Peter Camfield

Outcome of Childhood Epilepsy - a Population-Based Study with a Simple Predictive Scoring System for those Treated with Medication

A population-based study was conducted in an attempt to predict which childs epilepsy will remit. Use of data from a regional electroencephalography laboratory allowed identification of all children in Nova Scotia with epilepsy onset from 1977 through 1985 (excluding those with absence and minor motor seizures). Children were followed for an average of 7 years. On the basis of clinical characteristics, a multivariate analysis was used to develop a scoring scheme to predict remission (defined as off medication at the end of the follow-up period). Survival curve methods were used to estimate the duration of medication treatment for those with remission. Of the 504 eligible patients, approximately 70% became seizure free long enough to discontinue medication. Approximately 70% of those stopping medication a first time remained seizure free. At the end of follow-up, 55% of the total cohort were in remission. At diagnosis, the best predictors of remission were age < 12 years at onset, normal intelligence, no prior neonatal seizures, and fewer than 21 seizures before treatment. If predicted to have a remission, then, on the basis of survival curve analysis, 80% were without medication 100 months after diagnosis. After 12 months of treatment, prediction was enhanced by including a score for the number of seizures between 6 and 12 months on treatment. We conclude that approximately 55% of childhood epilepsy will remit. Our scoring system predicts reasonably accurately who will have a remission and when medication is likely to be discontinued.

Biologic factors as predictors of social outcome of epilepsy in intellectually normal children: A population-based study

We studied social outcome for all the normally intelligent children in our province with onset of epilepsy between 1977 and 1985 (excluding absence and minor motor seizures). After follow-up averaging 7 1/2 years, the 337 patients were 7 to 28 years of age. Outcome measures were age dependent. Of those old enough to be at risk, the percentage with each unfavorable outcome was as follows: school failure 34%, use of special educational resources 34%, mental health consultation 22%, psychotropic medication 5%, unemployment 20%, social isolation 27%, inadvertent pregnancy 12%, and criminal conviction 2%. In social isolation 27%, inadvertent pregnancy 12%, and criminal conviction 2%. In a multivariate model correcting for number of potential unfavorable outcomes (based on age at end of follow-up), many variables related to epilepsy, seizure control, and electroencephalographic findings were not associated with social outcome. Only two variables were associated with at least one unfavorable outcome--learning disorder (p < 0.001) and more than 21 seizures before treatment was begun (p < 0.03). The only variable with no unfavorable outcome was simple partial seizures (p < 0.003). Sensitivity and specificity of this model were 54% and 68%, respectively, indicating that social outcome for these children was often not related to biologic factors reflected by the medical details and clinical course of their disorder.

Melatonin Appears Ineffective in Children With Intellectual Deficits and Fragmented Sleep: Six "N of 1" Trials

9. Starkman SP, Brown TC, Linell EA: Cerebral arachnoid cysts. J Neuropathol Exp Neurol 1958; 17:484-500. 10. Klein TE, Bernard EM, Gold JWM, Armstrong D: Candidiasis, detection by gas-liquid chromatography of D-arabinitol, a fungal metabolite, in human serum. Science 1979;206:577-580. 11. Wong B, Brauer KL: Entantioselective measurement of fungal D-arabinitol in the sera of normal adults and patients with candidiasis. J Clin Microbiol 1988;26:1670-1674.

Duchenne Muscular Dystrophy—Parental Perceptions

Quality of life and availability of services are important for boys with Duchenne muscular dystrophy (DMD) and their families. Families attending our neuromuscular clinic completed a questionnaire on parental perception regarding the importance of services, health issues, and quality of life issues both “now” and “in the future.” Eighty-nine percent of the families (31/35) completed questionnaires. Services and health issues related to prolonging ambulation were most important, especially for the parents of younger boys. Mental health issues such as social isolation, anger, and depression were very important, particularly for the families of older boys and were anticipated to be more important in the future. Pediatricians should be aware of both the immediate needs of families to meet the physical and emotional challenges of DMD and the increasing requirement to address the social needs of these patients and their families as the boys become older.

EEG results are rarely the same if repeated within six months in childhood epilepsy.

