A Study of 43 Patients with Panayiotopoulos Syndrome, a Common and Benign Childhood Seizure Susceptibility

Abstract

Summary:Purpose: To determine prevalence, clinical, EEG features, and prognosis of Panayiotopoulos syndrome and to examine the proposition that clinical manifestations are more important than EEG findings.Methods: We analyzed retrospectively the clinical and EEG records of 1,340 children with one or more focal seizures seen in the last 18 years, supplemented with a prospective study from 1998. Panayiotopoulos syndrome was defined by clinical criteria, mainly ictal emesis, irrespective of EEG findings.Results: We analyzed 43 of 90 patients with Panayiotopoulos syndrome who were seizure free >2 years. Girls predominated. Mean age at first seizure was 5 years. Seizures consisted mainly of autonomic manifestations; ictal emesis was often the first symptom, culminating in vomiting in 86%. Of nonautonomic manifestations, lateral eye deviation was the most common; visual symptoms were exceptional. Impairment of consciousness ensued in all seizures, half of which ended with hemi or generalized convulsions. Nearly 46.5% of cases had at least one seizure >30 min, constituting autonomic status epilepticus. Seizures during sleep (84%) were more common than those in wakefulness. EEG showed occipital spikes in 29 patients. Of the other 14 cases, five had extraoccipital abnormalities or brief generalized discharges, and nine had normal awake and sleep EEG. Prognosis was excellent. All 43 children have been free of seizures for Ն2 years, 53% having a single seizure, and 47%, an average two to three seizures.Conclusions: Panayiotopoulos syndrome is common and needs wider recognition. EEG shows occipital or extraoccipital abnormalities, is normal in one third of patients, and does not determine clinical manifestations or prognosis, which is excellent despite the high prevalence of lengthy seizures.

Autonomic seizures and autonomic status epilepticus in early onset benign childhood occipital epilepsy (Panayiotopoulos syndrome)

“…In a significant way, differences were not observed between the children presenting occipital or centro-temporal focal EA or normal EEG with respect to the total number of seizures, the duration or the type of seizure. These findings are consistent with the initial description of Panayiotopoulos and that of other authors 1,2,5,9 .…”

“…In 29.6% of the cases, blockage or attenuation of the occipital EA was caused by opening the eyes, a lower percentage than the 80% and 100% of cases reported respectively by Caraballo et al 9 and Verroti et al 13 , but similar to that reported by other authors 5,14 .…”

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

“…В одном ис-следовании сообщалось о наличии связи с хромосо-мой 15 при идиопатической детской эпилепсии с центротемпоральными спайками [22], хотя в дру-гом исследовании этот локус найден не был [36]. О наличии 1 сиблинга с синдромом Панайотопулоса сообщалось в исследовании одной группы [15], и 2 пары больных сиблингов были отмечены в ка-ждом из двух других исследований [5,21]. В 1987 г. было опубликовано сообщение о наличии у 3 сиблингов затылочной эпилепсии типа Гасто, хотя описанные симптомы (в том числе дебют в возрасте 4-5 лет) полностью соответствовали синдрому Па-найотопулоса [20].…”

“…Так, иктальная рвота, в целом нетипичная для эпилепсии, при син-дроме Панайотопулоса встречается приблизительно в 80 % случаев [5,7,15,21]. При ночных приступах рвота обычно является первым и очевидным призна-ком [7].…”

Origin of frontal lobe spikes in the early onset benign occipital lobe epilepsy (Panayiotopoulos syndrome)

“…The early onset benign idiopathic occipital lobe epilepsy, presently reported as Panayiotopoulos syndrome (PS), has generated a lot of interest in recent years (Lada et al, 2003;Carabalo et al, 2007;Koutroumanidis, 2007). Despite the relatively easy recognition of clinical symptoms (Ferrie et al, 2006), the highly variable characteristics of the interictal EEG activity have prevented the determination of a consistent localization for the epileptic focus (Panayiotopoulos, 2002).…”