Nathalie Poznanski

Levetiracetam efficacy in epileptic syndromes with continuous spikes and waves during slow sleep : Experience in 12 cases

Summary:  Purpose: To assess the add‐on efficacy of levetiracetam on the EEG, behavior, and cognition of children with continuous spikes and waves during slow sleep (CSWS).

Regional cerebral glucose metabolism in epilepsies with continuous spikes and waves during sleep.

Background: Epileptic syndromes with continuous spikes and waves during sleep (CSWS) represent a wide spectrum of epileptic conditions associated with cognitive dysfunctions that have the EEG pattern of CSWS as a common feature. Reported are the results of voxel-based analyses of brain glucose metabolism performed in a group of 18 children with CSWS. Methods: Voxel-based analyses of cerebral glucose metabolism were performed using statistical parametric mapping (SPM). First, each patient was compared with a control group and the influence of age, epileptic activity, and corticosteroid treatment on metabolic abnormalities was studied. Also, disease-related changes in the contribution of a brain area to the level of metabolic activity in another brain area were investigated using pathophysiologic interactions in groups of patients compared with the control group. Results: Individual SPM analyses identified three metabolic patterns: association of hypermetabolic and hypometabolic areas, hypometabolic areas only, and normal pattern. Age and intensity of awake interictal spiking did not significantly differ in patients showing focal hypermetabolism compared with the other ones. Treatment with corticosteroids was associated with absence of focal hypermetabolism. In the group of patients with hypermetabolic areas, analyses of pathophysiologic interactions showed disease-related altered functional connectivity between the parietal and frontal cortices. Conclusions: Cerebral metabolic patterns are heterogeneous among patients with CSWS. This metabolic heterogeneity could be related to the use of corticosteroid treatment before PET. The parietofrontal altered connectivity observed in patients with hypermetabolism is interpreted as a phenomenon of remote inhibition of the frontal lobes induced by highly epileptogenic and hypermetabolic posterior cortex.

Coexistence of Idiopathic Rolandic Epilepsy and CSWS in Two Families

Summary:  Purpose: To report two families combining benign childhood epilepsy with centrotemporal spikes (BCECS) and cryptogenic epilepsy with continuous spike–waves during sleep (CSWS) in first‐degree relatives.

Acquired Alexia With Agraphia Syndrome in Childhood

The acquired alexia with agraphia syndrome is a conspicuous disorder of reading and writing in the absence of significant other language impairments that has mainly been recorded in adults. Pure cases are rare, with most patients displaying mild aphasic deficits. In children, acquired reading and writing disorders are generally reported as part of more encompassing aphasic syndromes affecting oral and written language equally, for example, Broca or Wernicke aphasia. Documented instances of predominant acquired reading and writing disorders in childhood are exceptional. We report an 11-year-old, right-handed boy who sustained a left temporoparieto-occipital hematoma following rupture of an arteriovenous malformation and who consecutively presented with the acquired alexia with agraphia syndrome associated with word-finding difficulties. Neuropsychologic and neurolinguistic data showed that there was no concomitant Gerstmann and/or angular gyrus syndrome. The recovery from the anomia was quite favorable, but recovery of written language was more protracted and acted on the patients further scholastic achievement. This case is reminiscent of a historical childhood case reported in 1939 and is consonant with adult cases in terms of lesion location and semiologic picture. (J Child Neurol 2006;21:324—330; DOI 10.2310/7010.2006.00087).

Regional changes in cerebral glucose metabolism induced by status epilepticus in patients with partial epilepsy

In 1990, the World Health Organization (WHO) estimated that about 50 million people worldwide are afflicted with epilepsy. A disproportionately high number live in developing countries. Prevalence in industrialized countries lies between 5 and 8 per 1,000 population. I n the developing countries where prevalence has been studied it may be up to 6 times higher. Most epilepsy sufferers in industrialized countries receive medical treatment, and are able to live a normal life. The treatment gap (percentage ofpeople who should, but do not, receive treatment) in developing countries is dismal and may reach 80-90%. Most people with epilepsy in developing countries do not lead a normal life. On the basis of her research and work with epilepsy in Africa for over 30 years, the author determined possible reasons for the high prevalence of epilepsy and the poor attendance at available medical facilities in developing countries. From the beginning of history, epilepsy has impressed people as mysterious and frightening. Traditional treatment and attitude towards the afflicted is closely linked to the socio-cultural make up of the population and often leads to additional suffering. All the aforementioned factors have to be taken into consideration if preventive measures and medical treatment are to be successful in developing countries.