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Thesis

English

ID: <

10670/1.408ygh

>

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Reversible acquired epileptic frontal syndrome and CSWS suppression in a child with congenital hemiparesis treated by hemispherotomy

Abstract

summary report: This thesis examined in detail the case of a child suffering from congenital haemplegia on an extended prenatal infarction that developed a particular form of epilepsy, the continuous sleep wave syndrome (POCS), combined with massive mental regression. The characteristics of this deterioration pointed towards a frontal malfunction. Epilepsy surgery (haemispheromtomy) has not only allowed epilepsy recovery to recover rapidly from the behavioural and cognitive point of view, followed by a slower recovery in development, eventually at the age of 11 with a moderate level of intellectual impairment. The relevance of this study is that the child could be followed prospectively between the age of 4.5 and 11 by electro-encephaliographic (EEG) recordings as well as neuropsychological tests and serial behavioural questionnaires, making it possible to compare pre-, peri- and post-operative periods, which is rarely feasible. An EEG surface recording could even be carried out during the operation on the unharmed hemisphere, making it possible to document the stopping of generalised epileptiform landfills at the end of the operation. The hypothesis we wanted to demonstrate is that the behavioural and cognitive regression presented by the child after an almost normal period of early development (delay in language) was epileptic: this is explained by the spread of abnormal electrical activity from the lesion of the left hemisphere to the preserved regions, in particular bilateral fronts. The hemispheromics allowed rapid recovery by disconnecting the damaged left hemisphere and epileptogenic from the healthy hemisphere, which allowed the most important cognitive functions to resume. The slower progress thereafter and the lack of catching-up beyond a moderate level of mental impairment are more difficult to explain: epilepsy is assumed to have an effect on the development of initially unharmed neuronal networks of the hemisphere, which are both at an early stage of ripening and being reorganised as a result of prenatal injury. The literature on cognitive deficits before and after hemisphertomy has focused on language and its possible recovery. To our knowledge, our study is the first to document the reversibility of mental deterioration with the characteristics of a frontal syndrome after haemisphertomia. Epilepsy surgery has provided a unique opportunity to document the role of epileptic activity in cognitive regression, since by suddenly halting the spread of abnormal electrical activity, the dynamics of development before and after intervention have been compared. Linking the multiple clinical and EEG examinations carried out in a single child over several years has provided important information in understanding cognitive disorders and behaviour associated with refractory focal epilepsies. ABSTRACT: A boy with a right congenital hemiparesis due to a left Pre-natal middle cerebral artery infarct developed focal Epilepsy at 33 months and then an ongoing spike-waves during sleep (CSWS) on the EEG. Both the Epilepsy and the CSWS were immediately supported by hemispherotomy at the age of 5 years and 4 months. A behavioural-cognitive follow-up prior to hemispheratomy, an per-operative EEG and corticography and serial post-operative neuropsychological assessments were performed until the age of 11 years. The spread of the epileptic activity to the ‘healthy’ frontal region was the cause of the reversible frontal syndrome. A later gradual long-term but incomplete cognitive recovery, with moderate mental disability was documented. T9ris outcome is probably explained by another Facet of the Epilepsy, namely the structural effects of prolonged epileptic dischazges in recent developing cerebral networks which are, at the same time Undergoing the Reorganisation imposed by a single early hemispheric lesion. Group studies on the outcome of children before and after hemispherectomy using only single IQ measures, pre- and postoperatively, may be particular epileptic cognitive Dysfunctions as they are likely to be different from case to case. This detailed and clearly available complementary clinical and EEG data obtained in a single case at different time periods in relation to the Epilepsy, including peroperative Electrophysiological findings, may help to present the various cognitive profiles and recovery profiles and the limits of full cognitive recovery.

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