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Panayiotopoulos syndrome is a childhood epileptic illness. The first attack occurs between 1st and 14th year of age, usually at the age of 3–6 years (80% of patients). The characteristic features of this syndrome are seizures with autonomic symptoms or the presence of autonomic epileptic status. The most helpful diagnostic is EEG with abnormal spikes, often with occipital predominance. We presented the case of an 8-year-old, previously healthy boy, who was hospitalized because of indicating initially poisoning or encephalitis. Finally, we diagnosed Panayiotopoulos syndrome, which was by autonomic epileptic status.

Purpose: To clarify the clinical implications of the preceding positive spikes (PPSs) observed primarily in rolandic spikes, we analyzed PPSs in the rolandic and occipital spikes observed in the electroencephalograms (EEGs) of patients with two types of benign partial epilepsies (benign childhood epilepsy with centro-temporal spikes [BECT] and Panayiotopoulos syndrome [PS]) and febrile seizures (FS). Subjects and methods: We identified patients from our outpatient EEG database that were seen between 2006 and 2008 that had BECT, PS, and FS with rolandic or occipital spikes. We generated an averaged spike for each patient from the rolandic and occipital spikes that were detected using an automatic spike detection and clustering system. We compared the presence rate of the averaged spikes with the PPS among the three groups (BECT vs. PS vs. FS) using sequential mapping. Results: We identified 25 BECT, 18 PS, and 15 FS patients with rolandic spikes. Fifteen BECT and nine PS patients exhibited a PPS in their averaged rolandic spikes, whereas only four FS patients did. Three of these four FS patients later developed afebrile seizures, and one of them was diagnosed as having PS. We analyzed eight PS and six FS patients with occipital spikes. Five PS patients exhibited a PPS in their averaged occipital spikes, whereas only one FS patient did. This FS patient later developed prolonged autonomic febrile seizures. Conclusion: PPSs are observed not only in rolandic spikes associated with BECT that is related strictly to sylvian seizures, but also in rolandic and occipital spikes associated with PS. Although PPSs are rare in such spikes observed in FS, patients with FS and PPSs may have an increased risk of developing afebrile seizures or prolonged autonomic febrile seizures. Further studies are warranted to determine the diagnostic utility of PPSs as a marker of the future development of epilepsy when they are observed in FS patients.

医学部小児科学教室 大澤眞木子教授退任記念特別号

Introduction Amongst autosomal dominant genetic epilepsy with febrile seizures plus (GEFS+) families, SCN1A variants are the most common genetic cause. Initially regarded as a generalized form of epilepsy, the GEFS+ spectrum is now known to include some focal epilepsies, but it is generally not conceptualized as extending to the self-limited focal epilepsies of childhood, such as Panayiotopoulos syndrome. There are, however, three reports of SCN1A variants in Panayiotopoulos syndrome. We describe the variable clinical phenotypes that include the self-limited focal epilepsies of childhood, present in a large GEFS+ family, segregating a heterozygous SCN1A missense variant. Material and methods Electro-clinical details on all putatively affected family members were sought and blood samples were taken for genetic analysis. Two individuals were chosen for SCN1A testing. All 26 exons and exon–intron junctions were amplified, sequenced and analyzed. This was followed by pedigree segregation analysis of the variant identified. Results A pathogenic heterozygous SCN1A (c.2624C>A; p.Thr875Lys) variant was identified. Sixteen of the 18 variant positive family members were affected (88% penetrance): 8 with febrile seizures, 2 febrile seizures plus, 1 unclassified seizures and 5 with self-limited focal epilepsy of childhood. Of these, one was diagnosed with atypical childhood epilepsy with centrotemporal spikes and four with Panayiotopoulos syndrome. Discussion By characterizing the heterogeneous clinical phenotypes in a large, SCN1A mutation positive GEFS+ family, we conclude that the GEFS+ spectrum can extend to the self-limited focal epilepsies of childhood, including Panayiotopoulos syndrome, and in turn highlight the complex genotype-phenotype correlations associated with SCN1A -related epilepsies.

