BRIVA-ONE study: 12-month outcomes of brivaracetam monotherapy in clinical practice.

Objective: This study investigated the effectiveness and tolerability of brivaracetam (BRV) monotherapy in a large series of patients with epilepsy.

Method: This was a multicenter, retrospective, observational, non-interventional study in 24 hospitals across Spain. Patients aged ≥18 years who started on BRV monotherapy, either as first-line or following conversion, at least 1 year before database closure were included.

Epilepsy and musical perception. A perspective involving 14 patients.

Introduction: The relationship between epilepsy and music is poorly understood. Musicogenic epilepsy, which involves seizures triggered by music, and epilepsy that triggers or involves musical experiences are rare. Anti-seizure medications (ASMs) may affect cognition and possibly the musical sphere.

[Seizures in the emergency service: the clinical and therapeutic characteristics of 122 patients].

Introduction: Epileptic seizures are a common cause of admission in emergency services at hospitals. Performing the correct diagnosis can be difficult, and deciding when and which anti-seizure medication (ASM) to prescribe is critical. Our objective is to detail the characteristics of patients treated in a medium-sized hospital for this reason.

Real-life evidence about the use of intravenous brivaracetam in urgent seizures: The BRIV-IV study.

Purpose: Urgent seizures are a medical emergency for which new therapies are still needed. This study evaluated the use of intravenous brivaracetam (IV-BRV) in an emergency setting in clinical practice.

Methods: BRIV-IV was a retrospective, multicenter, observational study.

[The sign of the cross: a very rare automatism in temporal lobe epilepsy. Two case reports].

Introduction: In the evaluation of drug-resistant epilepsy (DRF), a detailed analysis of the semiology is essential to establish a diagnostic hypothesis of the location of the epileptogenic zone. Cross-sign (CS) is a very infrequent complex manual automatism described for the first time in 2008 and rarely reported in the literature.

Case Report: We present two cases from our series of patients monitored by videoEEG, one of whom also studied with deep electrodes, in which we describe the location of the discharge while performing the CS.

ATG16L1 WD40 domain-dependent IL10R (interleukin 10 receptor) signaling is insensitive to the T300A Crohn disease risk polymorphism.

A coding allele of that increases the risk of Crohn disease (T300A; rs2241880) impairs the interaction between the C-terminal WD40 domain (WDD) and proteins containing a WDD-binding motif, thus specifically inhibiting the unconventional autophagic activities of ATG16L1. In a recent publication we described a novel atypical role of ATG16L1 in the regulation of IL10R (interleukin 10 receptor) trafficking and signaling, an activity that involves direct interaction between the WDD and a target motif present in IL10RB (interleukin 10 receptor subunit beta). Here we show that, unexpectedly, neither the ability of ATG16L1 to interact with IL10RB nor its role in supporting IL10 signaling are altered by the T300A mutation.

Unconventional WD40 domain-dependent role of ATG16L1 in the regulation of IL10R (interleukin 10 receptor) endocytosis, trafficking and signaling.

ATG16L1 is a critical mediator of macroautophagy/autophagy required for LC3 lipidation and autophagosome formation. However, ATG16L1 has a C-terminal domain including 7 WD40-type repetitions (WD40 domain, WDD) that is unnecessary for the conventional autophagic pathway. Instead, this domain mediates unconventional activities where LC3 is lipidated in atypical subcellular localizations unrelated to canonical double-membrane autophagosomes.

Regulation of cytokine signaling through direct interaction between cytokine receptors and the ATG16L1 WD40 domain.

ATG16L1, an autophagy mediator that specifies the site of LC3 lipidation, includes a C-terminal domain formed by 7 WD40-type repeats (WD40 domain, WDD), the function of which is unclear. Here we show that the WDD interacts with the intracellular domain of cytokine receptors to regulate their signaling output in response to ligand stimulation. Using a refined version of a previously described WDD-binding amino acid motif, here we show that this element is present in the intracellular domain of cytokine receptors.