Self-reported quality of life and degree of youth-parent agreement: A long-term follow-up of childhood-onset epilepsy.

Objective: To prospectively delineate self-reported health-related quality of life (HRQOL) of adolescents and young adults (AYAs) 8 and 10 years after an epilepsy diagnosis and evaluate the degree of AYA-parent agreement in ratings of AYA's HRQOL.

Methods: Data came from the Health-Related Quality of Life in Children with Epilepsy Study (HERQULES), a 10-year longitudinal study of children, aged 4-12 years, with newly diagnosed epilepsy. Epilepsy-specific HRQOL was self-reported by AYA 8 and 10 years after diagnosis and by parents at multiple time points throughout the 10-year follow-up.

Trajectories of quality of life 10 years following a diagnosis of epilepsy in childhood.

Objective: This study estimated trajectories of health-related quality of life (HRQOL) over a 10-year period among children newly diagnosed with epilepsy. We also modeled the characteristics of children, parents, and families associated with each identified trajectory.

Methods: Data came from the HERQULES (Health-Related Quality of Life in Children With Epilepsy Study), a Canada-wide prospective cohort study of children (aged 4-12 years) with newly diagnosed epilepsy.

Intractable seizures after a lengthy remission in childhood-onset epilepsy.

Objectives: To establish the risk of subsequent intractable epilepsy after ≥2, ≥5, and ≥10 years of remission in childhood-onset epilepsy.

Methods: From the Nova Scotia childhood-onset epilepsy population-based cohort patients with all types of epilepsy were selected with ≥20 years follow-up from seizure onset (incidence cases). Children with childhood absence epilepsy were excluded.

Treatment issues for children with epilepsy transitioning to adult care.

This is the third of three papers that summarize the second symposium on Transition in Epilepsies held in Paris in June 2016. This paper focuses on treatment issues that arise during the course of childhood epilepsy and make the process of transition to adult care more complicated. Some AEDs used during childhood, such as stiripentol, vigabatrin, and cannabidiol, are unfamiliar to adult epilepsy specialists.

What do epileptologists recommend about discontinuing antiepileptic drugs for a second time in children?

Objective: There is a broad consensus that antiepileptic drugs (AEDs) may be withdrawn after two years of seizure freedom for most children with epilepsy. If seizures recur and are, again, completely controlled with AEDs, little is known about discontinuing a second time. We surveyed American and Canadian pediatric epileptologists to understand their current practice.

Transition issues for benign epilepsy with centrotemporal spikes, nonlesional focal epilepsy in otherwise normal children, childhood absence epilepsy, and juvenile myoclonic epilepsy.

This chapter covers the syndromes of benign epilepsy with centrotemporal spikes (BECTS), nonlesional focal epilepsy in otherwise normal children (NLFN), and the genetic generalized epilepsies. BECTS is an epilepsy syndrome that always enters terminal remission before the general age of a planned transition of adolescents. This is also the case for the majority (65%) of those with childhood absence epilepsy (CAE).

Epidemiologic aspects: lost in transition.

Population-based studies focusing on the long-term prognosis of childhood-onset epilepsy show that despite seizure remission in 70-80% of cases, cognitive, behavioral and psychosocial complications are common and will require management and monitoring in adulthood. This type of study design also demonstrates that death is rare in children who are intellectually and neurologically normal and followed for many years, which is the same for the general population. Only those children with neurologic problems sufficiently severe to interfere with activities of daily living have an increased risk of death in childhood.

Convulsive status epilepticus and health-related quality of life in children with epilepsy.

Objectives: The objective of this study was to examine the association between convulsive status epilepticus (CSE) and health-related quality of life (HRQL) during a 24-month follow-up in a multisite incident cohort of children with epilepsy.

Methods: Data were collected in the Health-Related Quality of Life Study in Children with Epilepsy Study from 374 families of children with newly diagnosed epilepsy. The Quality of Life in Childhood Epilepsy (QOLCE) Questionnaire was used to evaluate parent-reported child HRQL.

What happens to children with epilepsy when they become adults? Some facts and opinions.

Background: The adult outcome after childhood onset epilepsy is a complex subject because seizure types and severity are diverse, comorbidities are common, and additional factors influence social outcome. We review selected data about seizure remission or persistence and social outcome in adulthood.

Methods: Information came from published literature, especially population-based studies.

Rolandic epilepsy has little effect on adult life 30 years later: a population-based study.

Objective: To establish the adult social outcome for childhood-onset rolandic epilepsy.

Methods: Patients with medication-treated rolandic epilepsy were identified from the Nova Scotia prospective population-based cohort of childhood-onset epilepsy. Epilepsy onset was in 1977-1985 and follow-up was in 2010-2013 with chart review plus structured telephone interview for those older than 21 years.

Trajectories of health-related quality of life in children with epilepsy: a cohort study.

Purpose: Little is known about subgroups of children with epilepsy who may experience less favorable outcomes over time. The objectives of this study were to document trajectories of health-related quality of life (HRQL) and to identify predictors of the trajectory group in children with new-onset epilepsy.