Purpose The aim was to identify the demographic, electroencephalographic findings, clinical features, especially ictal semiological symptoms of patients diagnosed with Panayiotopoulos Syndrome. Method The semiologic and EEG findings of 42 patients were reviewed. Statistical analysis was used to determine the frequencies of semiologic features and to define the symptom clusters. Results The most common clinical features were nausea (54.8%), vomiting (50.0%), pallor (50.0%), deviation of head and eyes (45.2%), generalized seizures (35.7%), ictal syncope (33.3%) and stomach ache (26.2%). Visual manifestations which were observed in 38.9% of the patients were amaurosis, flashes of colourful lights, flashes of brightful lights, visual hallucinations and blurring of vision. Autonomic status epilepticus was observed in 21.4% of the patients. Cluster analysis of 13 most common symptoms showed a tendency toward 3 clusters. Pallor constituted a subgroup on its own whereas nausea, vomiting, deviation of head and eyes tended to occur together in the second subgroup and the third subgroup included semiologic features some of which were atypical for PS. Conclusions A broad spectrum of typical and atypical clinical features might be observed in patients with PS. Some of the semiologic features of PS might have tendency to occur together. Overlapping of typical autonomic features with some of the atypical features might suggest that PS, RE and ICOE-G were the constituents of the same neurobiological spectrum. Our detailed data might contribute to the awareness of a wide range of variabilites in the electroclinical features of PS.

Introduction: Panayiotopoulos syndrome is the second commonest idiopathic focal childhood epilepsy, with a peak of age at onset 4-5 years. Its incidence is usually underestimated, because of the typical unusual clinical manifestations of the seizures with predominant autonomic changes.Case report: We describe a classical case of Panayiotopoulos syndrome, with recurrent seizures, but good response to antiepileptic drug treatment and remission after a few years.Conclusion: The importance of the clinical suspicion of this epileptic syndrome and the usefulness of an electroencephalographic recording including a period of sleep is underlined. In most diagnosed cases prognosis is good, although therapy is usually necessary for a variable period of time., Introdução: O Síndrome de Panayiotopoulos é a segunda epilepsia focal idiopática infantil mais comum, com um pico de incidência aos 4-5 anos. A sua incidência é subestimada devido às manifestações clínicas atípicas, caracterizando-se por crises com predomínio de sintomas autonómicos.Caso clínico: Descreve-se um caso clássico de Síndrome de Panayiotopoulos, com crises recorrentes, com boa resposta à terapêutica antiepiléptica e remissão após alguns anos.Conclusão: Salienta-se a importância da suspeição clínica deste síndrome epiléptico e a utilidade do registo electroencefalográfico que inclua um período de sono. Na maioria dos casos o prognóstico é bom, apesar de ser necessário manter o tratamento por um período de tempo variável.

SummaryObjectives To provide insight into the pathophysiology of idiopathic childhood occipital epilepsies (ICOEs), by mapping the contribution of retinotopic visual areas to the generation and sustainment of epileptic activity. Methods Thirteen patients affected by ICOEs (mean age = 10.9 years) underwent a video electroencephalography–functional magnetic resonance imaging (EEG-fMRI) study. A flexible-related fMRI analysis was applied to estimate the shape of the blood oxygen level–dependent (BOLD) response in each patient. Second-level analysis was performed using the interictal EEG discharge (IED)–specific response shape for the ICOE group. The resulting fMRI t-maps were warped to the Population-Average, Landmark- and Surface-based (PALS)-B12 atlas in Caret. For localization purposes, functional results were plotted and compared against 19 retinotopic areas for each hemisphere. A correlation analysis was performed between the hemodynamic maps and electroclinical variables. Results The shape of the group-averaged hemodynamic response in ICOE patients showed an earlier time-to-peak and a more pronounced undershoot than the canonical hemodynamic response function (HRF). The random-effect analysis showed positive hemodynamic changes in the bilateral temporooccipital network. With regard to the retinotopic subdivision of the visual cortex, the primary visual area was consistently spared. Conversely, an extensive involvement of the occipitotemporal cortex, including the fusiform gyrus, and the occipitoparietal areas was observed. Moreover, a linear relationship was detected between the occipital spike-density and BOLD increases at the postcentral gyrus and temporooccipital cortex. Significance Our data indicate that both the ventral and dorsal visual pathways are involved in spike generation in ICOEs, to extents that vary between patients, and reinforce the concept of benign childhood seizure susceptibility syndrome as a substrate for ICOEs. Finally, these results underscore the need for appropriate neuropsychological testing in these children, aimed at revealing selective impairments in functions subserved by both visual pathways.

