A Role for Electroconvulsive Therapy in the Management of New Onset Refractory Status Epilepticus (NORSE) in a Young Child.

New-onset refractory status epilepticus (NORSE) describes prolonged or recurring new onset seizures which fail to respond to antiseizure medications. NORSE poses a challenge in diagnosis and treatment, and limited high-quality evidence exists to guide management. The efficacy of Electroconvulsive therapy (ECT) in aborting refractory status epilepticus has been described in case reports, but its application remains uncommon, particularly in young children. We describe a case of NORSE in a 3-year old child in which ECT played an important role in aborting status epilepticus, facilitating the diagnosis and surgical excision of an underlying focal cortical dysplasia. Although further research is needed, our case suggests that ECT can be a valuable tool in the treatment of refractory status epilepticus in children.

Robot-assisted stereoelectroencephalography electrode placement in twenty-three pediatric patients: a high-resolution analysis of individual lead placement time and accuracy at a single institution.

PURPOSE: We describe a detailed evaluation of predictors associated with individual lead placement efficiency and accuracy for 261 stereoelectroencephalography (sEEG) electrodes placed for epilepsy monitoring in twenty-three children at our institution. METHODS: Intra- and post-operative data was used to generate a linear mixed model to investigate predictors associated with three outcomes (lead placement time, lead entry error, lead target error) while accounting for correlated observations from the same patients. Lead placement time was measured using electronic time-stamp records stored by the ROSA software for each individual electrode; entry and target site accuracy was measured using postoperative stereotactic CT images fused with preoperative electrode trajectory planning images on the ROSA computer software. Predictors were selected from a list of variables that included patient demographics, laterality of leads, anatomic location of lead, skull thickness, bolt cap device used, and lead sequence number. RESULTS: Twenty-three patients (11 female, 48%) of mean age 11.7 (± 6.1) years underwent placement of intracranial sEEG electrodes (median 11 electrodes) at our institution over a period of 1 year. There were no associated infections, hemorrhages, or other adverse events, and successful seizure capture was obtained in all monitored patients. The mean placement time for individual electrodes across all patients was 6.56 (± 3.5) min; mean target accuracy was 4.5 (± 3.5) mm. Lesional electrodes were associated with 25.7% (95% CI: 6.7-40.9%, p = 0.02) smaller target point errors. Larger skull thickness was associated with larger error: for every 1-mm increase in skull thickness, there was a 4.3% (95% CI: 1.2-7.5%, p = 0.007) increase in target error. Bilateral lead placement was associated with 26.0% (95% CI: 9.9-44.5%, p = 0.002) longer lead placement time. The relationship between placement time and lead sequence number was nonlinear: it decreased consistently for the first 4 electrodes, and became less pronounced thereafter. CONCLUSIONS: Variation in sEEG electrode placement efficiency and accuracy can be explained by phenomena both within and outside of operator control. It is important to keep in mind the factors that can lead to better or worse lead placement efficiency and/or accuracy in order to maximize patient safety while maintaining the standard of care.

Length of stay linked to neurodiagnostic workup for seizures presenting to the pediatric emergency department.

INTRODUCTION: Patients presenting to the pediatric emergency department (ED) often undergo unnecessary testing that leads to prolonged ED visits. Lower ED length of stay has been linked to improved patient experience and may reduce ED overcrowding, a costly burden on a health system. METHODS: This is a retrospective cohort study involving patient records over the period of 6 months at an urban tertiary children's hospital who presented with seizures. Febrile seizures, seizures associated with trauma, and charts of patients who did not present initially through our ED were excluded. RESULTS: 328 charts were obtained through this search criteria. Head imaging was performed in 52 (16%) patients and consisted of 81% CT (n = 42) and 19% (n = 10) magnetic resonance imaging (MRI). Obtaining an MRI was associated with a 3.5 h longer ED visit (p = 0.07); obtaining a CT was associated with a 1.5 h longer ED visit (p = 0.005). An Electroencephalogram (EEG) was obtained for 67 (20%) visits and was associated with a 3.0 h longer ED length of stay (p < 0.001). Ten % of the CT scans showed new or progressive findings and 40% of the MRIs done provided useful information for management. Thirty-seven % of EEGs performed in new onset seizure patients revealed epileptiform findings and 5% of EEGs in established seizure patients provided meaningful findings important to management. CONCLUSION: Obtaining neurodiagnostic studies significantly prolongs duration of stay in the ED. Electroencephalograms appear to have the greatest yield in new onset seizure patients and can help make a diagnosis of an epilepsy syndrome in children.