OBJECTIVEnTo assess the reliability of interictal spike discharge in routine electroencephalography (EEG) testing in children.nnnMETHODnEEG results of all children diagnosed in Nova Scotia with epilepsy onset between 1977-85 (excluding myoclonic, akinetic-atonic and absence) were reviewed. The results of the EEG at time of diagnosis (EEG1) were compared with those of a second EEG (EEG2) within 6 months.nnnRESULTSnOf 504 children with epilepsy, 159 had both EEG1 and EEG2. EEG2 was more likely ordered if EEG1 was normal or showed focal slowing but less likely if EEG1 contained sleep (p < 0.05). EEG1 and EEG2 were both normal in 23%. If EEG1 was abnormal, there was a 40-70% discordance for the type of abnormality on EEG2. Abnormalities were present on both EEG1 and EEG2 in 67 cases. Of the 42/67 with major focal abnormalities on EEG1, 7 had only generalized spike wave on EEG2. Of the 17/67 with only generalized spike wave on EEG1, 7 showed only major focal abnormalities on EEG2. Statistical testing showed low Kappa scores indicating low reliability.nnnCONCLUSIONSnThe interictal EEG in childhood epilepsy appears to be an unstable test. A repeat EEG within 6 months of a first EEG may yield different and sometimes conflicting information.

Families are content to discontinue antiepileptic drugs at different risks than their physicians

Summary: Purpose: To define the risk of seizure recurrence (RSR) that families and physicians would accept before discontinuing antiepileptic drugs (AEDs) for children with controlled epilepsy.

The Association of Chiari Type I Malformation and Neurofibromatosis Type 1

The association of neurofibromatosis type 1 (NF1) with Chiari malformations of the cerebellum and brain stem has been reported on only two previous occasions.1,2 The pathogenesis of both conditions has remained unclear, although the Chiari type I malformation is most likely due to hypoplasia of the posterior fossa with subsequent extension of the cerebellum through the foramen magnum.3 NF1 is also associated with a variety of cerebral dysplasias.4 We present a patient with both of these dysplastic lesions whose Chiari malformation was asymptomatic.

Toxicity From Vacuumed Mercury: A Household Hazard

Department of Pediatrics, Dalhousie University, I.W.K. Children’s Hospital, Halifax, Nova Scotia. Correspondence to: Dr. Peter R. Camfield, I.W.K. Children’s Hospital, P.O. Box 3070, Halifax, N.S. B3J 3G9. Presented in part at the Canadian Pediatric Society Meeting, Halifax, Nova Scotia July 1989. A 14-year-old boy is described who developed severe mercury (Hg) poisoning. He inhaled Hg vapor when he vacuumed up spilled Hg that he had obtained from two thermostats. He was successfully treated with chelating agents. Of 70 adults questioned, 17 indicated that they would clean up such a Hg spill in a similar fashion. Because

Benign Familial Neonatal Convulsions Are Epileptic

An infant with benign familial neonatal convulsions fortuitously had a clinical seizure during a routine EEG. The seizures had started on day 2 of life, and the EEG recording was performed on day 6. The EEG, although not complete, did show a simultaneous electrographic seizure. Our finding establishes that benign familial neonatal convulsions are indeed epileptic. (J Child Neurol 1991;6:340-342).

The Pharmacology of Chewable Versus Regular Carbamazepine in Chronically Treated Children With Epilepsy

We report the first comparison of Chewable and Regular Carbamazepine (CBZ) tablets in children with epilepsy. Forty-four children receiving chronic monotherapy CBZ participated. In month 1 children received regular CBZ; in month 2, the same dose of Chewable CBZ. Once per week fasting predose CBZ and CBZ epoxide serum levels were determined. In a subset of 15 children, at the end of each month serum levels were obtained every 2 hours for 12 hours beginning pre-dose. Standards for CBZ and CBZ epoxide were tested in each centre. Overall, weekly levels showed no consistent differences between the month on chewable CBZ and regular CBZ. Seizure control and rates of reported side effects were similar. In five patients chewable CBZ produced higher peak CBZ levels while five had higher peaks with regular CBZ. In conclusion, regular and chewable CBZ often have unpredictable differences in peak but not trough levels of CBZ suggesting that peak level side effects with one form of CBZ might be alleviated by changing to the other.