The term idiopathic focal epilepsies of childhood (IFE) is not formally recognised by the ILAE in its 2010 revision (Berg et al., 2010), nor are its members and boundaries precisely delineated. The IFEs are amongst the most commonly encountered epilepsy syndromes affecting children. They are fascinating disorders that hold many "treats" for both clinicians and researchers. For example, the IFEs pose many of the most interesting questions central to epileptology: how are functional brain networks involved in the manifestation of epilepsy? What are the shared mechanisms of comorbidity between epilepsy and neurodevelopmental disorders? How do focal EEG discharges impact cognitive functioning? What explains the age-related expression of these syndromes? Why are EEG discharges and seizures so tightly locked to slow-wave sleep? In the last few decades, the clinical symptomatology and the respective courses of many IFEs have been described, although they are still not widely appreciated beyond the specialist community. Most neurologists would recognise the core syndromes of IFE to comprise: benign epilepsy of childhood with centro-temporal spikes or Rolandic epilepsy (BECTS/RE); Panayiotopoulos syndrome; and the idiopathic occipital epilepsies (Gastaut and photosensitive types). The Landau-Kleffner syndrome and the related (idiopathic) epilepsy with continuous spikes and waves in sleep (CSWS or ESES) are also often included, both as a consequence of the shared morphology of the interictal discharges and their potential evolution from core syndromes, for example, CSWS from BECTS. Atypical benign focal epilepsy of childhood also has shared electro-clinical features warranting inclusion. In addition, a number of less well-defined syndromes of IFE have been proposed, including benign childhood seizures with affective symptoms, benign childhood epilepsy with parietal spikes, benign childhood seizures with frontal or midline spikes, and benign focal seizures of adolescence. The term "benign" is often used in connection with the IFEs and is increasingly being challenged. Certainly most of these disorders are not associated with the devastating cognitive and behavioural problems seen with early childhood epileptic encephalopathies, such as West or Dravet syndromes. However, it is clear that specific, and sometimes persistent, neuropsychological deficits in attention, language and literacy accompany many of the IFEs that, when multiplied by the large numbers affected, make up a significant public health problem. Understanding the nature, distribution, evolution, risk and management of these is an important area of current research. A corollary to such questions regarding comorbidities is the role of focal interictal spikes and their enduring impact on cognitive functioning. What explains the paradox that epilepsies characterised by abundant interictal epileptiform abnormalities are often associated with very few clinical seizures? This is an exciting area in both clinical and experimental arenas and will eventually have important implications for clinical management of the whole child, taking into account not just seizures, but also adaptive functioning and quality of life. For several decades, we have accepted an evidence-free approach to using or not using antiepileptic drugs in IFEs. There is huge international variation and only a handful of studies examining neurocognitive outcomes. Clearly, this is a situation ready for an overhaul in practice. Fundamental to understanding treatment is knowledge of aetiology. In recent years, there have been several significant discoveries in IFEs from studies of copy number variation, exome sequencing, and linkage that prompt reconsideration of the "unknown cause" classification and strongly suggest a genetic aetiology. The IFE are strongly age-related, both with regards to age of seizure onset and remission. Does this time window solely relate to a similar age-related gene expression, or are there epigenetic factors involved that might also explain low observed twin concordance? The genetic (and epigenetic) models for different IFEs, their comorbidities, and their similarities to other neurodevelopmental disorders deserve investigation in the coming years. In so doing, we will probably learn much about normal brain functioning. This is because these disorders, perhaps more than any other human brain disease, are disorders of functional brain systems (even though these functional networks may not yet be fully defined). In June 2012, an international group of clinical and basic science researchers met in London under the auspices of the Waterloo Foundation to discuss and debate these issues in relation to IFEs. This Waterloo Foundation Symposium on the Idiopathic Focal Epilepsies: Phenotype to Genotype witnessed presentations that explored the clinical phenomenology, phenotypes and endophenotypes, and genetic approaches to investigation of these disorders. In parallel, the impact of these epilepsies on children and their families was reviewed. The papers in this supplement are based upon these presentations. They represent an updated state-of-the-art thinking on the topics explored. The symposium led to the formation of international working groups under the umbrella of "Luke's Idiopathic Focal Epilepsy Project" to investigate various aspects of the idiopathic focal epilepsies including: semiology and classification, genetics, cognition, sleep, high-frequency oscillations, and parental resources (see www.childhood-epilepsy.org). The next sponsored international workshop, in June 2014, was on randomised controlled trials in IFEs and overnight learning outcome measures.

