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Objective: Although >30% of epilepsy patients have drug-resistant epilepsy (DRE), typically those with generalized or multifocal disease have not traditionally been considered surgical candidates. Responsive neurostimulation (RNS) of the centromedian (CM) region of the thalamus now appears to be a promising therapeutic option for this patient population. We present outcomes following CM RNS for 13 patients with idiopathic generalized epilepsy (IGE) and eight with multifocal onsets that rapidly generalize to bilateral tonic-clonic (focal to bilateral tonic-clonic [FBTC]) seizures., Methods: A retrospective review of all patients undergoing bilateral CM RNS by the senior author through July 2022 were reviewed. Electrodes were localized and volumes of tissue activation were modeled in Lead-DBS. Changes in patient seizure frequency were extracted from electronic medical records., Results: Twenty-one patients with DRE underwent bilateral CM RNS implantation. For 17 patients with at least 1 year of postimplantation follow-up, average seizure reduction from preoperative baseline was 82.6% (SD = 19.0%, median = 91.7%), with 18% of patients Engel class 1, 29% Engel class 2, 53% Engel class 3, and 0% Engel class 4. There was a trend for average seizure reduction to be greater for patients with nonlesional FBTC seizures than for other patients. For patients achieving at least Engel class 3 outcome, median time to worthwhile seizure reduction was 203.5 days (interquartile range = 110.5-343.75 days). Patients with IGE with myoclonic seizures had a significantly shorter time to worthwhile seizure reduction than other patients. The surgical targeting strategy evolved after the first four subjects to achieve greater anatomic accuracy., Significance: Patients with both primary and rapidly generalized epilepsy who underwent CM RNS experienced substantial seizure relief. Subsets of these patient populations may particularly benefit from CM RNS. The refinement of lead targeting, tuning of RNS system parameters, and patient selection are ongoing areas of investigation., (© 2024 International League Against Epilepsy.)

Background and Objectives: Epilepsy education has been transformed over the past 2 decades, leading to a need for structured formative assessment tools. The American Epilepsy Society developed the Epilepsy Fellowship In-Training Examination (EpiFITE) to provide high-quality formative assessment for fellows, to stimulate program improvement, and to guide future learning and teaching. The aim of this study was to explore validity evidence for the EpiFITE in meeting these goals., Methods: Validity evidence was sought from multiple sources. The content of the examination was linked to the American Board of Psychiatry and Neurology blueprint for initial certification in epilepsy, and items were developed by trained experts. Internal structure was studied using internal consistency and item analysis. Surveys of fellows and fellowship directors focused on the examination experience (response process) and how results influenced fellow assessment, future learning, and program improvement (relationship to other variables and consequences)., Results: The EpiFITE was first administered in 2020, with 172 examinees from 67 programs. By 2022 (year 3), the EpiFITE was completed by 195 epilepsy fellows from 77 programs. The overall mean score of the examination was stable from year to year, and the committee predicted the difficulty of individual items with a high degree of accuracy. The examination had high internal consistency (Cronbach α 0.76-0.81). The median item discrimination index ranged from 0.17 in 2020 to 0.21 in 2022. Discrimination indices were lower (mean ≤0.10) for items that were either very easy or very difficult and significantly higher (mean >0.20) for other items. Program directors and epilepsy fellows agreed the examination questions were appropriate and agreed that the EpiFITE helped them identify areas for self-directed learning. Program directors also found the examination helpful in identifying areas of strength and areas for improvement within their programs., Discussion: There are several sources of evidence of the quality and validity of the EpiFITE. By exploring this validity evidence, we have identified several best practices in the development and evaluation of a subspecialty examination, and this experience could be helpful for developers of in-training examinations in other subspecialties., Competing Interests: The authors report no disclosures relevant to the manuscript. Go to Neurology.org/NE for full disclosures., (© 2023 American Academy of Neurology.)

