Assessment of everyday executive functioning in children with frontal or temporal epilepsies.

Executive functions are particularly vulnerable in case of brain disruption during childhood, when the brain is not fully mature. Some studies showed impairments of executive functions in children with epilepsy, but only a few of them investigated the impact of executive dysfunctions on daily life. The aim of this study was to understand the everyday executive functioning of children with epilepsy both at home and in school. We administered the Behavior Rating Inventory of Executive Function to parents and teachers of 53 children (7-16 years of age) with structural epilepsies or epilepsies of unknown cause of temporal lobe (n=25) or frontal lobe (n=28). The results indicated a global executive impairment in the whole group of patients, compared with normative data, with no difference between the group with temporal lobe epilepsy (TLE) and that with frontal lobe epilepsy (FLE), except for monitor domain, which seemed more frequently impaired in the group with FLE. Congruence between parent and teacher ratings was found. The frequency of seizures was not related to executive dysfunction, whereas the number of antiepileptic drugs tended to positively correlate with working memory impairment. Onset of epilepsy at a younger age was also related to more executive difficulties but only according to teacher ratings. Lastly, duration of epilepsy was strongly associated with executive deficits reported in the context of school. Our results support the executive dysfunction hypothesis in daily life of children with structural focal epilepsy or focal epilepsy of unknown cause and are consistent with the early brain vulnerability hypothesis currently prevalent in the context of child neuropsychology. The BRIEF appears to be a clinically useful tool for assessing executive function impairment in this clinical population.

EEG criteria predictive of complicated evolution in idiopathic rolandic epilepsy.

BACKGROUND: Although so-called "benign" epilepsy with centrotemporal spikes (BECTS) always has an excellent prognosis with regard to seizure remission, behavioral problems and cognitive dysfunctions may sometimes develop in its course. To search for clinical or EEG markers allowing early detection of patients prone to such complications, the authors conducted a prospective study in a cohort of unselected patients with BECTS. METHODS: In 35 children with BECTS, academic, familial, neurologic, neuropsychological, and wake and sleep EEG evaluations were repeated every 6 to 12 months from the beginning of the seizure disorder up to complete recovery. RESULTS: In 25 of 35 patients (72%), behavioral and intellectual functioning remained unimpaired. In 10 of 35 patients (28%), educational performance and familial maladjustment occurred. These sociofamilial problems were correlated with impulsivity, learning difficulties, attention disorders, and minor (7/35 cases, 20%) or serious (3/35 cases, 8%) auditory-verbal or visual-spatial deficits. Worsening phases started 2 to 36 months after onset and persisted for 9 to 39 months. Occurrence of atypical evolutions was significantly correlated with five qualitative and one quantitative interictal EEG pattern: intermittent slow-wave focus, multiple asynchronous spike-wave foci, long spike-wave clusters, generalized 3-c/s "absence-like" spike-wave discharges, conjunction of interictal paroxysms with negative or positive myoclonia, and abundance of interictal abnormalities during wakefulness and sleep. Clinical deterioration was not linked with seizure characteristics or treatment. CONCLUSION: Different combinations of at least three of six distinctive interictal EEG patterns and their long-lasting (> or =6-month) persistence seem to be the hallmarks of patients with BECTS at risk for neuropsychological impairments.

[Cognitive consequences of Rolandic Epilepsy]. / Retentissement cognitif de l'épilpsie à pointes centro-temporales.

Idiopathic focal epilepsies and particularly rolandic epilepsy are considered to have an excellent outcome with spontaneous recovery during adolescence. The characterization of this syndrome in 1959 by Beaussart and Nayrac was a great progress for childhood epileptology: the existence of a focal epilepsy with a good prognosis and no underlying brain lesion, so-called "benign" epilepsy, was recognized. Since the first descriptions, numerous neuropsychological studies were performed showing variable results. All the studies agree with the fact that children with rolandic epilepsy keep a normal global intellectual efficiency and a good long-term outcome. Nevertheless, some children may suffer transiently during the active phase of the epilepsy from oromotor dysfunction, neuropsychological deficits, or attention deficits with learning disorders. The analysis of cognitive and neurophysiological correlations evidenced a significant correlation between the epileptic focus localization and few specific dysfunctions. We evidenced mainly a significant effect of the persistence of a prolonged slow focus and a strong activation of night EEG spike and waves on cognitive decline and attention disorders. These abnormalities are mainly observed during severe or atypical evolutions of rolandic epilepsy. Preliminary longitudinal studies show that these cognitive deficits are transient. Thus, the presence of an active epileptic focus, without underlying brain lesion, could interfere with normal maturation of cognitive function.

Landau-Kleffner syndrome: sleep EEG characteristics at onset.

OBJECTIVES: Landau-Kleffner syndrome (LKS) and benign epilepsy with centro-temporal spikes (BECTS) are two forms of non-lesional age-related focal epilepsies. LKS is a severe disease, affecting language abilities, attention and behavior, and evolving to acquired global aphasia. As LKS is usually readily responsive to an adequate pharmacological management, an early diagnosis of children at risk for this syndrome is essential. BECTS is characterized by the absence of neurological or neuropsychological deficits throughout the course of epilepsy. However, children initially presenting some clinical and EEG features suggesting BECTS may develop severe cognitive impairments during the course of epilepsy. These cases raise the question of whether LKS and BECTS delineate fundamentally different conditions, or represent subclasses of a broad continuum. METHODS: We compared sleep EEG characteristics of 7 children with typical LKS to those of 6 children with classical BECTS. RESULTS: Morphology, topography, organization, and abundance of interictal abnormalities during sleep differentiated these two syndromes from epilepsy onset, before the occurrence of aphasic deficits in LKS. The specific sleep EEG patterns possibly predictive of LKS were (1) unilateral slow wave foci, (2) bilateral independent spike-and-wave discharges, and (3) major activation of spike-and-wave discharges during sleep, exceeding 40% (40-90%) of the first sleep cycle and 30% (30-80%) of the following cycles. CONCLUSIONS: These data support the hypothesis that during LKS evolution, language networks involved in the spread of abundant idiopathic interictal abnormalities (and mainly slow waves) may be progressively inhibited and become unable to carry out their normal physiological role.

Interaction of the alerting effect of noise with partial sleep deprivation and circadian rhythmicity of vigilance.

Only a few studies have been devoted to the interaction of noise with sleep deprivation and time of day. In a previous study we demonstrated that noise had an alerting effect on cognitive performance during the early night but not during the late night. However, it was not clear whether these different effects of noise were related to prior sleep debt or to time of day as both factors varied simultaneously. In the present experiment, we further studied this issue to identify which of these two factors was responsible for the noise effects. Analysis showed that, when performance was tested at different times with an equivalent prior sleep debt, noise improved speed of response at 0500 but not at 0800 at which variability of response time increased. Noise had no effect on errors. It is suggested that the effect of noise depends on the underlying arousal and raises arousal from its low level due either to time of day or to partial sleep deprivation.