graduating CN residents in the United States do not feel confident interpreting EEGs independently. As part of a needs assessment, we conducted a descriptive study characterizing EEG practices in CN and NDD residency programs in the United States and Canada. Methods: A 30-question e-survey focused on characteristics of residency programs and their EEG teaching practices was sent to all 88 CN and NDD residency program directors listed in the Accreditation Council for Graduate Medical Education, Child Neurology Society, and Canadian Residency Matching Service websites. Results: Twenty-nine (n = 29/88; 33%) residency programs completed the survey, most of which were CN (90%), academic (90%), and located in the United States (83%). The mean number of weeks dedicated to EEG training required to graduate was 7.3 ± 4 (mean ± SD). EEG rotations involved the clinic/outpatient setting (83%), epilepsy monitoring unit (EMU) (76%), and inpatient setting (excluding EMU) (72%). During a 4-week EEG rotation, residents typically read 16-45 EEGs (62%). The most common methods of EEG teaching in CN/NDD programs were teaching during EEG rotation and yearly didactics. The mean number of EEGs read per rotation had a significant positive correlation with the average percentage of residents who reportedly achieve EEG competence by graduation (coefficient 0.461; p = 0.007). Barriers to EEG education were reported by 28% of the programs; the most common barrier identified was insufficient EEG exposure. Possible solutions were primarily related to increasing quality and quantity of EEG exposure. Almost two-thirds of programs reported not using objective measures to assess EEG competence. Discussion: Our results characterize resident EEG education in a third of CN/NDD residency programs in the United States and Canada. We suggest that residency leaderships consider standardization of EEG learning along with establishment and implementation of objective measures in training requirements and competence assessment.

A comparison of intrathecal dexmedetomidine, clonidine, and fentanyl as adjuvants to hyperbaric bupivacaine for lower limb surgery: A double blind controlled study.

BACKGROUND: Various adjuvants are being used with local anesthetics for prolongation of intraoperative and postoperative analgesia. Dexmedetomidine, the highly selective 2 adrenergic agonist is a new neuraxial adjuvant gaining popularity. SETTINGS AND DESIGN: The study was conducted in prospective, double blind manner. It included 120 American Society of Anesthesiology (ASA) class I and II patients undergoing lower limb surgery under spinal anesthesia after approval from hospital ethics committee with written and informed consent of patients. MATERIALS AND METHODS: The patients were randomly allocated into four groups (30 patients each). Group BS received 12.5 mg hyperbaric bupivacaine with normal saline, group BF received 12.5 mg bupivacaine with 25 g fentanyl, group BC received 12.5 mg of bupivacaine supplemented 30 g clonidine, and group BD received 12.5 mg bupivacaine plus 5 g dexmedetomidine. The onset time to reach peak sensory and motor level, the regression time of sensory and motor block, hemodynamic changes, and side effects were recorded. RESULTS: Patients in Group BD had significantly longer sensory and motor block times than patients in Groups BC, BF, and BS with Groups BC and BF having comparable duration of sensory and motor block. The mean time of two segment sensory block regression was 147 ± 21 min in Group BD, 117 ± 22 in Group BC, 119 ± 23 in Group BF, and 102 ± 17 in Group BS (P > 0.0001). The regression time of motor block to reach modified Bromage zero (0) was 275 ± 25, 199 ± 26, 196 ± 27, 161 ± 20 in Group BD, BC, BF, and BS, respectively (P > 0.0001). The onset times to reach T8 dermatome and modified Bromage 3 motor block were not significantly different between the groups. Dexmedetomidine group showed significantly less and delayed requirement of rescue analgesic. CONCLUSIONS: Intrathecal dexmedetomidine is associated with prolonged motor and sensory block, hemodynamic stability, and reduced demand of rescue analgesics in 24 h as compared to clonidine, fentanyl, or lone bupivacaine.

Ictal blinking triggered by isolated spikes as the only manifestation of seizures.

