Summary: Idiopathic generalized epilepsies (IGEs) are a relatively new category of disorders defined by strict clinical and electroencephalogram (EEG) features proposed by the International League Against Epilepsy (ILAE) classification of epileptic syndromes. IGEs are usually easy to diagnose when clinical and EEG data are collected, but epilepsy is not synonymous with epileptic syndrome. So far, IGEs are studied in the large group of epilepsies of undetermined or unknown etiology although the genetic origin is now largely accepted. ILAE-proposed criteria are helpful in the clinical and therapeutic management of IGEs, but many epidemiologic studies still confuse the cryptogenic and idiopathic groups. Some syndromes in childhood, which are completely described by strict electroclinical criteria such as the absence epilepsies, juvenile myoclonic epilepsies, are usually included and analyzed in epidemiologic studies; however, other epileptic syndromes observed in infancy, such as benign familial neonatal seizures and benign myoclonic epilepsy in infancy, are quite rare and are usually excluded from epidemiologic surveys because they are difficult to describe completely in electro-clinical terms. Another strong limitation in the study of epidemiology of IGEs is the lack of EEG data, either because EEG is not available or the routine EEG is normal. This is particularly relevant in the inclusion of patients with only tonic-clonic seizures. IGEs encompass several different syndromes, and a few patients shift from one phenotype to another. The overlapping of some syndromes during infancy and adolescence increased the difficulty to individualize strictly the correct syndrome. Many discrepancies can be observed in the distribution of the different syndromes included in the group of IGEs, because the strict criteria for classifying these syndromes proposed by the ILAE are often not respected. With this understanding, the general frequency of IGEs can be assessed at 15-20% of all epilepsies. The frequency and the distribution of incidence and prevalence of the different syndromes are tentatively reported and discussed. When the term idiopathic is used following the restrictive ILAE criteria, the mortality data concerning patients with idiopathic epilepsies do not show an increased standardized mortality ratio.

Six severe epileptic patients developed stuporous encephalopathy with marked cognitive impairment when topiramate (TPM) and sodium valproate (VPA) were coprescribed for five patients, and when monotherapy with TPM was introduced for one patient. In four patients, ammonaemia increased and then returned to normal after TPM or VPA withdrawal. This severe potential side effect must be recognized. Moreover two distinct mechanisms might explain this toxicity: (1). a pharmacokinetic interaction between VPA and TPM, leading to hyperammonaemia, (2). a pharmacodynamic mechanism due to a direct toxicity of TPM in at-risk epileptic patients.

Despite controversies and the lack of research, dogs are empirically selected and trained to perform as service dogs, in relation to the dogs’ and future owners’ characteristics. We assessed the characteristics of both humans and dogs in an unbiased population (not selected or trained) of spontaneous seizure-alert by pet dogs and investigated whether we could replicate previous findings. We addressed a self-reporting questionnaire to French people with epilepsy. We analyzed the general characteristics of the humans and pet dogs and their behaviors that could alert their owner before a seizure. In addition, we used the Monash Canine Personality Questionnaire refined to evaluate pet dogs’ personality through five different traits, and the Monash Dog-Owner Relationship scale to assess human–dog relationships. In line with previous reports, we found no particular factor, either pet-, people- or epilepsy-related that could be associated with the presence or absence of alert behaviors. Alert behaviors and circumstances were explored and three different alert patterns emerged. In terms of personality, seizure-alert pet dogs scored significantly higher than non-alerting dogs for the traits “Motivation” and “Training Focus” and lower for “Neuroticism”. The owner–dog bond score was significantly higher for seizure-alert dogs than for non-alerting dogs.

Careful consideration should be given before prescribing propofol as first-line therapy for RSE, and this drug should be avoided altogether if high doses are required. Close biochemical monitoring is needed if propofol is used for more than a few hours, so that PRIS can be recognized promptly. Immediate discontinuation of propofol is essential, and early hemofiltration should be initiated. ECMO should be considered in cases of cardiocirculatory failure.

The authors wish to make the following corrections to this paper: [...]