Study on the Early‐Onset Variant of Benign Childhood Epilepsy with Occipital Paroxysms Otherwise Described as Early‐Onset Benign Occipital Seizure Susceptibility Syndrome

Abstract

Summary: Purpose: We studied the early-onset variant of benign childhood epilepsy with occipital paroxysms (EVBCEOP) proposed by Panayiotopoulos, to confirm whether his five criteria are sufficient to delineate EVBCEOP as a new epileptic syndrome, as well as to predict a good outcome prospectively at the time of the first examination.Subjects: The subjects were 649 children with localizationrelated epilepsies (LREs) observed in our hospital for >4 years.Methods: We applied the International Classification of Epilepsies and Epileptic Syndromes to the 649 patients and identified patients who had EVBCEOP from among those with nonspecific idiopathic LRE. The inclusion criteria were to satisfy all five criteria and all but one criterion (i.e., either ictal vomiting or occipital EEG paroxysms). We were blind as to the outcomes and selected patients who satisfied the following teria, 22 who exhibited all but occipital EEG foci, and 2 1 who exhibited all but ictal vomiting. The incidence of status convulsivus was higher in those with ictal vomiting than in those without ictal vomiting (p < 0.05). Interictal EEG performed every 6 months showed shifting and multiplication of EEG foci in 42 and 52% of all subjects, respectively. We identified 57 patients, 42 (74%) of whom were in remission by age 12 years. The number of patients who experienced remission did not differ significantly between those with (76%, n = 25) and without (72%, n = 32) ictal vomiting (p > 0.05). Conclusions:Nosologically, EVBCEOP appears to constitute the earliest form of idiopathic epileptic syndrome different from classic BCEOP. However, its clinical spectrum, ranging from the absence of ictal vomiting to a combination of extraoccipital and multifocal EEG foci, is broad, such that further three of the five criteria at the time of the first examination, (a) normal development before the onset, (b) epilepsy onset age between 2 and 8 years, and (c) occipital EEG foci. We attempted to determine whether the outcome can be predicted prospectively, and whether the presence or absence of ictal vomiting affects the prognosis.Results: We identified 19 patients who satisfied all five criprospective study is expected to reveal the exact prerequisite criteria for determining the border of this epileptic syndrome and for clarifying the clinical spectrum within this syndrome. Key Words: Early-onset variant of benign childhood epilepsy with occipital paroxysms-Migrating EEG foci-Ictal vomiting-Idiopathic localization-related epilepsy-Occipital EEG foci.Panayiotopoulos proposed classifying benign childhood epilepsy with occipital paroxysms (BCEOP) into early-onset and late-onset types based on the results of his 15-year prospective follow-up study of children with occipital paroxysms (1-8). In his proposal, the latter is classified as BCEOP (9), which Gastaut originally defined, and visual seizures are the predominant seizure type. The early-onset type has been proposed to be a new idiopathic epileptic syndrome, characterized by the fol-~ ~~~

A study on spike focus dependence of high‐frequency activity in idiopathic focal epilepsy in childhood

“…It was later clarified that the foci are multiple in both ictal and interictal recordings 22. It was suggested that there is no actual focus in a certain region and that spikes only indicate extensive cortical excitability 23.…”

“…In other words, even when spikes are multifocal, the occipital lobes may play a crucial role in PS. As for why PS is characterized by autonomic seizures even though they originate from the occipital lobes, it is speculated that autonomic symptoms, including vomiting, may be attributed to the involvement of the limbic and insular regions, to which occipital seizures originating below the calcarine fissure may spread 22. High sensitivity of the autonomic center unique to young children may be related to these symptoms.…”

Early Onset Benign Childhood Occipital Epilepsy (Panayiotopoulos Syndrome). A Case Report

“…Унилатеральная девиация глаз -такой же распро-страненный симптом, как рвота, он встречается прибли-зительно у 80 % пациентов [5,15,24,26,27]. Девиация глаз может быть кратковременной или длительной, она часто сопровождается девиацией головы и нарушением сознания.…”

“…Эпилептический статус (обычно не сопровождаю-щийся судорогами) длится более 30 мин и развивается приблизительно в 30 % случаев [5,15,24,27,41]. Эта форма эпилептического статуса, подобная энцефало-патии, может прогрессировать до гемиклонических судорог.…”

“…Иктальные изменения при синдроме Панайотопу-лоса характеризуются ритмичными мономорфными замедлениями тета-и дельта-активности, которые замет-но отличаются от эпизодической быстрой активности «зрительных» приступов при типе Гасто и начинаются с задних [1,9,24,40] или фронтальных областей [8,24]. Поскольку большинство пациентов с синдромом Пана-йотопулоса -дети в возрасте от 3 до 6 лет, во многих слу-чаях ЭЭГ-исследование включало сон, что и позволило выявить характерные изменения во время сна.…”