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.

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.