Consensus statements on the information to deliver after a febrile seizure.

Febrile seizures (FS) are usually self-limiting and cause no morbidity. Nevertheless they represent very traumatic events for families. There is a need to identify key messages that reassure carers and help to prevent inappropriate, anxiety-driven behaviors associated with "fever phobia." No recommendations have been proposed to date regarding the content of such messages. Using a Delphi process, we have established a consensus regarding the information to be shared with families following a FS. Twenty physicians (child neurologists and pediatricians) from five European countries participated in a three-step Delphi process between May 2018 and October 2019. In the first step, each expert was asked to give 10 to 15 free statements about FS. In the second and third steps, statements were scored and selected according to the expert ranking of importance. A list of key messages for families has emerged from this process, which offer reassurance about FS based on epidemiology, underlying mechanisms, and the emergency management of FS should they recur. Interestingly, there was a high level of agreement between child neurologists and general pediatricians.Conclusion: We propose key messages to be communicated with families in the post-FS clinic setting. What is Known: • Febrile seizures (FS) are traumatic events for families. • No guidelines exist on what information to share with parents following a FS. What is New: • A Delphi process involving child neurologists and pediatricians provides consensual statement about information to deliver after a febrile seizure. • We propose key messages to be communicated with families in the post-FS clinic setting.

Exploring the informational sources of metaperception: the case of Change Blindness Blindness.

Perceivers generally show a poor ability to detect changes, a condition referred to as "Change Blindness" (CB). They are, in addition, "blind to their own blindness". A common explanation of this "Change Blindness Blindness" (CBB) is that it derives from an inadequate, "photographical" folk-theory about perception. This explanation, however, does not account for intra-individual variations of CBB across trials. Our study aims to explore an alternative theory, according to which participants base their self-evaluations on two activity-dependent cues, namely search time and perceived success in prior trials. These cues were found to influence self-evaluation in two orthogonal ways: success-feedback influenced self-evaluation in a global, contextual way, presumably by recalibrating the norm of adequacy for the task. Search time influenced it in a local way, predicting the success of a given trial from its duration.

Tremor-like subcortical myoclonus in STXBP1 encephalopathy.

The phenotypic spectrum of STXBP1-related encephalopathy ranges from infantile epileptic encephalopathy to intellectual disability with nonsyndromic or absent epilepsy. Although being frequently reported, the tremor associated with STXBP1 has not been fully characterized to date. The aim of our study was to describe it. We recruited patients with intellectual disability due to STXBP1 variants, regardless of their epileptic phenotype, who had tremor at examination and who underwent neurophysiological testing including polymyographic registration of upper limbs muscles activity at rest, during posture maintenance and action. Six patients met the inclusion criteria over four years. Clinically, all had a postural and action distal tremor increased by emotions. Neurophysiological recordings showed a specific myoclonus pattern and were highly suggestive of a subcortical generator. The tremor-like observed in STXBP1 encephalopathy is due to a subcortical pseudo-rhythmic myoclonus.

Autistic spectrum disorder and epilepsy: diagnostic challenges.

Introduction: Epilepsy is more frequent in individuals with Autism Spectrum Disorder (ASD) than in the general population; however, its diagnosis is frequently challenging. Areas Covered: We report the current diagnostic criteria for both ASD and epilepsy. We describe the incidence, prevalence, and risk factors for epilepsy in patients with ASD. We then focus on the electro-clinical approach, including the clinical evaluation of cognitive regression. Expert Opinion: A diagnosis of epilepsy should be made based on the International League Against Epilepsy (ILAE) definition. A diagnosis of epilepsy should be established based on a single seizure with electroencephalography (EEG) abnormalities. Considering the high prevalence of EEG abnormalities in children with ASD without epilepsy, EEG should only be performed at epilepsy onset, and more precisely when a clinical interview has confirmed that repetitive paroxysmal events could be seizures. There are still many gaps in our understanding of epilepsy in patients with ASD. It would be of interest to further understand the links, if any, between EEG abnormalities and ASD phenotype. The identification of epilepsy syndromes in ASD would help analyze the possible underlying etiologies, for the administration of more appropriate antiepileptic drugs (AED), and to explain the prognosis to caregivers.

Absence of increased blood decanoic acid levels in children with epilepsy treated with classic ketogenic diet.

Recently, decanoic acid (C10), a medium-chain fatty acid, was shown to be a direct inhibitor of the AMPA receptor. Accordingly, C10 has been suggested as a potential anticonvulsant factor in the ketogenic diet (KD) or the medium-chain triglyceride KD. Here, we tested whether C10 serum levels correlate with the response to KD in five children (1.5 ± 0.6 years of age) with epilepsy. The serum levels of C10 were measured before and after KD initiation (n=2 at one month, n=3 at three months, and n=1 at six months after initiation) by gas chromatography-mass spectrometry. After three months on KD, two patients were found to be responders. The mean serum level before KD initiation was 63.2 µM. Only one patient, who was a non-responder, showed an increase (5%) in C10 serum level after a month of KD. The remaining four patients (two responders) showed a decrease in the C10 level from -5.3% to -75.5%. Our preliminary data show that KD does not lead to an increase in C10 serum levels, suggesting that increased concentration of C10 might not be directly involved in the anticonvulsant effects of classic KD.