Joseph Dooley

Epilepsy after a first unprovoked seizure in childhood

One hundred sixty-eight children with an initial afebrile, unprovoked seizure were identified from a regional EEG laboratory. This casefinding method seemed justified because 86% of regional physicians indicated they order an EEG after a first seizure. Clinical information and recurrence rate were determined from records and telephone calls. Eighty-one percent had been seen by a pediatric neurologist. Overall, 51.8% recurred, and of those with a recurrence, 79% had additional seizures. Recurrence rates were highest in those with abnormal neurologic examination, focal spikes on EEG, and complex partial seizures. The lowest rates of recurrence followed a generalized tonic-clonic seizure, with normal EEG and normal neurologic examination. Prescription of anticonvulsants did not alter the recurrence rate.

Long-term prognosis of typical childhood absence epilepsy: Remission or progression to juvenile myoclonic epilepsy

Objective: To determine the proportion and characteristics of children presenting with childhood absence epilepsy (CAE) who were not taking anti-epileptic drugs (AEDs) and were seizure-free over the last year of long-term follow-up. Methods: For case finding, centralized EEG records for the province of Nova Scotia allowed identification of all children with typical CAE diagnosed between 1977 and 1985. Follow-up was done in 1994 to 1995. Results: Of 81 children with CAE, 72 (89%) were contacted for follow-up. Mean age at seizure onset was 5.7 years (range, 1 to 14 years) and at follow-up was 20.4 years (range, 12 to 31 years). Forty-seven (65%) were in remission. Twelve others (17%) were not taking AEDs but continued to have seizures. Thirteen (18%) were taking AEDs; five were seizure-free over the last year (in four of these a trial without AEDs had previously failed). Fifteen percent of the total cohort had progressed to juvenile myoclonic epilepsy (JME). Multiple clinical and EEG factors were examined as predictors of outcome. Factors predicting no remission (p < 0.05) included cognitive difficulties at diagnosis, absence status prior to or during AED treatment, development of generalized tonic clonic or myoclonic seizures after onset of AEDs, abnormal background on initial EEG, and family history of generalized seizures in first-degree relatives. Conclusions: Only 65% of children presenting with CAE had remission of their epilepsy. Forty-four percent of those without remission had developed JME. At the time of diagnosis, remission is difficult to predict accurately in most patients. However, development of generalized tonic-clonic seizures or myoclonic seizures during AED treatment is ominous, predicting both lack of remission of CAE and progression to JME. NEUROLOGY 1996;47: 912-918

Discontinuation of anticonvulsant therapy in children free of seizures for 1 year A prospective study

We studied 97 children who were weaned from antiepileptic drug therapy 1 year after their last seizure.Medication was withdrawn over 4 to 8 weeks, and patients were followed for 12 to 57 months (32.4 plus minus 13.1; mean plus minus SD) or until seizure recurrence. The overall probability of remaining seizure free was 78% at 3 months, 71% at 6 months, 66% at 12 months, and 61% at 24 months (95% CI, 51, 71), similar to studies that have required longer treatment periods. Factors retained in multivariate analysis were female sex, age at seizure onset over 120 months of age, seizure type, and clinical evidence of neurologic abnormalities. Using these risk factors, a simple method of predicting the 24-month recurrence risk was possible. NEUROLOGY 1996;46: 969-974.

A randomized study of carbamazepine versus no medication after a first unprovoked seizure in childhood

We randomized 31 children with a 1st afebrile unprovoked seizure to receive carbamazepine (CBZ) or no medication for 1 year or until the time of a 2nd seizure. All seizures had a focal onset or were generalized tonicclonic. Overall, 2/14 randomized to CBZ and 9/17 with no medication had a recurrent afebrile seizure. Compliance with CBZ was excellent in 12/14, but noncompliance may have contributed to 1 of the recurrences with CBZ. Four discontinued CBZ because of side effects. Two additional children taking CBZ had a febrile seizure. Thus, only 6/14 taking CBZ had a year completely seizure-free with no unacceptable medication side effects. Of those taking no medication, 2 had a febrile recurrence, and 7/17 had a year completely-seizure free. Side effects and febrile recurrences may limit the value of CBZ for some children, although CBZ appears to reduce significantly recurrences after a 1st afebrile seizure.

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

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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.

LAMOTRIGINE-INDUCED RASH IN CHILDREN

Article abstract-Of 68 children treated with lamotrigine, a new antiepileptic medication, five developed a rash. One child developed Stevens-Johnson syndrome on lamotrigine monotherapy. Of the five patients with the rash, three were admitted to the hospital (two to the intensive care unit). All recovered completely, but one child had a recurrence of the rash within 30 minutes of reexposure to lamotrigine after 6 months. NEUROLOGY 1996;46: 240-242

Aspartame exacerbates EEG spike‐wave discharge in children with generalized absence epilepsy A double‐blind controlled study

There are anecdotal reports of increased seizures in humans after ingestion of aspartame. We studied 10 children with newly diagnosed but untreated generalized absence seizures. Ambulatory cassette recording of EEG allowed quantification of numbers and length of spike-wave discharges in a double-blind study on two consecutive days. On one day the children received 40 mg/kg aspartame and on the other day, a sucrose-sweetened drink. Baseline EEG was the same before aspartame and sucrose. Following aspartame compared with sucrose, the number of spike-wave discharges per hour and mean length of spike-wave discharges increased but not to a statistically significant degree. However, the total duration of spike-wave discharge per hour was significantly increased after aspartame (p = 0.028), with a 40% ± 17% (SEM) increase in the number of seconds per hour of EEG recording that the children spent in spike-wave discharge. Aspartame appears to exacerbate the amount of EEG spike wave in children with absence seizures. Further studies are needed to establish if this effect occurs at lower doses and in other seizure types.

Startle disease or hyperekplexia: Adolescent onset and response to valproate

We report a boy with rigidity and hyperekplexia or startle disease which began in adolescence. His symptoms completely resolved with valproic acid therapy. Two relatives have the minor form of this disorder, suggesting autosomal dominant inheritance with variable penetrance.

Pseudo-tics in Tourette Syndrome

We describe two patients with Tourette syndrome who also had pseudo-tics. The psychogenic movements resolved in both children when the stressful issues in their lives were addressed. It is important to differentiate psychogenic symptoms from tics and compulsions in children with Tourette syndrome in order to avoid unnecessary medication and allow appropriate therapy. (J Child Neurol 1994;9:50-51).