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Purpose Temporal lobe epilepsy with antibodies (abs) against the glutamic acid decarboxylase 65 isoform (GAD-TLE) is known as an immune-mediated neurological syndrome. Here we evaluate the therapy response to various immunotherapies and epilepsy surgery in this syndrome. Method All patients with GAD-TLE and follow-up data and stored serum and CSF samples, identified and treated at the Bonn centre from 2002 to 2010, were studied retrospectively. Seizure freedom for ≥1 year and reduction of ≥50%, i.e. therapy response, were assessed. GAD-ab titres and neuropsychological performances were documented prior and after individual interventions. Results Thirteen patients with GAD-TLE were identified with the following seizure responses: corticosteroids (5 responders out of 11 treated patients); i.v. immunoglobulins (1/5), apheresis therapy (1/8); and natalizumab (1/1), selective amygdala-hippocampectomy (2/3). None of the patients achieved sustained seizure freedom apart from one patient. This patient was on antiepileptic drug treatment after discontinuation of immunotherapy. Conclusion The seizure response to immunotherapies in patients with GAD-TLE was poor. Corticosteroids were the most effective regarding seizure response. Especially the poor effects of apheresis therapies support the idea that GAD-abs are not directly pathogenic. None of three patients was seizure-free after temporal lobe surgery suggesting that GAD-TLE patients respond worse than others to this type of intervention. Our results reflect the chronic course of the disease with low likelihood for patients with GAD-TLE to attain long-term seizure freedom. [ABSTRACT FROM AUTHOR]

We describe two male patients with focal epilepsy in whom transitory episodes of atrial fibrillation (AF) lasting for up to 25h were detected in the context of generalized tonic-clonic seizures (GTCSs). In five of seven previously published cases of transitory AF associated with epileptic seizures, AF was also associated with GTCS, suggesting a pathophysiological link via GTCS-related increase in sympathetic tone and release of catecholamines. Importantly, AF increases the risk of thromboembolic cerebral ischemia, prompting the question of whether antithrombotic preventive treatment should be initiated in people with pharmacoresistant epilepsy and prolonged peri-ictal AF. Furthermore, AF can considerably impair cardiac output and may, via this mechanism, contribute to the risk of sudden unexpected death in epilepsy following GTCS. [ABSTRACT FROM AUTHOR]

Patient-reported seizure frequency is a key measure of modern epileptology regarding both research and individual treatment. Several studies from the last two decades, however, provided unambiguous evidence that patients underreport seizures. Here, we wanted to assess how patients experience the self-monitoring of their seizures and how they evaluate the data quality of their diaries. A novel 10-sided questionnaire addressed the following domains: sociodemographic characteristics, personality, epilepsy, treatment, seizure experience (separately for day and night), and documenting seizures/not documenting seizures, respectively. The survey was distributed to 1100 adult patients from our unit. We finally received N = 170 usable questionnaires (15.3%) indicating the patients’ little interest in this issue. On average, patients estimated to be aware of 5.3 out of 10 daytime seizures (95% CI 4.1–6.5) while relatives/colleagues appear to notice 7.1 (95% CI 6.1–8.1). For nocturnal seizures, patients stated to notice 2.6 out of 10 (95% CI 1.5–3.7) while relatives notice 4.6 (95% CI 3.4–5.8). One-hundred-four patients (61%, 95% CI 54–68%) reported to keep a seizure diary. These patients estimated to document 87% (95% CI 79–95%) of all daytime seizures they were aware of; for perceived nocturnal seizures the documentation rate was 77% (95% CI 67–87%). From this patient-reported data, the rate of documented daytime seizures can be estimated as 62% (95% CI 48–77%) in documenters and 38% (95% CI 26–53%) in the total sample. The respective percentages for documented nocturnal seizures are 35% (95% CI 23–50%) in documenters and 21% (95% CI 12–34%) in the total sample ( Fig. 1 ). In view of the low return rate, our data are even likely to overestimate the average documentation quality. Patients are well aware of the inherent difficulties to provide valid seizure counts and, consistent with many studies, lacking seizure awareness is the main cause of seizure underreporting. Note: First authorship is shared by CH and BB. [ABSTRACT FROM AUTHOR]

Purpose Epileptic seizures are frequently accompanied by alterations of autonomic function. Previous studies have suggested that especially changes in heart rate (HR) may help to distinguish epileptic from psychogenic, non-epileptic seizures (PNES). Some of these studies may be confounded by various features such as age, gender, state of wakefulness and seizure semiology. Here, we re-assessed periictal HR modulation in temporal lobe seizures (TLES) and PNES adjusted for a number of known confounders. Methods We retrospectively reviewed video-EEG data of people with refractory temporal lobe epilepsy undergoing presurgical assessment and of age- and gender matched patients without known epilepsy presenting with PNES. One seizure per patient was analyzed. HR was assessed with the help of a one-channel ECG lead at different time points (2 min before, during the seizure where HR was highest, immediately after seizure cessation, and 1, 2, 3, 5 and 10 min postictally). Data are given as mean ± SD. Results A total of 189 of patients and seizures were included in the study. Sixty TLES arising from wakefulness (20 men, 34 ± 12 years) and 129 PNES were analyzed. In the PNES group, 60 of the seizures had occurred spontaneously from wakefulness (PNES-S, 20 men, 37 ± 14 years), while 69 were induced by a provocation maneuver (verbal suggestion along with intravenous injection of vitamins; PNES-P, 23 men, 36 ± 14 years). In the PNES-P group, baseline HR was significantly higher as compared to the two other groups ( p < 0.01). In the TLES group, relative HR changes were significantly higher during and shortly after seizure cessation, as compared to the PNES-S group ( p 0.002). During seizures with major motor phenomena (generalized epileptic or psychogenic convulsions), relative HR changes during the seizures were not different ( p = 0.165, convulsive TLES n = 11, convulsive PNES-S n = 36). However, in the first three minutes after seizure cessation, HR was higher in the major motor TLES group ( p values < 0.01, 0.09 and 0.07). Furthermore, non-motor TLES displayed consistently higher relative HR changes during and shortly after seizure cessation up to 2 min postically as compared to the non-motor PNES-S. Conclusions TLES display higher relative HR changes during non-motor seizures as well as shortly after major motor and non-motor seizures, as compared to PNES-S. The setting of a provocation maneuver itself appears to enhance baseline HR. Taken together, our results support the notion that analysis of periictal HR is helpful in the differential diagnosis of epileptic versus psychogenic, non-epileptic seizures. [ABSTRACT FROM AUTHOR]