Background: Epilepsy has significant implications to quality of life (QOL) beyond the seizures themselves. While research has investigated QOL among people with epilepsy (PwE) from around the globe, minimal research exists on QOL among PwE living in Canada. Methods: People with drug-resistant epilepsy admitted to the Epilepsy Monitoring Unit in London, Ontario completed the “Quality of Life in Neurological Disorders” questionnaire (Neuro-QOL), a scale evaluating 13 QOL domains. We assessed objective cognition using a battery called Creyos. Results: Participants (N=42) scored significantly worse than the reference populations on the anxiety, satisfaction with social roles and activities, and cognitive functioning Neuro-QOL domains (p<.05). Scores on these domains, as well as depression and positive affect/well-being were unrelated to age (mean=39.3 years, SD=16.9), sex (28 females), education level, epilepsy duration, and age of epilepsy onset (p>.05). There were no correlations between scores on these Neuro-QOL domains and Creyos performance in short-term memory, reasoning, and verbal processing (p>.05). Conclusions: PwE living in Canada experience negative QOL in anxiety, social satisfaction, and subjective cognition, unrelated to the clinical factors or cognitive domains investigated. More attention is needed in Canadian clinical care and research to assess and address these affected attributes of QOL for all PwE.

Background: Autonomic nervous system (ANS) dysfunction in people with epilepsy (PwE) is a likely contributor to sudden unexpected death in epilepsy (SUDEP). However, the nature of autonomic dysfunction among PwE remains poorly understood. We aimed to delineate self-reported ANS functioning among people with drug-resistant epilepsy, a patient group at increased risk for SUDEP. Methods: People with focal drug-resistant epilepsy undergoing stereoelectroencephalography at the Epilepsy Monitoring Unit in London, Ontario completed the Composite Autonomic Symptom Score (COMPASS-31), a widely used questionnaire for ANS function. Results: The mean total COMPASS-31 score (N=34; 13 females) was 27.36 (SD=13.77). There was no significant correlation between total COMPASS-31 score and current age (mean=32.71 years, SD=10.58; r(32)= -0.04) or age of epilepsy onset (mean=17.31 years, SD=8.26; r(30)=0). Females scored higher than males (t(32)=3.41, p<.05), but scores did not differ between participants with an epileptogenic zone in the temporal lobe(s) (N=20) and participants with multi-focal, extra-temporal or unknown epileptogenic zones (t(32)=0.18). Participants prescribed 2-3 sodium channel blocking anti-seizure medications (cardiotoxic; N=17), scored worse than participants on 0-1 sodium channel blockers (N=17) (t(32)= -2.15, p<.05). Conclusions: Autonomic testing should be a standard component of clinical care for people with drug-resistant epilepsy, especially for females and for those on sodium channel blockers.

Background: Adverse effects and risks associated with glucocorticoid (GC) treatment are frequently encountered in immune-mediated neuromuscular disorders. However, significant variability exists in the management of these complications. Our aim was to establish international consensus guidance on the management of GC-related complications in neuromuscular disorders. Methods: An international task force of 15 experts was assembled to develop clinical recommendations for managing GC-related complications in neuromuscular patients. The RAND/UCLA Appropriateness Method (RAM) was employed to formulate consensus guidance statements. Initial statements were drafted following a comprehensive literature review and were refined based on anonymous expert feedback, with up to three rounds of email voting to achieve consensus. Results: Consensus was reached on statements addressing general patient care, monitoring during GC therapy, osteoporosis prevention, vaccinations, infection screening, and prophylaxis for Pneumocystis jiroveci pneumonia. A multidisciplinary approach to managing GC-related complications was highlighted as a key recommendation. Conclusions: This represents the first consensus guidance in the neurological literature on GC complications, and offer clinicians structured guidance on mitigating and managing common adverse effects associated with both short- and long-term GC use. They also provide a foundation for future debate, quality improvement, research work in this area.

Background: Antibodies directed against acetylcholine receptor (AChR) are absent in approximately 15% of patients with gMG. Approved treatment options represent an unmet need in the AChR-antibody (Ab)- gMG population. Efgartigimod is an immunoglobulin G1 (IgG1) antibody Fc fragment that selectively reduces IgG levels by blocking neonatal Fc receptor (FcRn)-mediated IgG recycling. Here, we describe efgartigimod efficacy in AChR-Ab- participants with gMG receiving either efgartigimod IV or subcutaneous (SC) efgartigimod PH20 (coformulated with recombinant human hyaluronidase PH20) across clinical studies. Methods: Post hoc analyses were conducted to examine efficacy and safety of efgartigimod IV and/or efgartigimod PH20 SC in AChR-Ab- participants in ADAPT/ADAPT+ and ADAPT-SC/ADAPT-SC+ trials. Results: Among pooled AChR-Ab- participants (n=56), mean (SE) MG-ADL total score improvement from baseline to Week 3 was -3.7 (Cycle 1: 0.44 [n=55]). Consistent MG-ADL improvements occurred with repeated cycles. Clinically meaningful improvements (CMI; ≥2-point MG-ADL decrease) occurred in 76.4% (n=42/55) of participants (Cycle 1, Week 3). In Cycle 1, 23.2% (n=13/56) of participants achieved minimal symptom expression (MG-ADL 0-1). Similar efficacy results occurred across all cycles. Overall safety profile was similar between AChR-Ab+ and AChR-Ab- participants. Conclusions: Both efgartigimod IV and efgartigimod PH20 SC were well tolerated and led to CMI in participants with AChR-Ab- gMG.