Improved seizure burden and cognitive performance in a child treated with responsive neurostimulation (RNS) following febrile infection related epilepsy syndrome (FIRES).

Responsive neurostimulation (RNS) is an emerging therapy for patients with refractory focal epilepsy who are not candidates for surgical resection, with limited published experience in the pediatric population. We report a case of refractory multifocal epilepsy following febrile infection related epilepsy syndrome (FIRES) in which surgical resection was not feasible due to multifocal independent seizures and risk of cognitive deficit, and RNS was pursued. Relevant RNS data and neuropsychological testing results were reviewed. By eight months after implantation, decreased frequency and severity of clinical seizures were noted, and RNS data revealed decreased "long episodes," reduced spread of electrographic seizures, and fewer detections. Neuropsychological assessment, though potentially confounded by stimulant medication, revealed significant improvement in multiple cognitive domains, particularly working memory and processing speed, at six months. These findings illustrate success in detecting and aborting seizures, and additionally suggest a neuromodulatory effect of RNS stimulation. Our case demonstrates feasibility, efficacy and safety of RNS in a pediatric patient with FIRES, with evidence to also suggest cognitive improvement.

Implementation of an Intravenous Dihydroergotamine Protocol for Refractory Migraine in Children.

OBJECTIVE: Retrospective chart review to assess the effectiveness of an institutional intravenous (IV) dihydroergotamine (DHE) therapy protocol for refractory migraine in the pediatric population. BACKGROUND: Limited high-quality evidence exists to guide the approach to treatment of refractory migraine with IV DHE, particularly in the pediatric population. This study reviews our institutional experience in implementing an IV DHE protocol in children to identify areas for improvement. We specifically sought to determine whether the outcome differed at follow-up between children who completed the full course of DHE (8 or 9 doses) as specified in our institutional protocol and those who did not. In addition, given the limited Food and Drug Administration-approved treatments for chronic migraine (CM) in the pediatric population, re-evaluating the response rate in this group of patients was of particular interest. METHODS: A retrospective cohort based on a chart review of 159 consecutive pediatric patients who received IV DHE while inpatient at University of Virginia Children's Hospital over a 9-year period (January 2011-January 2019) was identified. Patients were classified according to the International Classification of Headache Disorders, 3rd edition criteria as having CM, status migrainosus, or, in a small number of patients, Other headache with migrainous features. To investigate any benefit of completing the full course of DHE, patients were categorized as having completed the DHE protocol ("Protocol Complete" 8 or 9 doses) or not completing the protocol ("Protocol Incomplete" <8 doses). Patient-reported pain scores upon admission and discharge were recorded, and follow-up outcomes were categorized as headache freedom, >50% relief, <50% relief, or no relief. Pain outcomes were analyzed with respect to DHE protocol complete status and headache classification. RESULTS: A total of 159 patients were included in the analysis. The headache diagnosis was CM in 49% (78/159), status migrainosus in 44% (70/159), and Other headache with migrainous features in 7% (11/159). At discharge, 60% (96/159) of patients achieved headache freedom, and no statistically significant difference was found in relative change in headache among the CM, status migrainosus, and Other headache groups. Patients who completed the full 8 or 9 dose IV DHE protocol were more likely to have persistent headache at discharge, with a median pain score of 1.0 (IQR 0.0-4.0) compared to a score of 0.0 (IQR 0.0-1.0) in the DHE incomplete group (P < .001). No difference was found in pain relief outcomes at follow-up in the DHE protocol complete and DHE protocol incomplete groups. CONCLUSIONS: Although limited by the absence of a control group, our data support repetitive IV DHE as an abortive therapy for pediatric patients with status migrainosus or CM, with no evidence of differential efficacy in these groups. A higher rate of headache at discharge in the DHE protocol complete group reflects in large part the common decision to discontinue treatment once headache freedom was achieved, resulting in nonresponders being more likely to complete the full course of DHE. In our cohort, there was no difference in pain relief at follow-up between patients who completed the full 8 or 9 doses of DHE and those that did not. Discontinuing DHE once the patient has achieved headache freedom would therefore achieve the therapeutic goal while shortening the hospital stay. To potentially impact longer term pain relief, incorporation of a comprehensive treatment approach into the IV DHE admission is of interest for future study and quality improvement initiatives.

