Ictal asystole is a rare feature of patients with focal epilepsy. Delayed loss of tone is distinctly uncommon in patients with temporal lobe seizures, but may inevitably occur in patients with ictal asystole after a critical duration of cardiac arrest and cerebral hypoperfusion. Further cardiac monitoring in patients with temporal lobe epilepsy and a history of unexpected collapse and falls late in the course of a typical seizure may be warranted and can potentially help to prevent sudden unexplained death in epilepsy.

SUMMARYIctal asystole (IA) has been implicated as a preventable cause of sudden unexplained death in epilepsy presumably provoked by a direct autonomic effect of the electrical stimulus on the heart. An electronic database search of patients with IA was performed comparing heart rate (HR) characteristics to a group of patients with vasovagal asystole. IA was seen in eight patients, all with temporal lobe epilepsy. No statistical difference was found in duration of asystole, bradycardia, and baseline HR characteristics except of a higher HR acceleration postasystole in the controls. None of the six patients with IA who underwent pacemaker implantation had recurrence of asystolic events during mean follow-up of 5 years. This study in a small group of patients suggests that the epileptic activation leading to IA is possibly mediated through a transient increase in vagal tone and not by a direct autonomic effect on the heart. KEY WORDS: Ictal asystole, Syncope, Sudden unexplained death in epilepsy, SUDEP.

Introduction: Universally established comprehensive clinical bulbar scales objectively assessing disease progression in amyotrophic lateral sclerosis (ALS) are currently lacking. The goal of this working group project is to design a best practice set of provisional bulbar ALS guidelines, available for immediate implementation within all ALS clinics. Methods: ALS specialists across multiple related disciplines participated in a series of clinical bulbar symposia, intending to identify and summarize the currently accepted best practices for the assessment and management of bulbar dysfunction in ALS Results: Summary group recommendations for individual speech, Augmentative and Alternative Communication (AAC), and swallowing sections were achieved, focusing on the optimal proposed level of care within each domain. Discussion: We have identified specific clinical recommendations for each of the 3 domains of bulbar functioning, available for incorporation within all ALS clinics. Future directions will be to establish a formal set of bulbar guidelines through a methodological and evidence‐based approach. Muscle Nerve 59:531–531, 2019

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The working goal is to establish a clinical bulbar protocol, designed to be incorporated within ALS clinics and ultimately to formulate a best practice set of bulbar ALS guidelines, available for implementation throughout the international ALS community.

Current therapeutic development in amyotrophic lateral sclerosis (ALS) relies on individual randomized clinical trials to test a specific investigational product in a single patient population. This approach has intrinsic limitations, including cost, time, and lack of flexibility. Adaptive platform trials represent a novel approach to investigate several interventions for a single disease in a continuous manner. Already in use in oncology, this approach is now being employed more often in neurology. Here, we describe a newly launched platform trial for ALS. The Healey ALS Platform Trial is testing multiple investigational products concurrently in people with ALS, with the goal of rapidly identifying novel treatments, biomarkers, and trial endpoints.

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