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BACKGROUND AND OBJECTIVES: Seizures (SZs) and other SZ-like patterns of brain activity can harm the brain and contribute to in-hospital death, particularly when prolonged. However, experts qualified to interpret EEG data are scarce. Prior attempts to automate this task have been limited by small or inadequately labeled samples and have not convincingly demonstrated generalizable expert-level performance. There exists a critical unmet need for an automated method to classify SZs and other SZ-like events with expert-level reliability. This study was conducted to develop and validate a computer algorithm that matches the reliability and accuracy of experts in identifying SZs and SZ-like events, known as “ictal-interictal-injury continuum” (IIIC) patterns on EEG, including SZs, lateralized and generalized periodic discharges (LPD, GPD), and lateralized and generalized rhythmic delta activity (LRDA, GRDA), and in differentiating these patterns from non-IIIC patterns. METHODS: We used 6,095 scalp EEGs from 2,711 patients with and without IIIC events to train a deep neural network, SPaRCNet, to perform IIIC event classification. Independent training and test data sets were generated from 50,697 EEG segments, independently annotated by 20 fellowship-trained neurophysiologists. We assessed whether SPaRCNet performs at or above the sensitivity, specificity, precision, and calibration of fellowship-trained neurophysiologists for identifying IIIC events. Statistical performance was assessed by the calibration index and by the percentage of experts whose operating points were below the model's receiver operating characteristic curves (ROCs) and precision recall curves (PRCs) for the 6 pattern classes. RESULTS: SPaRCNet matches or exceeds most experts in classifying IIIC events based on both calibration and discrimination metrics. For SZ, LPD, GPD, LRDA, GRDA, and “other” classes, SPaRCNet exceeds the following percentages of 20 experts—ROC: 45%, 20%, 50%, 75%, 55%, and 40%; PRC: 50%, 35%, 50%, 90%, 70%, and 45%; and calibration: 95%, 100%, 95%, 100%, 100%, and 80%, respectively. DISCUSSION: SPaRCNet is the first algorithm to match expert performance in detecting SZs and other SZ-like events in a representative sample of EEGs. With further development, SPaRCNet may thus be a valuable tool for an expedited review of EEGs. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that among patients with epilepsy or critical illness undergoing EEG monitoring, SPaRCNet can differentiate (IIIC) patterns from non-IIIC events and expert neurophysiologists.

BACKGROUND AND OBJECTIVES: The validity of brain monitoring using electroencephalography (EEG), particularly to guide care in patients with acute or critical illness, requires that experts can reliably identify seizures and other potentially harmful rhythmic and periodic brain activity, collectively referred to as “ictal-interictal-injury continuum” (IIIC). Previous interrater reliability (IRR) studies are limited by small samples and selection bias. This study was conducted to assess the reliability of experts in identifying IIIC. METHODS: This prospective analysis included 30 experts with subspecialty clinical neurophysiology training from 18 institutions. Experts independently scored varying numbers of ten-second EEG segments as “seizure (SZ),” “lateralized periodic discharges (LPDs),” “generalized periodic discharges (GPDs),” “lateralized rhythmic delta activity (LRDA),” “generalized rhythmic delta activity (GRDA),” or “other.” EEGs were performed for clinical indications at Massachusetts General Hospital between 2006 and 2020. Primary outcome measures were pairwise IRR (average percent agreement [PA] between pairs of experts) and majority IRR (average PA with group consensus) for each class and beyond chance agreement (κ). Secondary outcomes were calibration of expert scoring to group consensus, and latent trait analysis to investigate contributions of bias and noise to scoring variability. RESULTS: Among 2,711 EEGs, 49% were from women, and the median (IQR) age was 55 (41) years. In total, experts scored 50,697 EEG segments; the median [range] number scored by each expert was 6,287.5 [1,002, 45,267]. Overall pairwise IRR was moderate (PA 52%, κ 42%), and majority IRR was substantial (PA 65%, κ 61%). Noise-bias analysis demonstrated that a single underlying receiver operating curve can account for most variation in experts' false-positive vs true-positive characteristics (median [range] of variance explained ([Image: see text]): 95 [93, 98]%) and for most variation in experts' precision vs sensitivity characteristics ([Image: see text]: 75 [59, 89]%). Thus, variation between experts is mostly attributable not to differences in expertise but rather to variation in decision thresholds. DISCUSSION: Our results provide precise estimates of expert reliability from a large and diverse sample and a parsimonious theory to explain the origin of disagreements between experts. The results also establish a standard for how well an automated IIIC classifier must perform to match experts. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that an independent expert review reliably identifies ictal-interictal injury continuum patterns on EEG compared with expert consensus.

