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We surveyed ketogenic diet centers in North America about their practices surrounding the ketogenic diet. An internet survey was disseminated via REDCap© to North American ketogenic diet centers identified from the Charlie Foundation and Ketocal© websites. Fifty-six centers responded. In addition to physicians, nurses and dieticians, ketogenic teams included social workers (39%), feeding specialists (14%), educational liaisons (4%), psychologists (5%), and pharmacists (36%). A child attending school (2%), non–English speaking family (19%), single-parent family (0%), and oral feeding (6%) were rarely considered barriers. Overall, the diet was considered the first or second (0%), third or fourth (67%), fifth or sixth (29%), and last resort treatment (4%) by centers. It was considered the first or second treatment for GLUT1 disease (86%) and third or fourth for Dravet (63%), West (71%), and Doose (65%) syndromes. Ketogenic diet is no longer a last resort option. Traditional barriers do not influence its use. [ABSTRACT FROM PUBLISHER]

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. [ABSTRACT FROM AUTHOR]

We conducted an online survey of elementary school teachers in Washtenaw County, Michigan, regarding their confidence in their knowledge of epilepsy and their preferred media or sources of information about epilepsy. Eighty-three teachers (9.3%) responded. One quarter expressed a lack of confidence in their ability to teach students with epilepsy or to respond appropriately to a seizure. Teachers most frequently (68%) cited the Internet as their primary source of information about epilepsy, with the school nurse and parents/guardians also frequently mentioned (55% and 48%, respectively). In contrast, most respondents prefer that their information come from the school nurse (74%) or a physician (73%), while only 25% cited the Internet as a preferred source. Teachers most frequently indicated EpilepsyFoundation.org (70.5%) as a trusted source of information. Future collaborative education efforts between school nurses and physicians, especially through use of the Internet, could improve teachers’ knowledge of epilepsy. [ABSTRACT FROM AUTHOR]

Objective The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without hypsarrhythmia. Methods Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of hypsarrhythmia were determined clinically at each site. Response to therapy was defined as resolution of clinical spasms (and hypsarrhythmia if present) without relapse 3 months after initiation. Results Eighty-two percent of patients had hypsarrhythmia, but this was not associated with gender, mean age, preexisting developmental delay or epilepsy, etiology, or response to first-line therapy. Infants with hypsarrhythmia were more likely to receive standard treatment (adrenocorticotropic hormone, prednisolone, or vigabatrin [odds ratio ( OR) 2.6, 95% confidence interval ( CI) 1.4-4.7] and preexisting epilepsy reduced the likelihood of standard treatment ( OR 3.2, 95% CI 1.9-5.4). Hypsarrhythmia was not a determinant of response to treatment. A logistic regression model demonstrated that later age of onset ( OR 1.09 per month, 95% CI 1.03-1.15) and absence of preexisting epilepsy ( OR 1.7, 95% CI 1.06-2.81) had a small impact on the likelihood of responding to the first-line treatment. However, receiving standard first-line treatment increased the likelihood of responding dramatically: vigabatrin ( OR 5.2 ,95% CI 2-13.7), prednisolone ( OR 8, 95% CI 3.1-20.6), and adrenocorticotropic hormone (ACTH; OR 10.2, 95% CI 4.1-25.8) . Significance First-line treatment with standard therapy was by far the most important variable in determining likelihood of response to treatment of infantile spasms with or without hypsarrhythmia. [ABSTRACT FROM AUTHOR]

