Interictal Epileptiform Discharge Dynamics in Peri-sylvian Polymicrogyria Using EEG-fMRI.

Polymicrogyria (PMG) is a common malformation of cortical development associated with a higher susceptibility to epileptic seizures. Seizures secondary to PMG are characterized by difficult-to-localize cerebral sources due to the complex and widespread lesion structure. Tracing the dynamics of interictal epileptiform discharges (IEDs) in patients with epilepsy has been shown to reveal the location of epileptic activity sources, crucial for successful treatment in cases of focal drug-resistant epilepsy. In this case series IED dynamics were evaluated with simultaneous EEG-fMRI recordings in four patients with unilateral peri-sylvian polymicrogyria (PSPMG) by tracking BOLD activations over time: before, during and following IED appearance on scalp EEG. In all cases, focal BOLD activations within the lesion itself preceded the activity associated with the time of IED appearance on EEG, which showed stronger and more widespread activations. We therefore propose that early hemodynamic activity corresponding to IEDs may hold important localizing information potentially leading to the cerebral sources of epileptic activity. IEDs are suggested to develop within a small area in the PSPMG lesion with structural properties obscuring the appearance of their electric field on the scalp and only later engage widespread structures which allow the production of large currents which are recognized as IEDs on EEG.

Early post-marketing experience with edaravone in an unselected group of patients with ALS.

Introduction: Treatment with edaravone has shown efficacy in a subgroup of patients with amyotrophic lateral sclerosis (ALS). However, it has been estimated that <7% of ALS patients fulfill the stringent inclusion criteria of the trial. In the current study, we aimed to explore retrospectively the efficacy of edaravone in unselected ALS patients. Methods: Demographic and clinical data were retrospectively collected for 22 patients who opted for treatment with edaravone and 71 untreated ALS patients attending the ALS clinic at Tel Aviv Sourasky Medical Center, Tel Aviv, Israel, between May 2017 and January 2018. Clinical data were extracted for a baseline visit at treatment onset, and for pre-baseline and post-baseline visits, roughly 6 months apart from the baseline visit. Results: Baseline demographic and clinical characteristics were similar between edaravone treated and untreated patients, except for shorter disease duration in edaravone treated patients. Muscle strength, ALS Functional rating scale (ALSFRS-R), and respiratory function were similar between edaravone treated and untreated patients, including monthly rate of decline before and after baseline visit. Among treated patients, 7 had major respiratory complications occurring between 8 days to 7 months after treatment initiation, during or within hours following infusions. Discussion: Results of our study comparing edaravone treated and untreated patients in a real-life setting showed no differences in the rate of monthly decline of ALSFRS-R, in line with previous reports in ALS patients not treated with edaravone, and a previous clinical trial. Our findings might suggest that edaravone is not effective in unselected ALS patients.