“Subclinical” generalized epileptiform discharges in patients with generalized epilepsy may be associated with transitory cognitive impairment upon appropriate testing.1-4 Increased reaction time is the most sensitive behavioral correlate to these discharges.3,4

Background High efficacy disease modifying therapies (DMT) in the management of Multiple Sclerosis (MS) have a favorable effect on relapse rate and disability progression; however, they can expose patients to significant risks, such as progressive multifocal leukoencephalopathy (PML). Objective The study aims to investigate prognostic factors that can determine outcome in MS-related PML patients. Methods We conducted a literature review and meta-analysis of 194 patients from 62 articles in PubMed, SCOPUS and EMBASE. Results Out of 194 patients (66.5% women, 33.5% men), 81% had progression in their EDSS score by at least 1 point from the time of PML diagnosis (EDSS-P group). The remaining patients had either stable or improved EDSS (EDSS-S group). In univariate analysis, older age at the time of PML diagnosis was associated with higher probability of disability accumulation and worsening of EDSS by at least 1 point (mean age = 44.8, p = 0.046). After adjusting for other variables, age at time of PML diagnosis remained a significant predictive variable in the multivariable logistic model (OR = 0.93, 95% CI: 0.88–0.99, p = 0.037). Natalizumab is the most commonly associated DMT linked to PML, followed by fingolimod and others including dimethyl fumarate, ocrelizumab, alemtuzumab. Among the different treatments used, no therapeutic agent was found to be superior in improving post-PML EDSS. Conclusions Younger age and lower JCV viral load at the time of PML diagnosis were associated with better outcome in MS-associate PML, while none of the PML therapies was superior over the others or associated with favorable outcome.

PURPOSE: Studies examining seizures (Szs) and epileptiform abnormalities (EAs) using continuous EEG in acute ischemic stroke (AIS) are limited. Therefore, we aimed to describe the prevalence of Sz and EA in AIS, its impact on anti-Sz drug management, and association with discharge outcomes. METHODS: The study included 132 patients with AIS who underwent continuous EEG monitoring >6 hours. Continuous EEG was reviewed for background, Sz and EA (lateralized periodic discharges [LPD], generalized periodic discharges, lateralized rhythmic delta activity, and sporadic epileptiform discharges). Relevant clinical, demographic, and imaging factors were abstracted to identify risk factors for Sz and EA. Outcomes included all-cause mortality, functional outcome at discharge (good outcome as modified Rankin scale of 0–2 and poor outcome as modified Rankin scale of 3–6) and changes to anti-Sz drugs (escalation or de-escalation). RESULTS: The frequency of Sz was 7.6%, and EA was 37.9%. Patients with Sz or EA were more likely to have cortical involvement (84.6% vs. 67.5% P = 0.028). Among the EAs, the presence of LPD was associated with an increased risk of Sz (25.9% in LPD vs. 2.9% without LPD, P = 0.001). Overall, 21.2% patients had anti-Sz drug changes because of continuous EEG findings, 16.7% escalation and 4.5% de-escalation. The presence of EA or Sz was not associated with in-hospital mortality or discharge functional outcomes. CONCLUSIONS: Despite the high incidence of EA, the rate of Sz in AIS is relatively lower and is associated with the presence of LPDs. These continuous EEG findings resulted in anti-Sz drug changes in one-fifth of the cohort. Epileptiform abnormality and Sz did not affect mortality or discharge functional outcomes.