OBJECTIVE: To describe blinking as the only manifestation of seizures from isolated focal and generalized cortical spikes and investigate the relationship between blinks and epileptic discharges. METHODS: We measured the latency from the onset of spikes to the onset of blinks in two patients using electroencephalogram (EEG) and an electrooculogram (EOG), and calculated the median latency in both cases. We analyzed the latency from spike onset to the onset of additional specific eye movements, seen only in the second case. To determine the frequency of spontaneous blinks (not triggered by spikes), we defined a "control point" at 45 s following a random spike for the first case. We tested for statistically significant associations between latencies of blinks (Case 1) as well as between latencies of blinks and specific eye movements (Case 2). RESULTS: A total of 174 generalized spike-waves followed by a blink were analyzed in the first patient. Approximately 61% of the blinks occurred within 150-450 ms after the onset of the spike. Median latency for blinks following a spike was 294 ms compared to 541 ms for control blinks (p = .02). For the second patient, a total of 160 eye movements following a right occipito-parietal spike were analyzed. The median spike-blink latency in the second case was 497 milliseconds. Median latencies of spike onset to contralateral oblique eye movements with blink and left lateral eye movements were 648 and 655 milliseconds, respectively. CONCLUSIONS: Our study shows that isolated cortical spikes can induce epileptic seizures consisting exclusively of blinks. These findings emphasize the importance of careful EEG and EOG analysis to determine blinking as the only ictal phenomenon. We additionally describe a new technique to prove the temporal relationship between cortical discharges and a specific movement when, in addition to the movements triggered by a spike, the same movement is also spontaneously performed by the patient (in this case, blinking).

Bilateral tonic seizures vs. bilateral tonic events in critically ill patients: differences in semiology.

PURPOSE: Our primary aim was to analyze bilateral epileptic tonic seizures (ETS) and bilateral non-epileptic tonic events (NTE) in critically ill patients. Our secondary aim was to analyze ETS per their epileptogenic zone. METHODS: We performed a retrospective analysis of clinical signs in patients with bilateral ETS and NTE. Two authors independently reviewed 34 videos of ETS in 34 patients and 15 videos of NTEs in 15 patients. Initial screening and review was performed in an unblinded manner. Subsequently, the semiology was characterized independently and blindly by a co-author. Statistical analysis was conducted using Bonferroni correction and two-tailed Fischer exact test. Positive predictive value (PPV) was calculated for all signs. Cluster analysis of signs with a PPV >80% was performed to evaluate co-occurring semiological features in the two groups. RESULTS: Compared to patients with ETS, those with NTEs more frequently had predominant involvement of proximal upper extremities (UE) (67% vs. 21%), internal rotation of UE (67% vs. 3%), adduction of UE (80% vs. 6%) and bilateral elbow extension (80% vs. 6%). In contrast, those with ETS more frequently had abduction of UE (82% vs 0%), elevation of UE (91% vs. 33%), open eyelids (74% vs. 20%), and involvement of both proximal and distal UE (79% vs. 27%). In addition, seizures that remained symmetrical throughout were more likely to have a generalized onset than focal (38% vs. 6%), p = 0.032, PPV 86%. CONCLUSIONS: A careful analysis of semiology can often help differentiate between ETS and NTE in the ICU. The combination of eyelids open, upper extremity abduction, and elevation reached a PPV of 100% for ETS. The combination of bilateral arms extension, internal rotation, and adduction reached a PPV of 90.9% for NTE.

Education Research: Competency-Based EEG Education: An Online Routine EEG Examination for Adult and Child Neurology Residents.