Acute Transverse and Flaccid Myelitis in Children.

PURPOSE OF REVIEW: The etiologies of myelitis in children are broad, and our understanding of inflammatory myelopathies in the pediatric population continues to evolve. Acute flaccid myelitis (AFM), increasingly linked to enterovirus infections, has risen in incidence over recent years. As with other infectious myelopathies, AFM can be challenging to distinguish from inflammatory causes of acute transverse myelitis (ATM) at initial presentation. This review outlines an approach to the treatment of children presenting with myelopathy of suspected inflammatory etiology, with attention to how management may differ in the specific case of acute flaccid myelitis. RECENT FINDINGS: Although high-quality evidence is limited, intravenous corticosteroids, intravenous immunoglobulin, and plasma exchange have important roles in the acute management of ATM. Spinal cord injury in AFM, though similar to ATM in clinical presentation, is largely mediated by direct infection as opposed to a primary inflammatory process, and treatment with corticosteroids may worsen outcomes. Awareness of the distinguishing clinical features of AFM and the underlying inflammatory conditions that commonly manifest with ATM is essential to judicious selection of appropriate acute (and potentially chronic) therapies in children presenting with myelitis.

Extreme delta brush and distinctive imaging in a pediatric patient with autoimmune GFAP astrocytopathy.

Autoimmune encephalitis has been increasingly recognized within the pediatric population, and the number of implicated autoantibodies continues to grow. The identification of characteristic clinical and paraclinical features helps direct the evaluation and increases the likelihood of making a definitive diagnosis of a specific antibody-mediated encephalitis. The finding of extreme delta brush on electroencephalogram (EEG) has been suggested to serve as a clinical clue to the diagnosis of anti-NMDA-R encephalitis. Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently described antibody-mediated meningoencephalomyelitis, reported almost exclusively in adult patients. We report a case of autoimmune GFAP astrocytopathy in a pediatric patient with extreme delta brush pattern on EEG, negative anti-NMDA-R antibodies, and distinctive MRI findings. The findings reported herein should prompt clinicians to consider the diagnosis of autoimmune GFAP astrocytopathy in patients with suspected autoimmune encephalitis.

Pediatric Multiple Sclerosis: Genes, Environment, and a Comprehensive Therapeutic Approach.

BACKGROUND: Pediatric multiple sclerosis is an increasingly recognized and studied disorder that accounts for 3% to 10% of all patients with multiple sclerosis. The risk for pediatric multiple sclerosis is thought to reflect a complex interplay between environmental and genetic risk factors. MAIN FINDINGS: Environmental exposures, including sunlight (ultraviolet radiation, vitamin D levels), infections (Epstein-Barr virus), passive smoking, and obesity, have been identified as potential risk factors in youth. Genetic predisposition contributes to the risk of multiple sclerosis, and the major histocompatibility complex on chromosome 6 makes the single largest contribution to susceptibility to multiple sclerosis. With the use of large-scale genome-wide association studies, other non-major histocompatibility complex alleles have been identified as independent risk factors for the disease. The bridge between environment and genes likely lies in the study of epigenetic processes, which are environmentally-influenced mechanisms through which gene expression may be modified. CONCLUSIONS: This article will review these topics to provide a framework for discussion of a comprehensive approach to counseling and ultimately treating the pediatric patient with multiple sclerosis.