The purpose of this qualitative study is to describe the clinical course of two patients who presented with new-onset seizures within hours of vaping and to survey neurologists' screening for vaping in such patients. A 30-subject single-institution survey found that 19 out of 30 neurology providers have not been subjectively qualifying vaping as a potential seizure-provoking factor since the 2019 emergence of literature on this topic. Inquiring about vaping during a new-onset seizure assessment could lead to earlier recognition of a seizure-provoking factor. Further investigations into the epileptogenicity of vaping are needed and the utility of AntiEpileptic Drug (AED) initiation for these patients is currently unknown., (© The Association of Military Surgeons of the United States 2021. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Summary: The health consequences of e-cigarette use (vaping) have recently garnered significant attention, most notably from reports of pulmonary pathologic examination although there are scattered case reports of vape-induced seizures, but thus far, there have been little objective data or rigorous study supporting this association with definitive quantification., Competing Interests: J. Oster: research support from Eisai and Biogen. The remaining authors have no conflicts of interest to disclose., (Copyright © 2021 by the American Clinical Neurophysiology Society.)

Objective: To study the pregnancy outcomes, including obstetric complications and fetal outcomes, in pregnant women with epilepsy (WWE) treated with direct brain-responsive neurostimulation (RNS System)., Methods: Retrospective review of obstetric outcomes and fetal outcomes in WWE treated with the RNS System at nine comprehensive epilepsy centers in the United States from 2014-2020. In addition, changes in seizure frequency, anti-seizure medications, and RNS System setting adjustments during pregnancy were investigated., Results: A total of 10 subjects and 14 pregnancies were identified. The mean age at conception was 30.6 ± 4.3 years old. The mean age at implantation was 29.8 ± 4.4 years old. The mean stimulation charge densities ranged from 1.0 to 3.0 μC/cm 2 during pregnancy. Obstetric complications included recurrent miscarriage (1 patient), cesarean section (3 patients) due to preeclampsia, non-reassuring fetal heart rate tracing or prolonged labor, preterm birth (1 patient), and preeclampsia (1 patient). No still birth, gestational hypertension, gestational diabetes, eclampsia, or maternal mortality were observed. No RNS System-exposed pregnancies had major congenital malformations. One offspring had a minor congenital anomaly of cryptorchidism in a pregnancy complicated with risk factors of advanced maternal age and bicornuate uterus., Significance: The present study is the first report of RNS System-exposed pregnancies in WWE to date. No major congenital malformations were identified. All of the obstetric complications were within the expected range of those in WWE based on previously published data. The sample size of our study is small, so accumulation of additional cases will further help depict the safety profile of treatment with the RNS System during pregnancy., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Mercury (Hg) is a persistent element in the environment that has the ability to bioaccumulate and biomagnify up the food chain with potentially harmful effects on ecosystems and human health. Twenty-four streams remotely located in forested watersheds in northwestern PA containing naturally reproducing Salvelinus fontinalis (brook trout), were targeted to gain a better understanding of how Marcellus shale natural gas exploration may be impacting water quality, aquatic biodiversity, and Hg bioaccumulation in aquatic ecosystems. During the summer of 2012, stream water, stream bed sediments, aquatic mosses, macroinvertebrates, crayfish, brook trout, and microbial samples were collected. All streams either had experienced hydraulic fracturing (fracked, n = 14) or not yet experienced hydraulic fracturing (non-fracked, n = 10) within their watersheds at the time of sampling. Analysis of watershed characteristics (GIS) for fracked vs non-fracked sites showed no significant differences (P > 0.05), justifying comparisons between groups. Results showed significantly higher dissolved total mercury (FTHg) in stream water (P = 0.007), lower pH (P = 0.033), and higher dissolved organic matter (P = 0.001) at fracked sites. Total mercury (THg) concentrations in crayfish (P = 0.01), macroinvertebrates (P = 0.089), and predatory macroinvertebrates (P = 0.039) were observed to be higher for fracked sites. A number of positive correlations between amount of well pads within a watershed and THg in crayfish (r = 0.76, P < 0.001), THg in predatory macroinvertebrates (r = 0.71, P < 0.001), and THg in brook trout (r = 0.52, P < 0.01) were observed. Stream-water microbial communities within the Deltaproteobacteria also shared a positive correlation with FTHg and to the number of well pads, while stream pH (r = -0.71, P < 0.001), fish biodiversity (r = -0.60, P = 0.02), and macroinvertebrate taxa richness (r = -0.60, P = 0.01) were negatively correlated with the number of well pads within a watershed. Further investigation is needed to better elucidate relationships and pathways of observed differences in stream water chemistry, biodiversity, and Hg bioaccumulation, however, initial findings suggest Marcellus shale natural gas exploration is having an effect on aquatic ecosystems.

