Approximately three-quarters of epileptic patients seen in consultation by specialists can easily be assigned to one of the diagnostic categories of the International League against Epilepsy. When patient age is taken into account, such a distribution shows that partial epilepsy is more common than generalized epilepsy (62% versus 38%) in patients of all ages, especially in those over 15 years of age (78% versus 22%). In contrast, generalized epilepsy is more frequent in subjects under 15 than partial epilepsy (55% versus 45%). In this younger age group the rarity of partial epilepsy is primarily due to the infrequency of temporal lobe epilepsy (21% of cases, as opposed to 56% in subjects over 15), whereas both primary and secondary generalized epilepsy contribute to the frequency of generalized epilepsy in this group.

Summary:Purpose: Gamma knife radiosurgery (GK) allows precise and complete destruction of chosen target structures containing healthy andor pathologic cells, without significant concomitant or late radiation damage to adjacent tissues. All the well-documented radiosurgery of epilepsy cases are epilepsies associated with tumors or arteriovenous malformations (AVMs). Results prompted the idea to test radiosurgery as a new way of treating epilepsy without space-occupying lesions.Methods: To evaluate this new method, we selected seven patients with drug-resistant "mesial temporal lobe epilepsy" (MTLE).The preoperative evaluation program was the one we usually perform for patients selected for microsurgery of TLE [video-EEG analysis of seizures, foramen ovale electrode recording, magnetic resonance imaging (MRI) positron emission tomography (PET) scan, neuropsychological testing]. In lieu of microsurgery, the amygdalohippocampectomy was performed by using GK radiosurgery.

Computerized transverse axial tomography (CTAT) of the brain has been used routinely, as well as the EEG, to study patients with epilepsy. In patients with the various electro-clinical types of epilepsy -- primary, secondary, and partial -- it gave accurate information about the frequency, topography, and severity of morphological abnormalities. In the various types of organic lesion -- tumor, posttraumatic, postischemic, postinfectious, etc. -- it markedly increased the ability to establish etiology. Especially notable was the finding of (1) tumor in 16% of patients over 20 years of age, and (2) the determination of a type of pathology that has received little attention -- postischemic occipital porencephaly probably due to occlusion of the posterior cerebral artery, either at birth or in early infancy.

To assess prognostic factors for absence epilepsy (AE), we analyzed data from 80 patients treated for childhood AE (CAE; n = 53) or juvenile AE (JAE; n = 27) in our epilepsy clinic between 1985 and 1992. All patients were classified according to the International Classification of Epileptic Syndromes which was proposed by the International League against Epilepsy in 1989. Patients were separated into two groups based on the course of disease under adequate treatment: Complete response group (CRG): disappearance of absence seizures (AS) or generalized tonic-clonic seizures (GTCS). Poor response group (PRG): persistence of AS and/or GTCS. Approximately 40% of both CAE and JAE patients had poor response. One parameter was associated with poor prognosis in CAE patients, the presence of polyspikes or polyspikes and waves during sleep. No statistical correlation was made for JAE patients. GTCS were frequent in JAE and GTCS occurrence in CAE patients was associated significantly with age at onset after 8 (p < 0.05). The fact that social and educational performance was poorer in the PRG of both types of AE underlines the importance of therapeutic response in patient rehabilitation.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant cerebral arteriopathy mapped to chromosome 19 and characterized mainly by recurrent subcortical ischemic strokes and extensive white-matter signal abnormalities (WMAs) on magnetic resonance imaging. Other clinical features include migraine attacks and progressive subcortical dementia. Herein, we describe several members of the same family who suffered migraine attacks, mostly with aura, associated with WMAs, segregating with an autosomal dominant pattern of inheritance. One individual had a progressive subcortical dementia with similar WMAs. Although ischemic stroke, one of the hallmarks of CADASIL, was not present in this family, we hypothesized that the present disorder resulted from an alteration of the CADASIL gene. Genetic linkage analysis, using four chromosome 19 markers spanning the CADASIL locus, supports this hypothesis.