Methods: Data were obtained from the Health Related Quality of Life in Children with Epilepsy Study, a prospective multisite study of children 4-12 years old with new-onset epilepsy followed for 24 months.

Lifetime prognosis of juvenile myoclonic epilepsy.

Juvenile myoclonic epilepsy (JME) is among the most common types of genetic epilepsies, displaying a good prognosis when treated with appropriate drugs, but with a well-known tendency to relapse after withdrawal. The majority of patients with JME have continuing seizures after a follow-up of two decades. However, 17% are able to discontinue medication and remain seizure-free thereafter.

Epidemiology of juvenile myoclonic epilepsy.

Juvenile myoclonic epilepsy (JME) is a widely recognized presumed genetic, electroclinical idiopathic generalized epilepsy syndrome. The prevalence of JME in large cohorts has been estimated to be 5% to 10% of all epilepsies and around 18% of idiopathic generalized epilepsies but may be lower in some settings. There is a marked female predominance.

Mortality risks in new-onset childhood epilepsy.

Objectives: Estimate the causes and risk of death, specifically seizure related, in children followed from onset of epilepsy and to contrast the risk of seizure-related death with other common causes of death in the population.

Methods: Mortality experiences from 4 pediatric cohorts of newly diagnosed patients were combined. Causes of death were classified as seizure related (including sudden unexpected death [SUDEP]), natural causes, nonnatural causes, and unknown.

The adult seizure and social outcomes of children with partial complex seizures.

Most intellectually normal children with focal epilepsy have partial complex or focal with secondary generalization seizures without a precise epilepsy syndrome. Their long-term outcome is largely unknown. Cases were identified from the population-based Nova Scotia Childhood Epilepsy cohort.

Quality of life in children with new-onset epilepsy: a 2-year prospective cohort study.

Objectives: To assess health-related quality of life (HRQL) over 2 years in children 4-12 years old with new-onset epilepsy and risk factors.

Methods: Data are from a multicenter prospective cohort study, the Health-Related Quality of Life Study in Children with Epilepsy Study (HERQULES). Parents reported on children's HRQL and family factors and neurologists on clinical characteristics 4 times.

Does Dravet syndrome have a recognizable face?

We hypothesized that children with Dravet syndrome manifest specific facial features that can be identified by pediatric neurologists and rendered objective by standard photographic measurements. This study comprised two parts. In Part 1, photographs of children with Dravet syndrome were compiled into a booklet with patients and their siblings randomly mixed.

Pain disrupts sleep in children and youth with intellectual and developmental disabilities.

Both chronic pain and sleep problems are common for children with intellectual and developmental disabilities (IDD). Although one study has revealed a relationship between having a medical condition and sleep problems in this population, the role of pain was not examined independently. Thus, the goal of this study was to clarify the specific role of pain in children's sleep problems.

Dietary therapies for epilepsy: future research.

Since 1921, dietary therapies have remained valuable options in the treatment of intractable childhood epilepsy. The traditional ketogenic diet has been well demonstrated, including in a recent randomized, controlled trial, as being highly effective. More recent alternative diets such as the medium-chain triglyceride diet, modified Atkins diet, and low-glycemic-index treatment have expanded the use of this modality to more children as well as adults.

The relation between children's pain behaviour and developmental characteristics: a cross-sectional study.

Aim: To determine whether children with developmental disabilities show responses to pain that vary according to developmental level.

Method: Factor analytical methods were used to explore whether pain behaviour is independent of developmental characteristics. As part of a longitudinal study, caregivers of 123 children (67 males, 56 females; age range 40 mo-21 y 6 mo) completed the Non-communicating Children's Pain Checklist-Revised (NCCPC-R), the Vineland Adaptive Behavior Scales, Second Edition (VABS-II), and the Pediatric Evaluation of Disability Inventory (PEDI).

Juvenile myoclonic epilepsy 25 years after seizure onset: a population-based study.

Objective: To document the long-term evolution of juvenile myoclonic epilepsy (JME) in a population-based cohort.

Methods: All patients developing JME by 16 years of age in Nova Scotia between 1977 and 1985 were contacted in 2006-2008.

Results: Twenty-four patients (17 women) had JME, 3.

Stigma of epilepsy.

Epilepsy directly affects 50 million people worldwide. Most can achieve excellent seizure control; however, people living with epilepsy continue to suffer from enacted or perceived stigma that is based on myths, misconceptions and misunderstandings that have persisted for thousands of years. This paper reviews the frequency and nature of stigma toward epilepsy.

Coping with a child with Dravet syndrome: insights from families.

Dravet syndrome is a truly catastrophic childhood-onset epilepsy. Families are faced with repeated episodes of status epilepticus, intractable seizures, mental handicap, behavior disorders, and a life of dependency; understandably, coping with Dravet syndrome is very difficult. Twenty-four families with a child with Dravet syndrome were interviewed and identified some practical suggestions to improve their daily life.