A Multicenter Study of Adherence to Best Practices and Application of Epilepsy Quality Metrics in a Pediatric Telemedicine Encounter.

Objective: To assess Epilepsy Quality Metrics (EQM) and guideline implementation in new pediatric patients seen in telemedicine. Methods: Multicenter, cross sectional, retrospective analysis. Results: Patients were similar across 3 centers for age, gender, and insurance type. Eighty-one percent presented for spells. One hundred sixty patients with epilepsy formed the EQM cohort. Results: Seizures described: 95%; frequency: 67%, last seizure documented: 81%, epilepsy syndrome documented: 67%; epilepsy diagnosis: 77%, medications reviewed: 56%, adverse events discussed: 73%. Quality of life discussed: 3%. Anticipatory guidance was described as follows: seizure safety, 57%; driving, 47%; SUDEP, 11%; vitamin D discussion, 19%; pregnancy and folic acid counseling, 4% and 10%. Epileptologists were 4 times as likely as generalists in discussing driving safety (odds ratio 3.93, 95% confidence interval 1.7-8.9; P = .001) for all ages. Significance: Performance on EQM and guideline implementation in pediatric epilepsy telemedicine encounters can be improved.

Semiautomated Spike Detection Software Persyst 13 Is Noninferior to Human Readers When Calculating the Spike-Wave Index in Electrical Status Epilepticus in Sleep.

PURPOSE: Our objective was to use semiautomatic methods for calculating the spike-wave index (SWI) in electrical status epilepticus in slow-wave sleep (ESES) and to determine whether this calculation is noninferior to human experts (HEs). METHODS: Each HE marked identical 300-second epochs for all spikes and calculated the SWI in sleep EEGs of patients diagnosed with ESES. Persyst 13 was used to mark spikes (high sensitivity setting) in the same 300-second epochs marked by HEs. The spike-wave index was calculated. Pairwise HE differences and pairwise Persyst 13 (P13)-HE differences for the SWI were calculated. Bootstrap resampling (BCa, N = 3,000) was performed to better estimate mean differences and their 95% confidence bounds between HE and P13-HE pairs. Potential noninferiority of P13 to HEs was tested by comparing the 95% confidence bounds of the mean differences between pairs for the SWI. RESULTS: Twenty EEG records were analyzed. Each HE marked 100 minutes of EEG. HEs 1, 2, 3, and 4 marked 10,075, 8,635, 9,710, and 9,898 spikes, respectively. The highest and lowest 95% confidence bound of the mean difference in the SWI between HE pairs was: High: 10.3%; Low: -10.2%. Highest and lowest 95% confidence bound of the mean difference in the SWI between P13 and HE pairings was as follows: high, 9.5% and low, -6.7%. The lack of a difference between P13 and HEs supports that the algorithm is not inferior to HEs. CONCLUSIONS: Persyst 13 is noninferior to HEs in calculating the SWI in ESES, thus suggesting that an automated approach to SWI calculation may be a useful clinical tool.

Management of Status Epilepticus in Children.

Status epilepticus is a common pediatric neurological emergency. Management includes prompt administration of appropriately selected anti-seizure medications, identification and treatment of seizure precipitant(s), as well as identification and management of associated systemic complications. This review discusses the definitions, classification, epidemiology and management of status epilepticus and refractory status epilepticus in children.

Generalized epilepsies: immunologic and inflammatory mechanisms.

This article focuses on the inflammatory processes in patients with generalized epilepsies. We specifically review the data regarding West, Lennox-Gastaut, and Landau-Kleffner syndromes as they have generalized clinical or electroencephalogram features. There is substantial evidence for a pathogenic implication of immune mechanisms in these epilepsies. Animal models and abnormalities in both cellular and humoral immunity support this hypothesis. They also appear to be particularly responsive to immunomodulatory therapies, which has raised the speculation that an unbalanced immune system may play an important role in the pathophysiology of these epileptic syndromes. In this article, we discuss clinical and experimental data that support the potential implication of immune mediated inflammation and immune response in the mechanism of these entities.

Ring chromosome 20: a pediatric potassium channelopathy responsive to treatment with ezogabine.

BACKGROUND: Ring chromosome 20 is a genetic disorder characterized by intractable epilepsy, behavioral problems, and cognitive deficit. The potassium channel-coding gene KCNQ2 is localized at the locus q13.3 on the chromosome 20, the most common site where the ring occurs. Ezogabine is the first potassium channel opener marketed in the United States. PATIENTS: We describe an 8-year-old girl with mosaic ring chromosome 20 and refractory epilepsy who had a remarkable improvement in seizure control with ezogabine. CONCLUSIONS: This is the first report using the new antiepileptic drug ezogabine to treat pediatric epilepsy. We hypothesize that ring chromosome 20 patients have epilepsy related to abnormalities in the potassium channels, making it susceptible for treatment with potassium channel openers.