Background and Objectives: We recently published expert consensus-based curricular objectives for routine EEG (rEEG) interpretation for adult and child neurology residents. In this study, we used this curriculum framework to develop and validate an online, competency-based, formative and summative rEEG examination for neurology residents. Methods: We developed an online rEEG examination consisting of a brief survey and 30 multiple-choice questions covering EEG learning objectives for neurology residents in 4 domains: normal, abnormal, normal variants, and artifacts. Each question contained a deidentified EEG image, displayed in 2 montages (bipolar and average), reviewed and optimized by the authors to address the learning objectives. Respondents reported their level of confidence (LOC, 5-point Likert scale) with identifying 4 categories of EEG findings independently: states of wakefulness/sleep, sleep structures, normal variants, and artifacts. Accuracy and item discrimination were calculated for each question and LOC for each category. The test was disseminated by the International League Against Epilepsy and shared on social media. Results: Of 2,080 responses, 922 were complete. Respondents comprised clinical neurophysiologists/experts (n = 41), EEG/epilepsy clinical fellows (n = 211), EEG technologists (n = 128), attending neurologists (n = 111), adult neurology residents (n = 227), child neurology residents (n = 108), medical students (n = 24), attending non-neurologists (n = 18), and others (n = 54). Mean overall scores (95% CI) were 82% (77-86) (clinical neurophysiologists), 81% (79-83) (clinical fellows), and 72% (70-73) (adult and child neurology residents). Experts were more confident than clinical fellows in all categories but sleep structures. Experts and clinical fellows were more confident than residents in all 4 categories. Among residents, accuracy and LOC increased as a function of prior EEG weeks of training. Accuracy improved from 67% (baseline/no prior EEG training) to 77% (>12 prior EEG weeks). More than 8 weeks of EEG training was needed to reach accuracy comparable with clinical neurophysiologists on this rEEG examination. Increase in LOC was slower and less robust than increase in accuracy. All but 3 questions had a high discrimination index (>0.25). Discussion: This online, competency-based rEEG examination, mapped to a published EEG curriculum, has excellent psychometrics and differentiates experienced EEG readers from adult and child neurology residents. This online tool has the potential to improve resident EEG education worldwide.

Diagnostic Yield of 8-Hour Video-EEG in Detecting Psychogenic Non-Epileptic Seizures (PNES).

To evaluate the diagnostic yield of the first 8 hours of video-EEG (vEEG) monitoring in detecting Psychogenic Non-Epileptic Seizures (PNES) during the Epilepsy Monitoring Unit (EMU) admission. We performed a retrospective chart review of patients ages ≥4 years who were admitted to the EMU between 2011 and 2018 (n = 616). We calculated the proportion of patients diagnosed with PNES within the first 8 hours of EEG recording and studied the associated risk factors for patients diagnosed with PNES and patients with epileptic seizures (ES). Out of the total 616 patients, 24% (149) patients had an EMU diagnosis of PNES. Of these, 44.3% had at least one typical event within the first 8 hours of vEEG monitoring. A higher incidence was seen within the pediatric subgroup (54.8% had an event within 8 hours). A diagnosis of chronic pain disorder was more common with PNES compared to ES (48.3% versus 16.5%, p < 0.001). A suspicion for PNES documented during an office visit was noted in a high proportion of patients (68.5%) who eventually had a PNES event during EMU. Our study suggests that in a well-selected group of patients (such as a high suspicion of PNES during a physician/neurology office visit), an outpatient 8-hour vEEG could open new avenues for a prompt diagnosis. This could especially be beneficial in hospital settings where there is either a lack of an EMU or a delay in admission to the EMU.

A Rare Case of Prolonged Ictal Aphasia.

Aphasia is commonly seen in focal brain lesions. Prolonged aphasia from an ictal state is rarely reported. We report the case of a 62-year-old man with focal motor status epilepticus manifested initially as episodic right cheiro-oral clonic movements with preserved awareness and expressive aphasia for 48 hours. EEG showed left frontal and central lateralized periodic discharges (LPDs) without plus features (rhythmicity, overlying fast) and electrographic seizures that correlated with right clonic movements. Treatment with two seizure medications (levetiracetam and lacosamide) resulted in complete electrographic and clinical resolution of his symptoms, including aphasia. In this case, aphasia was determined to be an ictal semiology, as patient had complete resolution of his symptoms supported by a normal EEG after receiving seizure medications. We suggest keeping high suspicion for an ictal process in patients with sudden-onset aphasia supported by EEG findings of LPDs.

Ictal Asystole in Temporal Lobe Epilepsy: A Possible Cause of Sudden Unexplained Death - A Case Report.

We report a case of a 47-year-old woman who presented with a 3-year history of paroxysmal events after suffering traumatic brain injury in a motor vehicle accident. She had not previously been diagnosed with epilepsy. On video-EEG monitoring, she was found to have a right temporal seizure associated with ictal asystole lasting for 18 seconds. Our case raises the possibility that undiagnosed ictal asystole could be a potential cause of sudden unexplained death.