The yield of long-term electrocardiographic recordings in refractory focal epilepsy.

OBJECTIVE: To determine the incidence of clinically relevant arrhythmias in refractory focal epilepsy and to assess the potential of postictal arrhythmias as risk markers for sudden unexpected death in epilepsy (SUDEP). METHODS: We recruited people with refractory focal epilepsy without signs of ictal asystole and who had at least one focal seizure per month and implanted a loop recorder with 2-year follow-up. The devices automatically record arrhythmias. Subjects and caregivers were instructed to make additional peri-ictal recordings. Clinically relevant arrhythmias were defined as asystole ≥ 6 seconds; atrial fibrillation < 55 beats per minute (bpm), or > 200 bpm and duration > 30 seconds; persistent sinus bradycardia < 40 bpm while awake; and second- or third-degree atrioventricular block and ventricular tachycardia/fibrillation. We performed 12-lead electrocardiography (ECG) and tilt table testing to identify non-seizure-related causes of asystole. RESULTS: We included 49 people and accumulated 1060 months of monitoring. A total of 16 474 seizures were reported, of which 4679 were captured on ECG. No clinically relevant arrhythmias were identified. Three people had a total of 18 short-lasting (<6 seconds) periods of asystole, resulting in an incidence of 2.91 events per 1000 patient-months. None of these coincided with a reported seizure; one was explained by micturition syncope. Other non-clinically relevant arrhythmias included paroxysmal atrial fibrillation (n = 2), supraventricular tachycardia (n = 1), and sinus tachycardia with a right bundle branch block configuration (n = 1). SIGNIFICANCE: We found no clinically relevant arrhythmias in people with refractory focal epilepsy during long-term follow-up. The absence of postictal arrhythmias does not support the use of loop recorders in people at high SUDEP risk.

Activity limitations in myasthenia gravis and relation to clinical variables.

INTRODUCTION: It is unknown how fluctuations in muscle weakness affect activity limitations in myasthenia gravis patients and how the severity of these limitations compares with published data on other neuromuscular disorders (NMD). METHODS: In this study we analyzed ACTIVLIM (acronym of "ACTIVity LIMitations") and quantitative myasthenia gravis (QMG) scores. We assessed the impact of QMG and other clinical variables on ACTIVLIM, using B coefficients. RESULTS: The mean ACTIVLIM score in 118 MG patients was 3.3. There was a correlation between QMG and ACTIVLIM (B coefficient = -0.206, P < 0.001) and between changes in both scores (B coefficient = -0.175, P = 0.002). Men and patients without another autoimmune disease had a better ACTIVLIM score (B coefficient = 0.785, P = 0.015 and B coefficient = 0.998, P = 0.008, respectively). CONCLUSIONS: The ACTIVLIM score in MG is higher than in other NMD. Fluctuations in QMG correlated significantly with changes in ACTIVLIM. Muscle Nerve 56: 64-70, 2017.

Cardiac arrhythmias during or after epileptic seizures.

Seizure-related cardiac arrhythmias are frequently reported and have been implicated as potential pathomechanisms of Sudden Unexpected Death in Epilepsy (SUDEP). We attempted to identify clinical profiles associated with various (post)ictal cardiac arrhythmias. We conducted a systematic search from the first date available to July 2013 on the combination of two terms: 'cardiac arrhythmias' and 'epilepsy'. The databases searched were PubMed, Embase (OVID version), Web of Science and COCHRANE Library. We attempted to identify all case reports and case series. We identified seven distinct patterns of (post)ictal cardiac arrhythmias: ictal asystole (103 cases), postictal asystole (13 cases), ictal bradycardia (25 cases), ictal atrioventricular (AV)-conduction block (11 cases), postictal AV-conduction block (2 cases), (post)ictal atrial flutter/atrial fibrillation (14 cases) and postictal ventricular fibrillation (3 cases). Ictal asystole had a mean prevalence of 0.318% (95% CI 0.316% to 0.320%) in people with refractory epilepsy who underwent video-EEG monitoring. Ictal asystole, bradycardia and AV-conduction block were self-limiting in all but one of the cases and seen during focal dyscognitive seizures. Seizure onset was mostly temporal (91%) without consistent lateralisation. Postictal arrhythmias were mostly found following convulsive seizures and often associated with (near) SUDEP. The contrasting clinical profiles of ictal and postictal arrhythmias suggest different pathomechanisms. Postictal rather than ictal arrhythmias seem of greater importance to the pathophysiology of SUDEP.

Value of video monitoring for nocturnal seizure detection in a residential setting.

OBJECTIVE: Following a sudden death at a residential care unit, the Dutch Health and Care Inspectorate advised intensification of the use of video monitoring (VM) at the unit. We assessed whether VM resulted in increased identification of seizures that required clinical intervention. METHODS: The unit provides care for 340 individuals with refractory epilepsy and severe learning disabilities. Acoustic detection systems (ADSs) cover all individuals; 37 people also have a bed motion sensor (BMS) and 46 people with possible nocturnal seizures are now monitored by VM. During a 6-month period, in all cases of a suspected seizure we asked the caregivers to specify which device alerted them and to indicate whether this led to an intervention. Staff costs of VM were estimated using payroll information. RESULTS: We identified 1,208 seizures in 37 individuals: 4 had no nocturnal seizures and 393 (33%) seizures were seen only on video. In 169 (14%) of 1,208 seizures an intervention was made and this included 39 (10%) of 393 seizures seen only on video. When compared to seizures observed with an ADS or BMS, seizures seen only on video were more often tonic seizures (71% vs. 22%, p < 0.001) and occurred mostly in the beginning or at the end of the night (40% vs. 26%, p < 0.001). The extra staff costs of monitoring was 7,035 euro per seizure seen only on video and leading to an intervention. SIGNIFICANCE: VM facilitates nocturnal surveillance, but the costs are high. This underscores the need for development of reliable seizure detection devices.

Nocturnal supervision and SUDEP risk at different epilepsy care settings.

OBJECTIVE: To estimate the incidence of sudden unexpected death in epilepsy (SUDEP) in people with intellectual disabilities in residential care settings and to ascertain the effects of nocturnal seizures and nocturnal supervision on SUDEP risk. METHODS: We conducted a nested case-control study reviewing records of all people who died at 2 residential care settings over 25 years. Four controls per case were selected from the same population, matched on age (±5 years) and residential unit. Nocturnal supervision was graded in 3 categories: (1) no supervision; (2) a listening device or a roommate or physical checks at least every 15 minutes; and (3) 2 of the following: a listening device, roommate, additional device (bed motion sensor/video monitoring), or physical checks every 15 minutes. Outcome measures were compared using Mann-Whitney U tests and Fisher exact tests. RESULTS: We identified 60 SUDEP cases and 198 matched controls. People who died of SUDEP were more likely to have nocturnal convulsive seizures in general (77% of cases vs 33% of controls, p < 0.001) and a higher frequency of nocturnal convulsive seizures. Total SUDEP incidence was 3.53/1,000 patient-years (95% confidence interval [CI] 2.73-4.53). The incidence differed among centers: 2.21/1,000 patient-years (95% CI 1.49-3.27) vs 6.12/1,000 patient-years (95% CI 4.40-8.52). There was no significant difference in nocturnal supervision among cases and controls, but there was a difference among centers: the center with a lowest grade of supervision had the highest incidence of SUDEP. CONCLUSIONS: Having nocturnal seizures, in particular convulsions, may increase SUDEP risk. Different levels of nocturnal supervision may account for some of the difference in incidence.