Summary Purpose:  Ictal asystole (IA) and ictal bradycardia (IB) are rare autonomic symptoms during epileptic seizures and may be potentially life‐threatening. Guidelines for the care of these patients are missing. The aim of this multicenter study was to evaluate the management and long‐term outcome in patients with IA and IB. Patients and Methods:  All patients with IA and IB were included from four epilepsy centers (Bielefeld, Kork, Marburg, and Zürich) from 2002 until 2009. Using a standardized assessment form, clinical data, treatment decisions, and outcomes were extracted from patient charts and simultaneous electroencephalography/electrocardiography (EEG/ECG) recordings. Key Findings:  Seizures with IA or IB were identified in 16 patients. In all patients an associated temporal seizure pattern was recorded and in 15 patients, sudden falls, fainting, or trauma was previously reported or recorded during the monitoring. In three patients (18.8%) diagnosis of focal epilepsy was newly established and anticonvulsive treatment was initiated. Two patients with refractory epilepsy underwent epilepsy surgery. In seven patients (43.8%) a cardiac pacemaker was implanted. In 14 of 16 treated patients, seizure freedom (n = 5) or absence of sudden falls, fainting, or trauma (n = 9) could be achieved. Two patients denied epilepsy surgery as well as a pacemaker and continue to have frequent falls and trauma. Significance:  Our study demonstrates that epilepsy surgery and antiepileptic drugs may lead to sustained freedom of seizures as well as ictal syncope. In drug‐resistant patients not suitable for epilepsy surgery, implantation of a cardiac pacemaker may prevent sudden falls as well as trauma. Based on our results and previously reported cases we propose a treatment algorithm.

Summary: In 45 patients suffering from secondary generalized epilepsy, we have recorded 239 drop seizures both by radio‐telemetered EEG/split‐screen video taping and poly‐graphically. Twenty of these patients had pure tonic drop seizures. Nine patients each had myoclonic‐atonic or pure atonic seizures, whereas seven had slow falls that occurred during gradually developing tonic or akinetic seizures. We term the pure tonic drop seizure “axial spasm.” It consists of a uniform pattern of movement lasting from 0.5 to 0.8 s and leading to a specific bearing characterized by a moderate flexion of the hips, the upper trunk, and the head. The arms are almost always involved being abducted, elevated and in a semiflexed position. A fall is provoked by the rapidity and violence of the flexion in the hips. The spasms can occur independently (pure axial spasm) or in connection with other seizure manifestations (combined axial spasm). In pure spasms, no disturbance of consciousness and no significant EEG changes—apart from a slight attentuation—are seen. In the combined axial spasms, distinct epileptic phenomena, in close connection with the axial spasms, can be observed, appearing either in the postspasm phase or in both the pre‐ and the afterphases. The prephase consists always of an absence and spike‐and‐wave activity. The onset of the spasm coincides with the last generalized spike, which is followed by a biphasic high‐voltage slow wave. In the afterphases, various seizure patterns can be seen; quite characteristic are arrest states during which the patients are completely immobile and take up a postural‐like or athetoid position. Almost as frequent are absences or absence‐like states and tonic seizures. The afterphase can be compounded by two or three of these seizure patterns, the most common sequence of events in these cases being tonic—arrest state—absence. From the clinical and neurophysiological points of view, axial spasms can be regarded as a more mature form of infantile spasms. Clinical and experimental findings suppose a brainstem origin. ZUSAMMENFASSUNG Bei 45 Patienten mit sekundärer generalisierter Epilepsie haben wir in den letzten Jahren 239 Sturzanfälle abgeleitet, 174 radio‐telemetrisch mit Video im Doppelbild‐Verfahren, 65 polygraphisch. Bei 20 Patienten waren die Sturzanfälle rein tonisch, bei je 9 Patienten myoklonisch‐atonisch oder rein atonisch. 7 Patienten hatten langsame Stürze, welche im Verlauf allmählich sich entwickelnder tonischer oder bei akine‐tischen Anfällen auftraten. Die tonischen Sturzanfälle, die wir als axiale Spasmen bezeichnen, beruhen auf einem gleichförmigen Bew‐egungsmuster von 0.5–0.8 Sekunden Dauer. Der axiale Spasmus führt zu einer charakteristischen Haltung mit mässiger Flexion der Hüftge‐lenke, des oberen Rumpfs und des Kopfes. Die Arme sind fast immer beteiligt. Sie werden in Semiflexions‐Stellung abduziert und bis auf Schulterhöhe eleviert. Die rasante Flexion in den Hüftgelenken ist für den Sturz verantwortlich. Zwei Formen axialer Spasmen können unterschieden werden: 1....

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The objective of this clinical practice guideline is to provide recommendations on the indications and minimum standards for inpatient long-term videoelectroencephalographic monitoring (LTVEM). The Working Group of the International League Against Epilepsy and the International Federation of Clinical Neurophysiology develop guidelines aligned with the Epilepsy Guidelines Task Force. We reviewed published evidence using the PRISMA (Preferred Reporting Items for Systematic Review and Meta-Analysis) statement. We found limited highlevel evidence aimed at specific aspects of diagnosis for LTVEM performed to evaluate patients with seizures and nonepileptic events. For classification of evidence, we used the Clinical Practice Guideline Process Manual of the American Academy of Neurology. We formulated recommendations for the indications, technical requirements, and essential practice elements of LTVEM to derive minimum standards used in the evaluation of patients with suspected epilepsy using GRADE (Grading of Recommendations Assessment, Development, and Evaluation). Further research is needed to obtain evidence about long-term outcome effects of LTVEM and to establish its clinical utility.