Gowers gave migraine a place in the borderland of epilepsy, justified by many relations and the fact that distinction of these two diseases is difficult. Gowers based his ideas on clinical histories from his patients, and he concluded that in extremely rare instances one may develop while the other goes on. In modern times, patient testimonies of the problem of differentiating migraine from epilepsy are given in health-chat Websites. It shows clearly that distinction between the two conditions is still problematic and that migralepsy is either nonexisting or extremely rare as Gowers noticed.

Benign epilepsy with centrotemporal spikes, early-onset childhood occipital epilepsy (Panayiotopoulos syndrome [PS]) and late-onset childhood occipital epilepsy (Gastaut type [LOCE-G]) are the principal pediatric focal epilepsy syndromes. They share major common characteristics: the appearance and resolution of electroclinical features are age related, there is a strong genetic predisposition, the clinical course is often mild with infrequent and easy to control seizures, interictal epileptiform activity is disproportionately abundant when compared with the clinical correlate, and tends to potentiate and generalize during sleep. In this review, we outline the relevant pathophysiology underlying this electroclinical spectrum. Then, the initial description of individual syndromes is followed by a summary of overlapping features and intermediate presentations that question the boundaries between these entities and provide the basis for the concept of a childhood seizure susceptibility syndrome. Additionally, we outline the main features of the related epileptic encephalopathies. An outlook on potential future lines of research completes this review.

The differential diagnosis of an episode of transient loss of consciousness can be sometimes very tricking, in particular when symptoms peculiar of syncope are mixed with focal neurological symptoms. We report the case of a 54-year-old woman who suddenly claimed, during a polygraphic recording (electroencephalography/electrocardiogram), a feeling of fear and tachycardia followed by loss of consciousness and then a tonic posturing of the left limbs. Polygraphic recording showed a critical electroencephalographic pattern starting from left temporo-zygomatic channels followed after few seconds by a sudden slowing of cortical background activity associated with an episode of asystole, as witnessed simultaneously by electrocardiogram. Muscular activity covered electroencephalographic activity of following minutes. This case provides an opportunity to highlight the existence of rare conditions such as ictal arrhythmias which should be considered in the differential diagnosis of episodes of transient loss of consciousness in particular when dysautonomic and neurological symptoms are intermingled. Autonomic symptoms (vomiting, tachycardia, cyanosis, bradycardia and asystole) may be also more frequent in idiopathic (more rarely symptomatic) epilepsies of childhood (Panayiotopoulos syndrome).

