Sharon Whiting

Epilepsy surgery in children with tuberous sclerosis complex: presurgical evaluation and outcome.

Summary: Purpose: Children with tuberous sclerosis complex (TSC) benefit from excisional surgery if seizures can be localized to a single tuber. We evaluated the role of noninvasive studies to localize the epileptogenic tuber/region (ET/R) and the outcome of focal resection.

Effect of secretin on children with autism: a randomized controlled trial

To determine the effect of intravenous porcine secretin on autistic behaviours in children aged 2 to 7 years, the effects of secretin on (1) performance on a standardized language measure, and (2) autistic behaviours, as rated by parents and child development professionals was examined. Employing a randomized, double‐blind, placebo‐controlled design, 95 participants were assigned to one of two groups and administered a single dose of either secretin or placebo. A follow‐up assessment was conducted 3 weeks after the injection. No significant differences in language or autistic behaviour measures were observed at the 3‐week follow‐up between the groups. Also, there was no significant difference in the proportion of individuals who improved by 6 points on the language measure at follow‐up. This study showed no significant effects of secretin on children with autism. Our results are consistent with a systematic review of randomized controlled trials evaluating the effect of secretin in children with autism.

Topical Review: Clinical Spectrum of Cortical Dysplasia in Childhood: Diagnosis and Treatment Issues

Disorders of cortical development form a spectrum of lesions produced by insults to the developing neocortex. These conditions typically first manifest in childhood with epilepsy, developmental delay, and focal neurologic signs. Although the clinical and electrophysiologic findings are often nonspecific, high-resolution magnetic resonance imaging facilitates diagnosis during life, and assists in delineating specific clinical syndromes. While many patients are dysmorphic and severely affected by mental retardation and epilepsy, some have normal or near-normal cognitive function and no seizures. Molecular studies of dysplastic cortex are providing new insights into the basic mechanisms of brain function and development, while pathologic analysis of tissue removed at surgery is helping to define epileptic circuitry. Treatment of the epilepsy associated with cortical dysplasia is often frustrating, but surgical approaches based on accurately defining epileptogenic regions are proving increasingly successful. Genetic diagnosis is important for accurate counseling of families. (J Child Neurol 1999;14:759-771).

Clobazam as an Add-on Drug in the Treatment of Refractory Epilepsy of Childhood

We report the results of a double-blind cross-over study comparing clobazam and placebo in the treatment of refractory childhood epilepsy. Fifty-two percent of patients had greater than 50% reduction in their seizure frequency when taking the clobazam. During the placebo phase no patient recorded a greater than 50% reduction in seizure frequency. Sex, age, seizure type, intellect did not appear to differentiate clobazam responsive from nonresponsive patients. Only 2/21 patients had behavioral changes on the drug sufficiently severe to require the patient to drop out of the study prematurely. Drug interactions between clobazam and the other anticonvulsant medicines did not occur.

A survey of behavior problems in children with epilepsy

Over a period of 3 consecutive months, a cross-sectional, descriptive survey was made of the frequency of behavior problems and needs, as reported by their caregivers, of 6- to 18-year-old epileptic children attending two neurology clinics, using the Child Behavior Checklist. Caregivers of 175 children were approached to take part in the study; 158 (90%) completed the Child Behavior Checklist. The mean age of the children was 11.3 (range, 6-18). t scores >70 were obtained for 15.9% of the group for total behavior, 12.7% of the group for internalized behavior and 11.4% of the group for externalized behavior. Compared with those with normal scores, children with elevated t scores were more likely to have learning difficulties and to require the use of more community services.

Quality of life in young adults who underwent resective surgery for epilepsy in childhood.

Purpose:  This study investigated quality of life (QOL) in young adults who had undergone epilepsy surgery before the age of 16 years. The contribution to QOL of seizure status in the prior year, sex, number of antiepileptic drugs, and mood were evaluated.

Insulin-Dependent Diabetes Mellitus Presenting in Children as Frequent, Medically Unresponsive, Partial Seizures

Severe partial seizures may be the presenting feature of nonketotic hyperglycemia in older adults, but cases in children are rare. We report three teenagers with well-controlled epilepsy who suddenly developed intractable partial seizures poorly responsive to anticonvulsants. Blood glucose levels were measured only after several days of hospitalization for frequent seizures when mild polyuria and polydipsia were first noted. Glucose levels were high with mild ketosis and acidosis in one patient and no ketosis in two. With institution of insulin, there was prompt cessation of seizures. The patients were diagnosed as having type I insulin-dependent diabetes mellitus and require ongoing insulin treatment. Hyperglycemia should be considered in children with epilepsy who develop intractable seizures. (J Child Neurol 1997;12:178-180).

Self-reported symptoms of psychological well-being in young adults who underwent resective epilepsy surgery in childhood

Purpose:  This study investigated the relationship of childhood resective surgery for lesional epilepsy and recent seizure history on self‐reported symptoms of mood and psychological distress in young adults (aged 18–30).

Flunarizine as a supplementary medication in refractory childhood epilepsy: a double-blind crossover study.

We report a double blinded cross-over study involving Flunarizine versus placebo in the treatment of refractory childhood epilepsy. The patients studied were between the ages of 2 and 18; and were having more than 4 seizures per month not responsive to regular anticonvulsant medications. Of the 34 patients treated, 8 had a 50% decrease in their seizures during the placebo phase, 5 had a 50% decrease during the Flunarizine phase, and 1 patient had a 50% increase in seizures while taking Flunarizine. The remaining 25 patients showed no change in seizure activity in either phase. Patients having partial seizures with secondary generalization tended to do better on Flunarizine than those with other seizure types. Monitoring serum Flunarizine levels showed no significant difference between patients having improved seizure control and those who were unimproved. No significant side effects were noted with this medication, nor were any significant drug interactions noted.

Patient‐reported outcome of pediatric epilepsy surgery: Social inclusion or exclusion as young adults?

Purpose:  The purpose of this study was to examine the social relationships and participation in educational, vocational, and community life in young adults who had undergone epilepsy surgery during childhood or adolescence.