Background: To study the nature and frequency of occurrence of “significant” neurological complications in coronavirus disease-2019 (COVID-19) via a systematic review of the literature.Methods: We screened all articles resulting from a search of PubMed, Cochrane, Google Scholar and Scopus, using the keywords "COVID-19 and CNS", "SARS-CoV-2 and CNS”, “COVID-19 and neurological manifestation”, “SARS2 and neurological manifestation” and “COVID-19 and Brain” looking for reports of significant neurological manifestations that would potentially have an impact on the outcome.Results: Twenty-six articles met the inclusion criteria. The significant neurological diagnoses reported were stroke, Guillain Barre Syndrome (GBS) and its variants, encephalitis, seizures, acute hemorrhagic necrotizing encephalopathy, acute disseminated encephalomyelitis (ADEM) and transverse myelitis. Although stroke, predominantly ischemic, was observed in ~ 6% of COVID-19 patients from Wuhan, China, mortality in this cohort was 38%. Of the 24 pooled patients with reports of etiology, 17 had large vessel occlusions. GBS occurred in 5/1200 (0.4%) of the COVID-19 cohort from Italy. One of the six reported encephalitis cases, the ADEM case and the report of transverse myelitis do not have data for conclusive diagnosis.Conclusion: The most frequent significant neurological association with COVID-19 is stroke, predominantly ischemic. In a cohort from Wuhan, China, this was as frequent as ~ 6%, with a 38% mortality. Most common reported etiology is large vessel occlusion. Other reported significant neurological complications are GBS/variants, encephalitis, seizures and acute hemorrhagic necrotizing encephalopathy. The reports of ADEM and transverse myelitis lacked diagnostically conclusive data.

Objective Primary intraventricular hemorrhage (pIVH) uncommonly presents with seizures. There are no prior data regarding the frequency of seizures, periodic and rhythmic patterns on continuous electroencephalography (EEG), (cEEG) in these patients. Methods We retrospectively assessed frequency of seizures, periodic discharges, and rhythmic patterns in pIVH patients undergoing cEEG monitoring. We reviewed indications for cEEG, demographics, GCS at presentation and during cEEG, modified Graeb score (mGS), presence of hydrocephalus, cEEG duration, findings and use of antiseizure medications (ASM). cEEG patterns were classified according to location and morphology. All patterns were considered “hyperexcitable” except GRDA. The ictal‐interictal continuum (IIC) was defined as LRDA, PDs, and/or SW >1 Hz but

Unilateral ptosis may be seen in focal seizures and should be actively sought for during video-EEG (scalp and intracranial) evaluations. Further studies are needed to confirm its lateralizing value, but it was contralateral to seizure onset in this case.

A 71-year-old woman with hypertension, diabetes, and recent stroke presented with acute left hemiparesis, which started 3.5 hours earlier but improved by the time of emergency department evaluation. Examination showed mild left-sided pronator drift and sensory extinction. Her history was significant for resection of a right frontoparietal meningioma for which she had received adjuvant radiation therapy 3 years previously.

Introduction: While seizures are uncommon but reported in primary intraventricular hemorrhage (IVH), little evidence is available on the prevalence of hyperexcitable patterns on long term EEG monitoring. Methods: We sought to determine the prevalence of hyperexcitable patterns and seizures in patients with primary IVH who were extracted from a cohort consisting of patients with spontaneous intracerebral hemorrhage (sICH) who underwent continuous electroencephalogram (cEEG) monitoring between January 2013 and December 2016 at Yale-New Haven Hospital. Indications for cEEG monitoring included fluctuation of or depressed mental status, abnormal movements and a limited clinical exam. We recorded demographics, radiologic hydrocephalus, duration of EEG recording and EEG findings. Hyperexcitable patterns comprised generalized, bilateral independent or lateralized periodic discharges (PDs), lateralized rhythmic delta activity (RDA), brief potentially ictal rhythmic discharges (B(I)RDs), and spike-and-wave discharges (SW). Results: Of 196 adults with sICH who had cEEG performed, 13 patients (54% female) had primary IVH. Hydrocephalus was present in 9 patients (69%). Patients were monitored for a mean duration of 22.4 (± 14.7) hours. 9 patients had hyperexcitable patterns and/or electrographic seizures (70%): Electrographic seizures and co-existent hyperexcitable patterns were captured in 2 of 13 patients (16%) and hyperexcitable patterns without seizures in 7 of 13 patients (54%). Hyperexcitable patterns included periodic discharges (PDs) (4) (generalized, lateralized and bilateral independent, with and without rhythmicity), rhythmic delta activity (RDA) (5) (both lateralized and generalized, with and without sharps), brief potentially ictal rhythmic discharges(B(I)RDs) (1) and spike-and-wave discharges (SW) (1). There was no significant difference between patients with and without hydrocephalus and hyperexcitability or electrographic seizures (p= 0.76). Conclusion: Both electrographic seizures and/or patterns of hyperexcitability on EEG are common in our cohort of primary IVH patients. The exclusion or detection of non-convulsive seizures offers an opportunity for therapeutic intervention.