Objective: There are no evidence-based guidelines on the preferred approach to treating early-life epilepsy. We examined initial therapy selection in a contemporary US cohort of children with newly diagnosed, nonsyndromic, early-life epilepsy (onset before age three years).Methods: Seventeen pediatric epilepsy centers participated in a prospective cohort study of children with newly diagnosed epilepsy with onset under 36 months of age. Details regarding demographics, seizure types, and initial medication selections were obtained from medical records.Results: About half of the 495 enrolled children with new-onset, nonsyndromic epilepsy were less than 12 months old at the time of diagnosis (n = 263, 53%) and about half (n = 260, 52%) had epilepsy with focal features. Of 464 who were treated with monotherapy, 95% received one of five drugs: levetiracetam (n = 291, 63%), oxcarbazepine (n = 67, 14%), phenobarbital (n = 57, 12%), topiramate (n = 16, 3.4%), and zonisamide (n = 13, 2.8%). Phenobarbital was prescribed first for 50 of 163 (31%) infants less than six months old versus seven of 300 (2.3%) of children six months or older (P < 0.0001). Although the first treatment varied across study centers (P < 0.0001), levetiracetam was the most commonly prescribed medication regardless of epilepsy presentation (focal, generalized, mixed/uncertain). Between the first and second treatment choices, 367 (74%) of children received levetiracetam within the first year after diagnosis.Conclusions: Without any specific effort, the pediatric epilepsy community has developed an unexpectedly consistent approach to initial treatment selection for early-life epilepsy. This suggests that a standard practice is emerging and could be utilized as a widely acceptable basis of comparison in future drug studies. [ABSTRACT FROM AUTHOR]

Purpose Lennox- Gastaut syndrome ( LGS) is a devastating childhood-onset epilepsy syndrome. The cause is unknown in 25% of cases. Little has been described about the specific clinical or electroencephalography ( EEG) features of LGS of unknown or genetic cause ( LGSu). The Epilepsy Phenome/ Genome Project ( EPGP) aims to characterize LGSu by phenotypic analysis of patients with LGSu and their parents. Methods One hundred thirty-five patients with LGS with no known etiology and their parents were enrolled from 19 EPGP centers in the United States and Australia. Clinical data from medical records, standardized questionnaires, imaging, and EEG were collected with use of online informatics systems developed for EPGP. Key Findings LGSu in the EPGP cohort had a broad range of onset of epilepsy from 1 to 13 years, was male predominant (p < 0.0002), and was associated with normal development prior to seizure onset in 59.2% of patients. Despite the diagnosis, almost half of the adult patients with LGSu completed secondary school. Parents were cognitively normal. All subjects had EEG recordings with generalized epileptiform abnormalities with a spike wave frequency range of 1-5 Hz (median 2 Hz), whereas 8.1% of subjects had EEG studies with a normal posterior dominant rhythm. Almost 12% of patients evolved from West syndrome. Significance LGSu has distinctive characteristics including a broad age range of onset, male predominance, and often normal development prior to the onset of seizures. Cognitive achievements such as completion of secondary school were possible in half of adult patients. Our phenotypic description of LGSu coupled with future genetic studies will advance our understanding of this epilepsy syndrome. [ABSTRACT FROM AUTHOR]

Abstract: Treatment adherence is often suboptimal among adolescents with epilepsy. However, knowledge is lacking regarding factors that affect adherence. Empirical studies and theories of human development suggest that self-management skills, self-efficacy, and sense of control are related to adherence. Eighty-eight adolescents with epilepsy, and their parents, completed standardized measures assessing epilepsy knowledge and expectations, treatment self-management, sense of control, and self-efficacy. Better self-reported parent adherence was correlated with greater epilepsy knowledge/expectations (p<0.001) and more medications (p=0.042). Better self-reported adolescent adherence was correlated with fewer siblings (p=0.003) and higher adolescent epilepsy knowledge/expectations (p<0.001). Greater adolescent epilepsy knowledge/expectations correlated with parent self-reported adherence (p<0.001), Powerful others locus of control (p=0.008), and adolescent/parent discordance regarding epilepsy knowledge/expectations (p<0.001). Interventions that enhance adolescent''s knowledge of epilepsy and their treatment plan, while ensuring that teens and parents are in agreement with regard to epilepsy treatment, might contribute to better adherence. [Copyright &y& Elsevier]

Keywords: Genetic epilepsy; SCN1B; Sodium channel; Generalized epilepsy with febrile seizures plus EN Genetic epilepsy SCN1B Sodium channel Generalized epilepsy with febrile seizures plus 121 122 2 06/19/20 20200701 NES 200701 Patient Description This child experienced a febrile seizure at age 26 months. His cvEEG at 31 months demonstrated myoclonic seizures occurring two to three times per hour, atonic seizures occurring twice a day, and bilateral tonic seizures occurring once a day. Discussion SCN1B was the first gene identified for genetic epilepsy with febrile seizures plus,[1] which spans a range of phenotypes including epilepsy with myoclonic-atonic seizures. [Extracted from the article]