We observed seizures resembling infantile spasms in patients with Lennox-Gastaut syndrome (LGS). Infantile spasms, the type of seizures that occurs in patients who have West syndrome, have been well characterized by video-EEG studies and typically occur as a series of sudden generalized flexor or extensor jerks. The seizure types that occur in LGS have not been as clearly delineated. Some patients with West syndrome (WS) in early infancy later develop LGS. Using intensive video-EEG monitoring, we evaluated 14 LGS patients who had seizures that occurred in series. Clinically, the seizures greatly resembled infantile spasms, and the ictal EEG changes were identical to those that occur with infantile spasms. These findings expand the number of features known to be shared by these two syndromes and strengthen the hypothesis that the two syndromes represent age-related manifestations of similar epileptogenic processes.

We found neurologically impaired children studied by time-locked video-EEG to have episodes of abnormal behaviors which had been mistaken for epileptic seizures. Recognition that other neurologically abnormal phenomena can closely mimic epilepsy is important for prevention of erroneous diagnoses of epilepsy, and thus overtreatment, in this patient population.

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Although the long-term effects of acute anoxia have been studied, the effects of chronic hypoxia on the developing human brain have received little attention. We studied children with a cyanotic congenital heart defect to assess the impact of chronic hypoxia by eight measures: neurologic examination, visual evoked response, EEG, behavioral adjustment, cognitive, perceptual-motor, and attentional functioning, and school performance. On outcome evaluation, these children evidenced diverse neurophysiologic dysfunction. Chronic hypoxia was associated with impaired motor function, inability to sustain attention, and low academic achievement.

A model of risk potential for developmental outcome was created based on cardiac, medical, surgical, and family-stress factors in 31 children with transposition of the great arteries who had undergone reparative open heart surgery utilizing cardiopulmonary bypass during infancy. Impact of these potential risk factors was assessed by 4 current neurologic measures (neurologic anatomic abnormalities, functional impairment, electroencephalograph [EEG], and Pattern Visual Evoked Potential [PVEP]) and 4 psychologic measures (IQ, achievement, perceptual-motor function, and behavior). Results indicated that adverse developmental outcome was significantly associated with the following medical risk variables: failure of palliative surgery to alleviate hypoxia, prolonged hypoxia, growth failure, congestive heart failure, absence of ameliorating shunting heart defects, stroke, and CNS infection; and two psychosocial moderator variables: socioeconomic status and current life stress. Analysis of a "cumulative risk score" indicated significantly higher risk scores in children with abnormal EEGs, PVEPs, and neurologic examinations. The cumulative risk score highly correlated with composite neurologic outcome (r = .62), IQ (r = -.66), achievement (r = -.60), and perceptual-motor function (r = -.48). While overall outcome was favorable for children with TGA who experienced a single risk event, outcome was compromised if multiple risk factors occurred.

A model of risk potential for developmental outcome was created based on cardiac, medical, surgical, and family-stress factors in 31 children with transposition of the great arteries who had undergone reparative open heart surgery utilizing cardiopulmonary bypass during infancy. Impact of these potential risk factors was assessed by 4 current neurologic measures (neurologic anatomic abnormalities, functional impairment, electroencephalograph [EEG], and Pattern Visual Evoked Potential [PVEP]) and 4 psychologic measures (IQ, achievement, perceptual-motor function, and behavior). Results indicated that adverse developmental outcome was significantly associated with the following medical risk variables: failure of palliative surgery to alleviate hypoxia, prolonged hypoxia, growth failure, congestive heart failure, absence of ameliorating shunting heart defects, stroke, and CNS infection; and two psychosocial moderator variables: socioeconomic status and current life stress. Analysis of a "cumulative risk score" indicated significantly higher risk scores in children with abnormal EEGs, PVEPs, and neurologic examinations. The cumulative risk score highly correlated with composite neurologic outcome (r = .62), IQ (r = -.66), achievement (r = -.60), and perceptual-motor function (r = -.48). While overall outcome was favorable for children with TGA who experienced a single risk event, outcome was compromised if multiple risk factors occurred.

The National Institutes of Health sponsored a randomized, double-blind, multicenter, placebo-controlled trial of flunarizine (FNR) in epileptic patients receiving concomitant phenytoin (PHT) or carbamazepine (CBZ). Because of FNR's long half-life (up to 7 weeks), a parallel rather than crossover design was used. Each patient received an individualized loading dose and maintenance dosage targeted at a 60-ng/ml plasma FNR concentration. Of 93 patients randomized, 92 provided seizure data for the full 25-week treatment period; one placebo-treated patient dropped out for personal reasons. Fifty-four patients received CBZ only, nine received PHT only, and 30 received both CBZ and PHT. Eighty-seven patients had a history of complex partial seizures, and 60 had secondarily generalized seizures. Eight patients discontinued FNR prematurely, all because of adverse neurologic or psychiatric signs or symptoms; depression was the specific cause in three cases. Calculated maintenance dosages, based on single-dose pharmacokinetic profiles, ranged from 7 to 138 mg/day (mean, 40 mg/day). Plasma FNR concentrations generally exceeded the target, with the highest concentrations observed immediately after loading; excluding the first three treatment weeks and all concentrations after a FNR dosage change, the median plasma FNR concentration was 71.7 ng/ml. The percent reduction from baseline seizure rate was statistically greater (p = 0.002) in the FNR-treated group (mean, 24.4%) than in the placebo-treated group (mean, 5.7%).

No abstract