ABSTRACT
Surgery is an effective but underused treatment for drug-resistant epilepsy in children. Algorithms to identify surgical candidates and estimate the likelihood of postoperative clinical improvement may be valuable to improve access to epilepsy surgery. We provide a focused review of these approaches. For adults with epilepsy, tools to identify surgical candidates and predict seizure and cognitive outcomes (Ie, Cases for Epilepsy (toolsforepilepsy.com) and Epilepsy Surgery Grading Scale) have been validated and are in use. Analogous tools for children need development. A promising approach is to apply statistical learning tools to clinical datasets, such as electroencephalogram tracings, imaging studies, and the text of clinician notes. Demonstration projects suggest these techniques have the potential to be highly accurate, and await further validation and clinical application.
Subject(s)
Drug Resistant Epilepsy , Epilepsy , Adult , Child , Electroencephalography , Freedom , Humans , Retrospective Studies , Seizures/diagnosis , Seizures/surgery , Treatment OutcomeABSTRACT
PURPOSE: The purpose of the study was to describe epileptologists' opinion on the increased use of remote systems implemented during the COVID-19 pandemic across clinics, education, and scientific meetings activities. METHODS: Between April and May 2020, we conducted a cross-sectional, electronic survey on remote systems use before and during the COVID-19 pandemic through the European reference center for rare and complex epilepsies (EpiCARE) network, the International and the French Leagues Against Epilepsy, and the International and the French Child Neurology Associations. After descriptive statistical analysis, we compared the results of France, China, and Italy. RESULTS: One hundred and seventy-two respondents from 35 countries completed the survey. Prior to the COVID-19 pandemic, 63.4% had experienced remote systems for clinical care. During the pandemic, the use of remote clinics, either institutional or personal, significantly increased (pâ¯<â¯10-4). Eighty-three percent used remote systems with video, either institutional (75%) or personal (25%). During the pandemic, 84.6% of respondents involved in academic activities transformed their courses to online teaching. From February to July 2020, few scientific meetings relevant to epileptologists and routinely attended was adapted to virtual meeting (median: 1 [25th-75th percentile: 0-2]). Responders were quite satisfied with remote systems in all three activity domains. Interestingly, before the COVID-19 pandemic, remote systems were significantly more frequently used in China for clinical activity compared with France or Italy. This difference became less marked during the pandemic. CONCLUSION: The COVID-19 pandemic has dramatically altered how academic epileptologists carry out their core missions of clinical care, medical education, and scientific discovery and dissemination. Close attention to the impact of these changes is merited.
Subject(s)
Attitude of Health Personnel , Coronavirus Infections , Education, Distance/trends , Epilepsy/therapy , Neurologists , Pandemics , Pneumonia, Viral , Telemedicine/trends , Adult , Africa , Aged , Asia , Betacoronavirus , COVID-19 , China , Computer Security , Confidentiality , Cross-Sectional Studies , Europe , Female , France , Humans , Italy , Male , Middle Aged , Neurology , North America , Practice Patterns, Physicians' , Remote Consultation/trends , SARS-CoV-2 , South America , Surveys and QuestionnairesABSTRACT
OBJECTIVE: To describe the prevalence and characteristics of comorbidities in persons with rare epilepsies. STUDY DESIGN: Persons with rare epilepsies and caregivers of those affected were recruited through the Epilepsy Foundation and more than 30 rare epilepsy advocacy organizations affiliated with the Rare Epilepsy Network (REN). A web-based survey was conducted using a questionnaire consisting of core sections to collect data from affected persons on various aspects, including comorbidities. Comorbidity information was grouped into 15 classes, 12 of which had a stem question followed by detailed branch questions and 3 that were created from a combination of related questions. RESULTS: Of 795 persons with more than 30 different rare epilepsy diagnosis groups, one-half had ≥5 comorbidity classes and 97% were classified as complex chronic disease (C-CD). The highest number of comorbidity classes reported per person were persons with Aicardi syndrome, Phelan-McDermid syndrome (median, 7.0; IQR, 5.0-9.0), and tuberous sclerosis complex (median, 6.0; IQR, 4.0-8.0). The most common comorbidity classes were learning/developmental disability (71%), mental health issues (71%), sleep disorders (60%), brain abnormalities (52%), oral issues (49%), bone-joint issues (42%), hyper/hypotonia (42%), and eye-vision disorders (38%). The prevalence of brain abnormalities, hyper/hypotonia, eye, and cardiac disorders was significantly higher in persons first diagnosed with epilepsy at a younger age (<9 months) than in those first diagnosed at an older age (P < .05 for trend). CONCLUSIONS: Nearly all persons with rare epilepsies are medically complex, with a high prevalence of multiple comorbidities, especially those who were diagnosed with epilepsy in the first year of life. Comorbidities should be carefully considered in the diagnosis and management of persons with rare epilepsies.
Subject(s)
Developmental Disabilities/diagnosis , Developmental Disabilities/epidemiology , Epilepsy/classification , Epilepsy/epidemiology , Surveys and Questionnaires , Adolescent , Age Factors , Child , Comorbidity , Cross-Sectional Studies , Databases, Factual , Epilepsy/diagnosis , Female , Humans , Information Services , Intellectual Disability/diagnosis , Intellectual Disability/epidemiology , Learning Disabilities/diagnosis , Learning Disabilities/epidemiology , Male , Prevalence , Prognosis , Rare Diseases , Reproducibility of Results , Severity of Illness Index , Sex Factors , United States/epidemiologyABSTRACT
OBJECTIVE: Infantile spasms are seizures associated with a severe epileptic encephalopathy presenting in the first 2 years of life, and optimal treatment continues to be debated. This study evaluates early and sustained response to initial treatments and addresses both clinical remission and electrographic resolution of hypsarrhythmia. Secondarily, it assesses whether response to treatment differs by etiology or developmental status. METHODS: The National Infantile Spasms Consortium established a multicenter, prospective database enrolling infants with new diagnosis of infantile spasms. Children were considered responders if there was clinical remission and resolution of hypsarrhythmia that was sustained at 3 months after first treatment initiation. Standard treatments of adrenocorticotropic hormone (ACTH), oral corticosteroids, and vigabatrin were considered individually, and all other nonstandard therapies were analyzed collectively. Developmental status and etiology were assessed. We compared response rates by treatment group using chi-square tests and multivariate logistic regression models. RESULTS: Two hundred thirty infants were enrolled from 22 centers. Overall, 46% of children receiving standard therapy responded, compared to only 9% who responded to nonstandard therapy (p < 0.001). Fifty-five percent of infants receiving ACTH as initial treatment responded, compared to 39% for oral corticosteroids, 36% for vigabatrin, and 9% for other (p < 0.001). Neither etiology nor development significantly modified the response pattern by treatment group. INTERPRETATION: Response rate varies by treatment choice. Standard therapies should be considered as initial treatment for infantile spasms, including those with impaired development or known structural or genetic/metabolic etiology. ACTH appeared to be more effective than other standard therapies.