Documentation of autonomic seizures and autonomic status epilepticus with ictal EEG in Panayiotopoulos syndrome Nicola Specchio a, ⁎, Marina Trivisano b , Dianela Claps a , Domenica Battaglia c , Lucia Fusco a , Federico Vigevano a a Division of Neurology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy b Clinic for Nervous System Diseases, University of Foggia, Foggia, Italy c Child Neurology Unit, Ospedale Gemelli, Rome, Italy abstract article info Article history: Received 23 June 2010 Accepted 26 June 2010 Available online 24 August 2010 Keywords: Panayiotopoulos syndrome Ictal EEG Ictal tachycardia Ictus emeticus Occipital epilepsy Panayiotopoulos syndrome (PS) is a common childhood susceptibility to autonomic seizures and status epilepticus. Despite its high prevalence, PS has been a source of significant debate. We present ictal EEG documentation of autonomic seizures and autonomic status epilepticus in six cases of PS and a review of 14 reported cases. Interictal EEGs showed spikes of variable locations that often changed with time. Ictal EEG onsets were also variable, starting from wide anterior or posterior regions usually with theta waves intermixed with small spikes and fast rhythms. Ictal vomiting and other autonomic manifestations, as well as deviation of the eyes, did not appear to relate to any specific region of EEG activation. These data document that PS is a multifocal autonomic epilepsy and support the view that the clinical manifestations are likely to be generated by variable and widely spread epileptogenic foci acting on a temporarily hyperexcitable central autonomic network. © 2010 Elsevier Inc. All rights reserved. 1. Introduction Panayiotopoulos syndrome (PS) is one of the most significant types of epilepsy recently recognized by the International League Against Epilepsy, which also has rightly abandoned the previously synony- mous descriptive term early onset benign childhood occipital epilepsy [1]. The clinical, EEG, and magnetoencephalographic features and prognosis of PS have been confirmed through independent studies of more than 600 patients [2–7]. PS is common, affecting around 6% of children with afebrile seizures, manifests as autonomic seizures and autonomic status epilepticus that are specific to childhood, provides links with mainly rolandic epilepsy, and is frequently misdiagnosed as encephalitis and other nonepileptic disorders. Prognosis with respect to seizure remission and neuropsychological development is excellent. Interictal EEGs show marked variability in spike localizations although initial studies diverted attention toward occipital spikes despite the fact that in the original study of Panayiotopoulos, one- third of patients had extra-occipital spikes and sometimes normal EEGs [8]. We were among the first to emphasize that such patients may have consistently normal interictal EEGs [9]. An intriguing feature of pathophysiological significance is that the clinical manifes- tations of autonomic seizures in PS are similar irrespective of interictal EEG abnormalities [5,10]. The ictal semiology of PS is based mainly on descriptions by patients and witnesses. This, together with the fact that seizures are mainly nocturnal, could explain variations among authors regarding the prevalence of the various types of ictal symptoms. Furthermore, because of its unusual ictal clinical manifestations, few authors do not distinguish between PS and idiopathic childhood occipital epilepsy or even doubt its existence [11–13]. EEGs and, in particular, video/EEG recordings of ictal events are the gold standard in these matters and are diagnostic for seizure categorization. In PS, as in rolandic epilepsy, because patients have single or rare seizures, only 14 cases for which ictal EEGs are available have been reported [9,14–22]. Our aim in this article was to contribute to the understanding of the clinical and EEG dimensions of PS by reporting another six cases with ictal EEG documentation. 2. Methods These six cases were recently described in a retrospective clinical/ EEG and neuropsychological study of 93 children with PS [7]. Patients were included if they had had one or more unequivocal autonomic epileptic seizures with or without ictal vomiting. We did not consider EEG abnormalities and localization as essential inclusion or exclusion criteria to comply with current evidence that PS is mainly a multifocal epileptic syndrome with significant EEG variability. We excluded patients with symptomatic epilepsy and patients with nonepileptic paroxysmal autonomic manifestations only. Epilepsy & Behavior 19 (2010) 383–393 ⁎ Corresponding author. Division of Neurology, Bambino Gesù Children's Hospital IRCCS, Piazza S. Onofrio, 4 00165 Rome, Italy. Fax: +39 06 69592463. E-mail address: nicola.specchio@opbg.net (N. Specchio). 1525-5050/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.yebeh.2010.06.046 Contents lists available at ScienceDirect Epilepsy & Behavior journal homepage: www.elsevier.com/locate/yebeh