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Panayiotopoulos syndrome is a type of benign childhood epilepsy syndrome characterized by episodic autonomic and mainly emetic symptoms. It predominantly affects children of 3-6 years of age (13% of those with one or more non-febrile seizures). EEG shows great variability, with occipital, extra-occipital spikes or brief generalised discharges alone or in combination; it may also be consistently normal. Despite the high prevalence of autonomic status epilepticus, the prognosis of Panayiotopoulos syndrome is usually excellent and most do not require anti epileptic medications. Remission usually occurs within 1-2 years from onset. One third have a single seizure but 5-10% may have more than 10 seizures or a more prolonged course. We present a 5 year old boy with this condition, who presented with episodes of severe vomiting, deviation of eyes, focal seizure, altered sensorium, characteristic EEG in the form of multiple occipital spikes and normal neuroimaging studies.J. Natl Inst. Neurosci Bangladesh 2015;1(1):27-30

Panayiotopoulos syndrome (PS) is a relatively frequent and benign epileptic syndrome seen in children in the age group of 3-6 years and is characterised by predominantly autonomic symptoms and/or simple motor focal seizures followed or not by impairment of consciousness. Although multifocal spikes with high amplitude sharp-slow wave complexes at various locations can be present in the EEG, interictal electroencephalogram (EEG) in children with this particular type of epilepsy characteristically shows occipital spikes. This syndrome has known to be a masquerader and can imitate gastroenteritis, encephalitis, syncope, migraine, sleep disorders or metabolic diseases. In the absence of thorough knowledge of types of benign epilepsy syndromes and their various clinical presentations, epilepsy such as PS can be easily missed. The peculiar aspects of this type of epilepsy in children should be known not only by paediatricians but also by general doctors because a correct diagnosis would avoid aggressive interventions and concerns on account of its benign outcome. In this case study, we report a case of PS in a 3 year old child.

Introduction. Paroxismal events can resemble epileptic seizures, however, some epileptic seizures, especially benign occipital childhood epilepsies can imitate migraine, cycling vomiting or encephalitis. Objective. The aim of this study was evaluation of clinical and electroencephalographic (EEG) features and outcome in children with benign occipital childhood epilepsies. Methods. Investigation included 18 patients with benign occipital childhood epilepsies hospitalized in the period from 2007 to 2010. The diagnosis was based on clinical and EEG characteristics of seizures, while treatment included acute therapy for seizures and chronic antiepileptic drugs. Prognosis was analyzed in terms of neurological outcome and seizure recurrence rate. Results. Benign occipital childhood epilepsy with early onset was diagnosed in 15 children. Vegetative symptoms, mostly ictal vomiting (13), eye deviation and loss of consciousness (13) dominated in the clinical presentation. The most frequent EEG findings showed occipital epileptic discharges. Benign occipital childhood epilepsy with late onset was diagnosed in three cases. Seizures were manifested by visual hallucinations, headache and secondary generalized convulsions. All three patients were administered chronic antiepileptic drugs and had good outcome. Conclusion. In our patients, clinical manifestations of benign occipital epilepsies had some similarities with clinical features of migraine and encephalitis. It could explain misdiagnosis in some of them. Knowledge about main features and differences between each of these disorders is crucial for making appropriate diagnosis.

This study intended to investigate the clinical and electroencephalographic benign occipital epilepsy of childhood (BOEC) characteristics in a population sample of patients from two tertiary Brazilian hospitals. We analyzed retrospectively 4912 electroencephalograms (EEGs) records, and the included patients were submitted to a new clinical and EEG evaluation. Were included 12 (0.92%) patients; 4 (33.3%) with criteria for early BOEC; 6 (50%) for late form and 2 (16.7%) with superimposed early and late onset forms. After new investigation, 2 (16.7%) had normal EEG; 4 (33.3%) had paroxysms over the occipital region; 3 (25%) over the temporal posterior regions and 3 (25%) over the posterior regions. Sharp waves were the predominant change, occurring in 8 (66.6%); spike and slow wave complexes in 1 (8.3%) and sharp and slow wave complexes in 1 (8.3%). Vomiting, headache and visual hallucinations were the most common ictal manifestations, presented in 100% of patients with superimposed forms. Vomiting were absent in the late form and headache was present in all forms of BOEC. Este estudo teve como objetivo investigar as características clínicas e eletrencefalográficas da epilepsia occipital benigna da infância (EOBI) em uma amostra populacional de pacientes de dois hospitais terciários brasileiros. Foram analisados retrospectivamente 4912 registros de eletrencefalograma (EEG). Os pacientes incluídos foram submetidos a nova avaliação clínica e eletrencefalográfica. Foram incluídos 12 (0,92%) pacientes; 4 (33,3%), com critérios para EOBI de início precoce; 6 (50%) para a forma tardia e 2 (16,7%), com superimposição de formas de início precoce e tardio. Após nova investigação, 2 (16,7%) apresentaram EEG normal; 4 (33,3%) paroxismos sobre a região occipital; 3 (25%) sobre a região temporal posterior e 3 (25%) sobre as regiões posteriores. Ondas agudas foram a alteração predominante, ocorrendo em 8 (66,6%); complexos espícula e onda lenta em 1 (8,3%) e complexos onda aguda e onda lenta em 1 (8,3%). Vômitos, cefaléia e alucinações visuais foram as manifestações ictais mais comuns, estando presentes em 100% dos pacientes com formas superimpostas de EOBI. Vômitos não foram relatados na forma tardia e cefaléia esteve presente em todas as formas de EOBI.

The last proposal for a classification of epileptic seizures and syndromes of the International League Against Epilepsy (ILAE) includes recognition of several groups of epileptic syndromes and among them one of idiopathicfocal epilepsies of infancy and childhood, and another offamilial (autosomal dominant) focal epilepsies. The syndromes here described belong to these two groups and are: benign familial infantile seizures, benign infantile seizures (nonfamilial), benign childhood epilepsy with centrotemporal spikes (BCECTS), idiopathic occipital epilepsy of childhood Gastaut type (IOEC) and benign occipital epilepsy of childhood Panayiotopoulos type (BOEC). Atypical evolutions of the cases with BCECTS, IOEC and BOEC diagnosed and followed in our group are described, showing that these conditions are not always so benign. Finally, the so called benign focal convulsions of adolescence are considered, even when they were not included in ILAE's last proposal.

Reply from the Author: The letter by Panayiotopoulos et al. raises an important issue that the tight format of a Brief Communication did not permit in the original publication. First, I wish to reassure the correspondents that the possibility of benign childhood occipital seizures(BCOS), also variously known as benign occipital epilepsy(BOE),11 benign epilepsy of childhood with occipital paroxysms,12 and epilepsy with occipital spike-wave complexes suppressed by eye opening,13 was seriously considered before settling on the diagnosis of acephalgic migraines. I …

Benign occipital epilepsy is a newly recognized form of partial epilepsy in children. It is characterized by motor seizures preceded in some cases by visual symptoms and a relatively benign course. We present three siblings with this condition and a fourth with the EEG abnormalities. A family study including 25 relatives demonstrated a typical EEG abnormality in 26% of relatives. These EEG changes were more evident in younger members. These findings suggest an autosomal dominant pattern for the EEG abnormalities with age-dependent expression and variable penetrance of the seizure disorder.