The Axial Spasm—The Predominant Type of Drop Seizure in Patients with Secondary Generalized Epilepsy

Abstract

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....

Partial Epilepsy Manifesting Atonic Seizure: Report of Two Cases

“…EMGs were not recorded in patient 2, but during the development of the atonic state, there was no apparent contamination of EMG activity in the scalp‐recorded EEG, and furthermore, hypotonus was confirmed in all limbs by clinical examination during the attack. All these facts suggest that the slow falls in our patients are different from “slow falls” reported by Egli et al (5).…”

“…This rather slow development might be characteristic of the atonic seizure occurring as a partial epilepsy, which has never been emphasized previously. “Slow falls” were reported in patients with secondary generalized epilepsy by Egli et al (5), but the falls in those patients occurred during a slowly developing tonic seizure. Patient 1 in the present study showed the slow falls when EMG activities of the deltoid muscle showed complete attenuation (Fig.…”

Seizures in Series: Similarities Between Seizures of the West and Lennox‐Gastaut Syndromes

“…Apart from WS and LGS, generalized seizures associated with generalized sharp or slow waves, decrements, and/or low-voltage fast rhythms are unusual (Delgado-Escueta, 1979 Egli et al (1985) described children and young adults with secondary generalized epilepsy who had axial spasms. They believed that this type of seizure may be a more mature form of infantile spasms.…”

Onset of action and seizure control in Lennox-Gaustaut syndrome: focus on rufinamide

“…Drop attacks caused by exclusive loss of muscle tone are rare 23. Most drop attack seizures are brief motor events associated with a fall and represent either myoclonic-atonic, myoclonic, or tonic seizures 23,24. The majority of the epileptic falls in patients are the result of tonic falls 23,24.…”

“…Most drop attack seizures are brief motor events associated with a fall and represent either myoclonic-atonic, myoclonic, or tonic seizures 23,24. The majority of the epileptic falls in patients are the result of tonic falls 23,24. These seizures are the most difficult to treat and most dramatic to witness due to bodily injuries involving the face and head.…”