A 4-year-old girl with Panayiotopoulos syndrome presented with a history of 4 prolonged autonomic seizures. The clinical features of her seizures included, in order of occurrence, blank staring, pallor, vomiting, hemi-clonic movement on the right side, and unresponsiveness. A brain magnetic resonance imaging (MRI) showed a slightly high T(2) signal in the left hippocampus. Interictal electoencephalogram revealed spikes in the occipital area of the left hemisphere. We analyzed the current-source distribution of the spikes to examine the relationship between the current source and the high T(2) signal. The current source of the occipital spikes was not only distributed in the occipital area of both cerebral hemispheres, but also extended to the posterior temporal area of the left hemisphere. These findings suggest that the left temporal lobe may be one of the hyperexcitable areas and form part of the epileptogenic area in this patient. We hypothesized that the high T(2) signal in the left hippocampus of our patient may not have been an incidental lesion, but instead may be related to the underlying electroclinical diagnosis of Panayiotopoulos syndrome, and particularly seizure. This notion is important because an abnormal T(2) signal in the hippocampus may represent an acute stage of hippocampal injury, although there is no previous report of hippocampal pathology in Panayiotopoulos syndrome. Therefore, long-term observation and serial follow-up MRIs may be needed to confirm the clinical significance of the T(2) signal change in the hippocampus of this patient.

Summary Purpose: We explored high-frequency oscillations (HFOs) in scalp sleep electroencephalography (EEG) studies of patients with idiopathic partial epilepsy (IPE) of childhood in order to obtain a better understanding of the pathologic mechanisms underlying IPE. Methods: The subjects were 45 patients, including 32 with benign childhood epilepsy with centrotemporal spikes (BCECTS) and 13 with Panayiotopoulos syndrome (PS). A total of 136 EEG records were investigated through temporal expansion and filtering of traces and time-frequency spectral analysis. Key Findings: HFOs with frequency of 93.8–152.3 Hz (mean 126.2 ± 13.6 Hz) in the band of ripples were detected in association with spikes in 97 records (71.3%). Time from last seizure to the EEG recording was significantly shorter in those with spike-related HFOs than in the EEG recordings with spikes without HFOs (p = 0.006). Although time from last seizure reflects age, age at the time of recording was not significantly different between EEG studies with and without HFOs. Peak-power values of the high-frequency spots in time-frequency spectra were significantly negatively correlated with time from last seizure (R2 = 0.122, p

Benign nocturnal alternating hemiplegia (BNAH) of childhood is distinct from the classic form of malignant alternating hemiplegia of childhood [1]. It is characterized by hemiplegic attacks occurring exclusively during sleep [2]. It can be misdiagnosed as migraine, nocturnal frontal lobe epilepsy, benign rolandic epilepsy, Panayiotopoulos syndrome, or sleep-related movement disorder [1-4]. Only nine patients have been described to date, with typically, a normal development [1,5-7]. In order to insist about the benignity of the affection, we report two cases: a new three-year-old boy suffering from BNAH and a patient already published to show positive evolution at fourteen years of age. BNAH is a rare disorder but may be underdiagnosed. Making an early diagnosis can help to describe to the parents the good prognosis without treatment.

In this study, we describe three patients who each had two different forms of idiopathic focal epilepsiy. Two of these patients had electroclinical features compatible with Panayiotopoulos syndrome and benign childhood epilepsy with centro-temporal spikes (BCECTS), one of whom developed a particular electroclinical picture of atypical benign focal epilepsy and the other an atypical evolution characterized by verbal auditory agnosia and aphasia. The third patient had clinical and electroencephalographic features of BCECTS and of idiopathic childhood occipital epilepsy (Gastaut type) which evolved into electroclinical features of continuous spikes and waves during slow sleep (CSWS). All three patients presented with two focal idiopathic epilepsies with a particular evolution associated with CSWS, supporting the concept of benign childhood seizure susceptibility syndrome as described by Panayiotopoulos (1993).

The diagnosis of benign epilepsy syndrome should meet the following criteria: age-related and self-limited; good response to medication; and no obvious neurological sequelae after seizure. However, the current concept of benign epilepsy syndrome has been challenged because of the advancements in genetic studies, neuroimaging, and molecular techniques. Many studies have revealed that the prevalence of behavioral problems and learning difficulties as well as subtle cognitive deficits is higher among patients with benign epilepsy, compared with the normal population. Here, we review updated results of these studies to show the latest and broad comprehensive knowledge of benign epilepsy in children.

