Rasmussen's encephalitis (RE) is a chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere. In the present study, a comprehensive assessment of the natural history of the disorder is presented. Seizure frequency, degree of hemiparesis and degree of cerebral hemiatrophy in 13 patients with histopathologically proven RE are analysed over the time course prior to resective epilepsy surgery or introduction of long-term immunosuppressive pharmacotherapy. For the assessment of the degree of cerebral hemiatrophy, on defined slices comprising the Sylvian fissure of hard copies of serial MRI investigations, the hemispheric ratio (HR) was determined. The data show an initial prodromal phase with an intermediate frequency of focal onset seizures and mostly no hemiparesis. The occurrence of this stage was mainly observed in the adolescent and adult patients. All patients went through an acute phase with a median duration of 8 months. During this stage, there were frequent simple partial motor seizures, development of hemiparesis and volume loss of the affected hemisphere. After this, the patients passed into a residual stage with a marked decrease in seizure frequency. Twelve months after the onset of the acute stage, the average HR was 0.72. These data allow an estimation of the prognosis of newly affected patients, and demonstrate that most of the brain damage in RE occurs during the first 8-12 months. These findings should be taken into consideration when future therapeutic approaches to RE are evaluated.
Hemispherotomies/functional hemispherectomies are very effective and safe procedures for treating drug-resistant epilepsy with extensive unihemispheric pathology. Etiology and surgery type clearly influence seizure outcome.
The countrywide incidence rate of diagnosed RE is estimated as 2.4 cases/10⁷ people ≤ age 18/year. Treatment with tacrolimus or IVIG may slow down tissue and function loss and prevent development of intractable epilepsy. However, immunotherapy may "arrest" patients in a dilemma state of pharmacoresistant epilepsy but too good function to be offered functional hemispherectomy. These compounds may therefore contribute to the therapeutic armamentarium for RE patients without difficult-to-treat epilepsies.
Start of AED withdrawal, number of AEDs reduced, and complete AED withdrawal were associated with improved postoperative IQ scores and gain in IQ, independent of other determinants of cognitive outcome.
Objectives-Epilepsy surgery has proved to be a successful intervention method to achieve freedom from seizures or seizure relief in children with pharmacoresistant epilepsy. Long term studies on operated children suggest that behavioural disorders, which are often seen before surgery, improve after surgery. However, the early postoperative development of behavioural problems has not been systematically evaluated. Methods-Parents of 28 children with pharmacoresistant focal epilepsies completed the child behaviour checklist (CBCL) preoperatively and 3 months after surgery. Surgeries comprised 24 focal resections (13 temporal, 11 extratemporal), two hemispherectomies, and two callosotomies. Twenty eight conservatively treated children with comparable CBCL scores served as a control group. A repeated measurement multivariate analysis of variance (MANOVA) and a regression analysis were computed to compare the development of behaviour between both groups and to identify predictors of postoperative changes in behaviour. Results-Preoperatively 39% of the children exhibited significant behavioural problems, a further 11% were within the borderline range. The MANOVA disclosed a significant interaction between time of examination and group (F=2.23, p<0.05). The surgery group showed significant improvements on the scales "internalising problems", "externalising problems", "attention problems", and "thought problems". Behavioural problems in the control group, however, remained unchanged. No changes were seen in social problems in both groups. The significant predictor of total behavioural improvement was a good seizure outcome (R 2 =0.11, p<0.05). Age, sex, onset, and duration of epilepsy, the site of the focus, and changes in antiepileptic drug regimen did not influence changes in behaviour.Conclusions-The data demonstrate an early improvement of behavioural problems after epilepsy surgery in children. The behavioural improvements can be assumed to result directly from the removal of the epileptic focus. They are not predictable on the basis of information available preoperatively, but depend on the seizure outcome. (J Neurol Neurosurg Psychiatry 2000;69:739-744)
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.