The efficacy and safety of topiramate 400 mg/day as adjunctive therapy to traditional antieleptic drugs for partial onset seizures with or without secondary generalization were assessed in a double-blind, parallel-group, placebo-controlled trial. Forty-seven patients with at least one seizure per week during an 8 week baseline were randomly assigned to topiramate (N = 23) or placebo (N = 24) double-blind treatment for a 3 week titration and an 8 week stabilization period. Median percent reduction from baseline in monthly seizure frequency during the double-blind phase was not significantly greater in the topiramate group than in the placebo group (41% vs. 1%; P = 0.065). Nevertheless, other efficacy variables evidenced statistically significant differences in favor of topiramate: a greater number of treatment responders (≥ 50% reduction in seizures; 35% vs. 8%; P = 0.033); better investigator (P = 0.002) and patient (P = 0.021) global assessments; and greater reductions in secondarily generalized seizures compared to placebo (P = 0.002). The most commonly reported topiramate treatment-emergent adverse events were somnolence, fatigue, abnormal vision, weight decrease, and anxiety. Most adverse events were mild or moderate in severity. Among 7 withdrawals due to limiting adverse events, 6 were CNS-related (in 5 topiramate-treated patients). Results of this trial strongly suggest that topiramate 400 mg/day is effective and well tolerated in the treatment of refractory partial epilepsy.

The decision to begin an antiepileptic treatment is not always an easy one, as this treatment will be long lasting with possible side-effects. However, if the patient has had several seizures or if, after a first seizure, there are certain features predicting that other seizures will follow, then in all but very rare cases this decision must be made and applied as soon as possible. Patients who will have chronic epilepsy are recognized within the first two years, and among the factors preventing a therapeutic failure, early treatment is of the utmost importance. After a first, strictly isolated seizure, and for lack of convincing figures as to the risk of recurrence, many data must be examined, the most important being the patient's wish. All this shows that there is room for various decisions.

Dix patients narcoleptiques sont traités par 2 à 6 mg par jour de Mazindol pendant 42,2 mois (extrêmes 31 et 63 mois). L'amélioration est très satisfaisante 6 fois sur 8 sur les accès narcoleptiques et la cataplexie dans 7 cas. Par contre, les troubles du sommeil de nuit persistent inchangés. L'amélioration est modérée dans 1 cas et nulle 1 fois malgré 6 mg par jour. Des effets secondaires mineurs (sécheresse de bouche) sont observés 3 fois, mais dans 2 cas, une rétention urinaire a imposé l'arrêt du traitement.

The use of evoked potentials for the evaluation of neuronal mechanisms by which convulsant drugs activate epilepsy and produce seizure has been reported by many authors. Electrophysiological effects of bemegride with augmented responsivity of brain structure to sensory stimulation is well known, especially in experiments performed with implanted electrodes in animals.

Data found in the literature and our own observations prompted us to consider the possibility that abnormally enlarged Somatosensory Evoked Potentials (SEPs) may have a diagnostic and physiopathological significance, particularly in a group of diseases which include common clinical features of encephalopathy with stimuli-sensitive myoclonus and epilepsy, whatever their etiology may be (degenerative or storage disease, metabolic, toxic or post-hypoxic encephalopathy…). We discuss the amplitude, morphology, diagnostic and therapeutic contribution of these ‘giant’ SEPs and pathogenic assumptions with reference to ‘cortical reflex myoclonus’. Studies of back-averaged encephalogram, SEPs and long-loop reflexes allow some illustration of a functional hyperreactivity of the sensori-motor cortex, but no conclusive demonstration of its origin.

458 patients who had their first epileptic fit between the ages of 40 and 65 are classified into 5 groups according to the seizure symptomatology: when the symptomatology remains unchanged, the frequency of fits is less than 1 per month in 84% of generalized fits and more than 1 per month in 52% of focal fits. When the symptomatology changes, the frequency of fits is less than 1 per month when the inaugural fit is generalized (23 out of 27) and more than 1 per month when the inaugural fit is focal (15 out of 21). Modifications of the symptomatology correlate positively with a prevalence of tumoral aetiology. Primary generalized epilepsies are rare (4.4%). Non-specific secondary generalized epilepsies (including vascular, metabolic or toxic encephalopathies) account for 24.4% of the cases. Focal epilepsies are the most common form (40.7%), elementary fits being for times more frequent than complex fits.

Maple syrup urine disease, or leucinosis usually presents between the 4 th and 10 th day of life with neurological and gastro-intestinal symptoms. A distinctive odour of maple syrup on the child's skin or of his urine enables diagnosis to be made clinically, confirmation coming from amino-acide chromatography which reveals increased levels of the branched amino acids, leucine, valine and isoleucin in the blood and urine.

The gangliosidoses belong to the family of diseases known as the lipidoses and are due to an excess of ganglioside I (GM1) or II (GM2). The illness described by Landing belongs to Group I, whilst Tay-Sachs and Sandhoff's disease are type 2.

Short-term anaesthesia is defined in terms of duration (allowing brief but what might be painful surgery), rapid recovery, metabolism and mode of administration. After a brief review of barbiturate narcoses, the authors study the electro-clinical features of anaesthesia under Althesin ®, Épontol ® and ketamine. For the first two, although there are some clinical differences, the E.E.G. effects are fairly similar with rapid onset of burst suppression. Recovery is rapid and its study (clinically, with psychomotor tests and E.E.G.) indicates that these drugs can safely be used with out-patients.