A reappraisal of interictal EEG characteristics in self-limited epilepsy with autonomic seizures, formerly known as Panayiotopoulos syndrome or early-onset benign occipital epilepsy.

PURPOSE: In the 2022 New International Classification of Epilepsy Syndromes, self-limited epilepsy with autonomic seizures (SeLEAS), formerly known as Panayiotopoulos syndrome is recognized as an electroclinical syndrome that is clinically characterized by autonomic seizures and electroencephalographically by multifocal EEG foci. EEG studies were reviewed herein and the suitability of the EEG definition to characterize SeLEAS was assessed.

METHODS AND RESULTS: The EEG findings of SeLEAS studies published to date were reviewed and typical sites of EEG foci and their evolutionary changes with age were analyzed. Although previous studies investigated the details of interictal EEG characteristics in a sufficient number of SeLEAS cases, there were few systematically analyzing cross sectional and longitudinal EEG changes except one study. Despite these limited evidence, I propose the following practical and useful EEG definition. The interictal EEG characteristics of SeLEAS are multifocal EEG foci with age-dependent predominant locations; occipital (O) at 2-5 years old, and occipital and frontopolar (synchronous and independent O and Fp spikes) at 4-7 years old and centro-parieto-temporal (CPT) at 6-10 years old. O EEG foci evolve to multifocal EEG foci with a O-Fp or CPT predominance with age and disappear by 12∼16 years old. O-Fp EEG foci may further evolve to generalized spike-wave complexes and rarely to spike-wave activated in sleep. In rare cases, the EEGs do not have epileptic EEG foci.

CONCLUSION: Interictal EEG foci in SeLAES may have different EEG patterns in terms of location and the mode of appearance depending on the age at which EEG is recorded. O-Fp EEG foci may be a specific EEG pattern indicating a diagnosis of SeLEAS.