Objectives: To highlight a case series of 3 cases of focal cortical dysplasia that were unrecognized for many years though the patients were seen by various neurologists and received the appropriate neuroimaging studies, and to retrospectively characterize the clinical elements, neuroimaging, electroencephalography, and pathologic findings in these cases., Design: Retrospective descriptive study., Setting: Tertiary urban and suburban neurology and epilepsy outpatient and inpatient clinic settings and hospitals., Patients: We analyze retrospectively 3 patients in whom magnetic resonance images were previously deemed as normal, who, in fact, exhibited subtle gyral abnormalities and who underwent focal surgical resections of these regions after invasive electroencephalography monitoring or electrocorticography and were cured of their epilepsy., Main Outcome Measures: Clinical semiology and neuroimaging findings., Results: Focal cortical dysplasias may present with subtle gyral abnormalities. These gyral abnormalities may guide invasive electroencephalography or electrocorticography and may delineate seizure onsets with precision. Resection of these areas in 3 such patients resulted in excellent surgical outcomes., Conclusions: Subtle gyral abnormalities may be associated with intractable epilepsy and seizure onsets. Focal resection after appropriate evaluations in selected patients may be curative. The magnetic resonance imaging features of focal cortical dysplasia can be subtle and require a high index of suspicion based on ictal semiology and clinical presentation.

This case study with video recording describes a brief neurological examination of a rare post-surgical finding in a patient with intractable seizures who had a right parietal topectomy in order to cure focal and disabling epilepsy. Contemporaneously during the resection and while awake intraoperatively, the patient developed features characterised by involuntary, purposeless, and almost ballistic movements of the contralateral left upper extremity. These involved the shoulder and, more distally, the arm with less involvement of the hand itself and some clonic movement at the elbow, persisting for approximately 24 hours after surgery. Although identified in only one case in our series, we have named the resulting clinical phenomenology "parietal hand syndrome". A Medline search does not reveal any other such case in the English literature with the clinical elements and actual video documentation of neurological examination noted in our case report in the immediate post-operative setting. In this regard, this is a unique clinical report. [Published with video sequences].

Objective: To denote the metabolic imaging localization of seizure onset with ictal and interictal Neurolite single-photon emission computed tomographic scans and difference images made by subtracting activity at individual voxels of these images, which ultimately identify intense focal uptake in the trunk motor region and other, probably secondarily, propagated pathways., Design: Retrospective review of a unique case report., Setting: A suburban epilepsy referral center., Patient: A 59-year-old man with truncal-onset seizure., Interventions: Ictal and interictal metabolic imaging., Main Outcome Measures: Onset location of ictal events., Results: The patient had a gliotic lesion in a focal region on magnetic resonance imaging undercutting the trunk motor area in the cortex of the precentral gyrus with concordant single-photon emission computed tomographic imaging., Conclusions: While truncal-onset seizures have been described previously in a few case reports, they are clinically rare. As far as we know, this is the only case report in the literature in which metabolic imaging was carried out with this entity and is consistent with the anatomical localization of seizure onset in the trunk motor area.

Background: Many factors have been implicated in the etiology of cerebral venous sinus thrombosis (CVT). These include head injury, cancer, infections (sepsis, sinusitis, and mastoiditis), coagulopathies, pregnancy, systemic lupus erythematosus, and dehydration., Case Summary: We present the case of a patient who received long-term estrogen therapy for approximately 15 years after feminizing genitoplasty. The patient experienced a CVT with an excellent clinical outcome. A similar case has not been reported in the literature., Conclusion: Because CVT may be associated with morbidity, mortality, and risks from the complications and treatments of the condition, further research is needed to clarify the factors that may contribute to the long-term risk of CVT in patients receiving long-term estrogen therapy after feminizing genitoplasty., (Copyright (c) 2010 Excerpta Medica Inc. All rights reserved.)