Successful use of fenfluramine as an add-on treatment for Dravet syndrome.

Purpose: Despite the development of new antiepileptic drugs, Dravet syndrome frequently remains therapy resistant and is a catastrophic epilepsy syndrome. Fenfluramine is an amphetamine-like drug that has been used in the past as a part of antiobesity treatments. Because of the possible cardiac adverse effects (valve thickening, pulmonary hypertension) associated with use of fenfluramine, it was withdrawn from the market in 2001.

Ring chromosome 20 syndrome: electroclinical description of six patients and review of the literature.

Background: Ring chromosome 20 syndrome is a rare chromosomal disorder.

Methods: In six patients, we focused on the presenting epileptic phenotype, the behavioral and mental problems and the relationship between the ratio of mosaicism and the age at onset of the epilepsy.

Results: All patients presented with pharmacoresistant frontal lobe complex partial seizures.

Supracondylar femur fracture complicating epileptic insult: a specific and under diagnosed complication?

Purpose: In epileptic patients fractures are six times more frequent than in the general population. Known predisposing factors are anticonvulsant drugs, malnutrition, lack of physical activity and sunlight exposure.

Methods: In this study we describe two patients, one with a bilateral supracondylar fracture and one with a unilateral supracondylar fracture after an epileptic seizure.

Behavioural and neuropsychological problems in refractory paediatric epilepsies.

The associated behavioural and neuropsychological profiles were studied in 573 children with refractory epilepsy, admitted in our residential Rehabilitation and Epilepsy Unit during the period 1984-2000. The aim of this study was to look for possible correlations between epileptic categories and behavioural profiles. The most frequent neuro-behavioural correlates in the different epilepsy categories were pervasive disorders (48/573=8%), attentional problems (43/573=7.

Severe myoclonic epilepsy in infancy: toward an optimal treatment.

Severe myoclonic epilepsy in infancy, or Dravet syndrome, is one of the catastrophic epilepsy syndromes. In the past, treatment was mainly based on valproate and phenobarbital. Recently, some of the new antiepilepsy drugs, such as topiramate and stiripentol, have been shown to be promising in the treatment of this epilepsy syndrome.