Cognitive, behavioral, and social functioning in children and adults with Dravet syndrome.

Aim: The objective of the study was to delineate the cognitive, behavioral, psychological, and social functioning of individuals with Dravet syndrome.

Method: Cognitive, behavioral, and social functioning were assessed in patients with Dravet syndrome by comprehensive, age-appropriate standardized neuropsychological testing. Primary caregivers completed standardized measures regarding participants' behavior, psychological status, adaptive functioning, and social skills, including their involvement with intervention services.

Pediatric epilepsy surgery patients show normal psychosocial development at long-term follow-up despite dissatisfying family dynamics.

Drug-resistant pediatric epilepsy involves unpredictable seizures and long-term medical management. Both factors can alter a child's psychosocial development and the dynamics of the family, to the detriment of patient and family wellbeing. While drug-resistant pediatric epilepsy can be successfully treated by neurosurgery in some cases, the outlook for psychosocial and family functioning after surgery remains unclear.

Dominant KCNA2 mutation causes episodic ataxia and pharmacoresponsive epilepsy.

Objective: To identify the genetic basis of a family segregating episodic ataxia, infantile seizures, and heterogeneous epilepsies and to study the phenotypic spectrum of KCNA2 mutations.

Methods: A family with 7 affected individuals over 3 generations underwent detailed phenotyping. Whole genome sequencing was performed on a mildly affected grandmother and her grandson with epileptic encephalopathy (EE).

Developmental outcomes of childhood-onset temporal lobe epilepsy: a community-based study.

Purpose: To assess the impact of childhood-onset temporal lobe epilepsy (TLE) on the attainment of normative developmental tasks and identify predictors of long-term developmental outcomes.

Methods: In 1992-1993, a prospective longitudinal cohort study of childhood-onset TLE was commenced in the State of Victoria, Australia. At review in 2004-2006, we assessed developmental tasks, which are age-specific individual psychosocial achievements tied to particular phases of the lifespan.

Psychological outcome profiles in childhood-onset temporal lobe epilepsy.

Purpose: To examine the effect of childhood-onset temporal lobe epilepsy (TLE) on long-term psychological function and to identify outcome profiles related to the natural course and treatment of TLE.

Methods: Psychological function was studied in a prospective, community-based cohort of childhood-onset TLE, approximately 13 years following seizure onset. Fifty-three patients were assessed using a semi-structured psychosocial interview, supplemented by self-report questionnaires measuring quality-of-life, depression, self-esteem, and anxiety.