Paolo Tinuper graduated from the University of Bologna (Italy) and trained at the Neurological Institute of Bologna under Prof. E. Lugaresi. He gained further expertise at the Marseille School with Prof. H. Gastaut and at the Montreal Neurological Institute with Dr. F. Andermann. He is Associate Professor of Neurology at the University of Bologna, in charge of the Epilepsy Center and the EEG laboratory of the Department of Neurological Sciences. President of the Italian League against Epilepsy, Certified Trainer of the European Epilepsy Academy and Ambassador for Epilepsy. He is involved in teaching epileptology and clinical neurophysiology in undergraduate and postgraduate university courses and in national courses. Author of many scientific papers in international peer-reviewed journals, his main scientific interests include semeiological and neurophysiological aspects of epileptic seizures, prognosis of the epileptic syndromes, prognosis of epilepsy after drug withdrawal, epilepsies with seizures during sleep and presurgical evaluation in drug-resistant epilepsies.
A 42-year-old right-handed woman began having attacks at 38 years of age. Seizures were characterized by a feeling of something warm flushing from her chest to the face. Then she lost consciousness and fell if she was standing up. No automatisms or other ictal signs were reported. Seizures occurred monthly, clustering around the time of her menses. Because her attacks were believed to be of cardiac origin, cardiological tests, including 24-hour electrocardiographic monitoring and autonomic investigations, were performed. These tests were normal. The patient was therefore referred to a neurologist.
Examination and investigations
Neurological examination was normal. Interictal EEG showed focal spikes in the left temporal region. Brain magnetic resonance imaging showed a left mesiotemporal lesion, probably a low grade astrocytoma.
A seizure was recorded during video-polygraphic monitoring with the patient lying down. At the beginning of the seizure, the EEG showed low amplitude fast activity over the left temporal area, which was then replaced by a prolonged run of sharp discharges in the same region. The beginning of the discharges coincided with a progressive slowing of heart rate, at which time the patient reported the usual sensation of flushing in her face and faintness. Normal heart rate was regained at the end of the seizure.
Lesional left temporal lobe epilepsy with arrhythmogenic seizures.
Phenytoin was started without any improvement in seizure frequency.
In clinical practice, the differential diagnosis between syncopal attacks and epileptic seizures is a frequent problem and the semiological and anamnestic data are not always sufficient to clarify the picture. In this case, the simultaneous EEG and electrocardiography documented the change in heart rate during the seizure. Had the patient been standing up instead of lying on the laboratory bed, she would have fallen to the ground with a syncopal attack.
An increase in heart rate is commonly reported during epileptic seizures; this is probably related to adrenergic activation. Arrhythmogenic seizures, in particular bradycardic, are rarely described in the literature, probably owing to the lack of polygraphic (extracephalic) parameters during prolonged video-monitoring sessions.
For many reasons, it is important to be aware of the possibility of ictal modifications of heart rate during seizures. First, ictal arrhythmias are potentially dangerous and affected patients must be considered at risk of life-threatening episodes. Secondly, commonly used antiepileptic drugs (such as carbamazepine or phenytoin) have some effect on heart conduction and may worsen the picture. Finally, in those patients with frequent and drug-resistant arrhythmogenic attacks, the need for a cardiac pacemaker must be evaluated.
What did I learn from this case?
This case enhanced my interest in vegetative changes during partial epileptic seizures. The mesial structures of the temporal lobes are densely interconnected with the central autonomic structures, and consequently mesial temporal epileptic foci may influence autonomic parameters such as heart rate and blood pressure, either interictally or, more dramatically, during seizures. Because most partial seizures are recorded during long-term video-monitoring as patients with drug-resistant seizures undergo presurgical evaluation, I recommend adding an electrocardiographic tracing to the recording montage. Otherwise, arrhythmogenic seizures may be missed. Patients with this unusual type of seizure must be investigated accurately and treated appropriately.
- Benarroch EE. The central autonomic network: functional organisation, dysfunction, and perspective. Mayo Clin Proc 1993;68:988–1001.
- Blumhardt LD, Smith PEM, Owen L. Electrocardiographic accompaniments of temporal lobe epileptic seizures. Lancet 1986;1(8489):1051–5.
- Devinsky O, Pacia S, Tatambhotla G. Bradycardia and asystole induced by partial seizures: a case report and literature review. Neurology 1997;48:1712–4.
- Jallon P. Arrhythmogenic seizures. Epilepsia 1997;38:43–7.
- Reeves AL, Nollet KE, Klass DW, Sharbrough FW, So EL. The ictal bradycardia syndrome. Epilepsia 1996;37:983–7.
- Van Buren JM, Ajmone-Marsan C. A correlation of autonomic and EEG components in temporal lobe epilepsy. Arch Neurol 1960;3:683–703.
- Wannamaker BB. Autonomic nervous system and epilepsy. Epilepsia 1985;26(suppl 1):S31–S39.
This selection for "Challenging Cases" is from 110 Puzzling Cases of Epilepsy, edited by Dieter Schmidt, MD, and Steven C. Schachter, MD (Martin Dunitz, Publisher, London, 2002).