We report a case of a 32-year-old woman with a 2-year history of multiple sclerosis (MS) who presented to the ophthalmology clinic 1 week after the completion of pulse steroids with complaints of blurred vision associated with pain in the left eye. She was treated with 3 courses of high-dose methylprednisolone (1,000 mg a day for 3 days) for recurrent relapses in the 5 months before presentation (brain MRI, figure, A). Her symptoms during these relapses were left hemiparesis, right hemiparesis, and worsening right hemiparesis, in that order. She was started on glatiramer acetate 1 month before presentation. Given the frequent relapses, changing her disease-modifying therapy to natalizumab was being considered.

A 38-year-old woman presented with a right-sided throbbing headache associated with visual symptoms, nausea, vomiting, photophobia, and phonophobia. She had experienced migraines since her teenage years characterized by monthly unilateral headache associated with nausea and photophobia. The current headache had started with symptoms she recognized as her regular monthly migraine, but it had uncharacteristically persisted for 3 days. She also described a persistent aura of “pixelated 3-dimensional objects” and “colored pinwheels,” which she had not experienced with her previous headaches. Two days into the headache, she had developed a defect in her left visual field and had narrowly missed hitting a tree on that side while driving. At the time of presentation to us, she had already undergone a head CT, which was reported normal, and she was prescribed prednisone, sumatriptan, and oxycodone for management of status migrainosus, without benefit.

A 40-year-old woman with complex partial seizures and right hippocampal atrophy (figure, A and B) had persistent seizures after epilepsy surgery. One of her seizures is shown in the video on the Neurology ® Web site at [Neurology.org][1]. She pushes the event button during the aura (rising epigastric sensation), rubs her mouth, reaches for water, and holds onto it through the seizure. Acknowledgment: The authors acknowledge the help of Ms. Sheila Jackson in obtaining the video and the EEG from the system. [1]: http://neurology.org/lookup/doi/10.1212/WNL.0000000000002662

Background: To assess the impact of new therapeutic strategies on outcomes and hospitalization charges among adult patients with botulism in the United States. Methods: We determined in-hospital outcomes and charges for patients with botulism hospitalized in 1993–1994 and compared them with those observed among patients hospitalized in 2006–2007. Mortality, length of stay, and hospitalization charges were calculated. Age, sex, race, ethnicity, and discharge status were also reported. Results: There were 66 and 132 admissions of adult patients with botulism in 1993–1994 and 2006–2007, respectively. Men predominance was observed in 2006–2007 compared to women predominance during the 1993–1994 time period. There was no significant difference in the average length of stay and in-hospital mortality rate between the two groups studied. However, in the 2006–2007 group, there was a significant increase in the mean hospitalization charges (USD 126,092 ± 120,535 vs. USD 83,623 ± 82,084; p = 0.0107) and in the proportion of patients requiring mechanical ventilation when compared to 1993–1994 (34 vs. 13.6%; p < 0.0001). Conclusion: Botulism continues to be an infrequent cause of hospitalization, with a significant increase in the average hospitalization charges in 2006–2007 when compared to 1993–1994, despite a nonsignificant change in the mortality rate and average length of hospitalization.