Responsive Vagus Nerve Stimulation for Drug Resistant Epilepsy: A Review of New Features and Practical Guidance for Advanced Practice Providers.

Vagus nerve stimulation (VNS) is a safe and effective therapy that has been available for over 20 years for adults and children with drug resistant epilepsy (DRE). Since U.S. Food and Drug Administration approval in 1997, VNS has been implanted in over 100,000 patients including over 30,000 children as an adjunctive therapy in reducing the frequency of seizures in patients 4 years of age and older with focal seizures that are refractory to antiseizure medications. VNS Therapy® has evolved over time and currently offers closed-loop, responsive stimulation as well as advanced features that streamline dosing and patient management. Advanced Practice Providers (APPs) such as nurse practitioners, physician assistants and clinical nurse specialists are integral in a comprehensive healthcare team, and dedicated VNS clinics have formed at comprehensive epilepsy centers across the world that are often managed by APPs. This approach improves access, education, and continuity of care for those with VNS or those considering VNS. Here we provide a review for APPs on the VNS Therapy® system focused on new features, dosing, and troubleshooting strategies with the goal to provide guidance to those managing VNS patients.

Seizure Outcomes in Children Following Electrocorticography-Guided Single-Stage Surgical Resection.

BACKGROUND: In children with abnormal imaging, single-stage epilepsy surgery is an attractive alternative to the two-stage approach that relies on invasive recording of seizures. Implanted electrodes carry risks of their own and extend hospitalization, but the efficacy of one-stage resections in a variety of pathologies and cerebral locations is not well established. We report our center's experience with single-stage epilepsy surgery guided by intraoperative electrocorticography (ECoG). METHODS: We retrospectively analyzed 130 consecutive patients who underwent single-stage epilepsy surgery before age 19 years and had at least a two-year follow-up. Intraoperative ECoG was available for review in 113. Patients were considered seizure-free if they were continuously Engel Class I up to the two-year postoperative mark. ECoG findings were classified according to the presence of interictal attenuation, spikes, both, or neither. Complications and hospital length of stay were evaluated. RESULTS: Eighty percent of 130 patients were seizure-free at two years. All but one had an abnormal MRI. Patients with tumor had a better seizure outcome than patients with cortical malformation. Frontal resections had worse outcome, especially among tumors. Intraoperative ECoG revealed both attenuation and spikes in 48%, attenuation only in 23%, spikes only in 20%, and neither in 9%. The complication rate was 6.9%, with no major neurological complications. The average length of stay was 5.7 nights. CONCLUSIONS: With ECoG-guided single-stage surgery, we achieved results comparable with other pediatric surgical series and with a low complication rate. An extensive two-stage approach may not be required when there is a lesion on imaging and other information is concordant, even when the MRI abnormality is subtle and unclearly delineated. Frontal foci may present a challenge because of their proximity to "eloquent" nonresectable cortex or critical structures.

A simple behavioral-developmental checklist versus formal screening for children in an epilepsy center.

We compare a 7-item checklist for developmental and behavioral concerns to formal screening with the Ages & Stages Questionnaires, Third Edition (ASQ-3) in children aged 1-5.5years old seen in a tertiary epilepsy care setting. The checklist was derived from a 35-item parent questionnaire used as a routine component of pediatric neuropsychology evaluations at our center. In our sample of 104 children, having even one checklist item endorsed was associated with a 97% (66/68) positive predictive value for screening positive on the ASQ-3. Fifty percent of children for whom no items were endorsed on the checklist scored negative for developmental concerns on the ASQ-3. The overall sensitivity of the checklist to a positive ASQ-3 screen was 83%, and the specificity was 88%. Use of this simple checklist may facilitate screening in the outpatient epilepsy care setting where time and resources are often limited.

Routine developmental, autism, behavioral, and psychological screening in epilepsy care settings.

AIM: Screening for cognitive impairment, developmental delay, and neuropsychiatric problems is not always performed in children with epilepsy. The aim of this study was to assess the value of this screening and its validity for determining previously unidentified ('actionable') problems in children with epilepsy. METHOD: New and existing patients with epilepsy were recruited from a hospital-based epilepsy center. The parent of the child completed screening evaluations for development (Ages and Stages Questionnaire [ASQ], 0-66mo), autism (Modified Checklist for Autism in Toddlers [mCHAT], 16-30mo), social communication (Social Communication Questionnaire [SCQ], ≥4y), and psychiatric concerns (Strengths and Difficulties Questionnaire [SDQ], 4-17y). RESULTS: We screened 236 children overall (136 males [58%], 100 females [42%]; mean age [SD] 6y 7mo [4y 6mo]). Of these, 176 children (75%) had established epilepsy diagnoses and 60 (25%) were patients with new-onset epilepsy. Of those with new-onset disease, 22 (37%) were determined not to have epilepsy. Positive findings by test were 82% (ASQ), 54% (mCHAT), 15%, (SCQ), and 58% (SDQ). Findings were actionable in 46 children (20%): 18% of findings in children with established epilepsy and 23% of findings in patients with new-onset epilepsy. Of the 46 children for whom further referrals were made, the parents of 28 (61%) have pursued further evaluations. INTERPRETATION: In this study, children with existing and new-onset diagnoses of epilepsy had actionable screening findings. These findings support the development of systematic screening of comorbidities for children with epilepsy.

Routine developmental and autism screening in an epilepsy care setting.

Developmental delay (delay) and co-morbidities like autism are common in children with epilepsy. We assessed the yield of routine screening for delay and autism in a hospital-based program. Parents completed developmental and autism screeners for 65 children (average age=2.5y; 38(58%) boys). Forty-nine (75%) were established epilepsy patients, and 16 (25%) were new patients. For development, 47 (72%) children screened positive and 8 (12%) had borderline results. Twenty-four (37%) scored positive for autism, all of whom also screened positive for developmental delay. Delays and neurologic deficits accounted for the positive autism results in 20 of the 24. Developmental findings were confirmatory (already receiving services) in 32/55 (58%) children and actionable in 17 (31%) (requiring further evaluation). Referrals for further evaluations were made for most with actionable findings. The yield of routine screening of children in a tertiary center is sufficiently high to support its use and to consider screening of all children seen with epilepsy.