Epileptic spike foci in children can be fixed in specific brain regions or migrate over time. We analyzed 969 encephalograms from 463 epileptic children to determine outcome differences between those with fixed foci (FF) and those with migrated foci (MF) on at least three encephalograms over 3 years. All epileptic spike foci were classified as frontal, temporal, central, parietal, or occipital. Migration directions were divided into anterior, posterior, lateral, and unclassified. Seventy-nine cases met the inclusion criteria: 24 (30%) FF and 55 (70%) MF. More patients in the FF than in the MF group required multiple antiepileptic drugs (P = 0.004), and had abnormal image findings (P = 0.014), mental retardation (P = 0.035), and worse seizure control (P = 0.047). Seizure frequency (P = 0.007; correlation coefficient = 0.56) and the number of prescribed drugs (P = 0.047; correlation coefficient = 0.372) were more significant in the FF group than in the MF group. When we compared only whether the cases were symptomatic or cryptogenic, we found the same outcome trends. In comparisons of only idiopathic epileptic patients, the FF group had non-significantly different outcomes compared with those in the MF group. When we compared 16 cases of benign childhood epilepsy with centrotemporal spikes (BECTs) and 8 Panayiotopoulos syndrome (PS), the cases with BECTs had more FF (38% vs. 0%, P = 0.03). We conclude that outcomes may not correlate as well in FF cases as they do in MF cases. Idiopathic epilepsy warrants more study.

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.

Summary Purpose: To explore the clinical, electroencephalography (EEG), neuropsychological features, and prognosis of Panayiotopoulos syndrome (PS). Methods: Of 1,794 children aged between 1 and 14 years referred for the first afebrile focal seizure, between January 1992 and December 2004, 93 (5.2%) had PS according to clinical criteria. Results: Age at onset ranged from 1.1 to 8.6 years, and was earlier in children with more than one seizure. Autonomic seizures followed a stereotypical onset and progression. Emesis, pallor, or flushing was almost always among the first symptoms that usually culminated in vomiting (77.4% of patients). More than half (55%) of seizures were longer than 30 min but these did not appear to affect remission and number of seizures. Interictal EEG showed great variability, with 79.5% of patients showing spikes of variable localizations and evolution over time; 16.1% had background abnormalities only, and 5.4% had consistently normal EEG studies. Onsets in five ictal EEGs were posterior or anterior—left or right. On neuropsychological testing, IQ and subtests of Wechsler Intelligence Scale for Children–Revised (WISC-R) were within normal limits, although some minor statistically significant differences were found in arithmetic, comprehension, and picture arrangement in comparison with controls. Cumulative probability of recurrence was 57.6%, 45.6%, 35.1%, and 11.7% at 6, 12, 24, and 36 months, respectively, after the first seizure. Thirty-four (58.6%) of 59 patients treated with antiepileptic drugs continued having seizures before ultimate remission. Discussion: PS is a uniform childhood susceptibility to autonomic seizures that is related to early age of development and with excellent prognosis with regard to seizure remission and neuropsychological development.

The International League Against Epilepsy (ILAE) Commission on Classification and Terminology has revised concepts, terminology, and approaches for classifying seizures and forms of epilepsy. Generalized and focal are redefined for seizures as occurring in and rapidly engaging bilaterally distributed networks (generalized) and within networks limited to one hemisphere and either discretely localized or more widely distributed (focal). Classification of generalized seizures is simplified. No natural classification for focal seizures exists; focal seizures should be described according to their manifestations (e. g., dyscognitive, focal motor). The concepts of generalized and focal do not apply to electroclinical syndromes. Genetic, structural-metabolic, and unknown represent modified concepts to replace idiopathic, symptomatic, and cryptogenic. Not all epilepsies are recognized as electroclinical syndromes. Organization of forms of epilepsy is first by specificity: electroclinical syndromes, nonsyndromic epilepsies with structural-metabolic causes, and epilepsies of unknown cause. Further organization within these divisions can be accomplished in a flexible manner depending on purpose. Natural classes (e. g., specific underlying cause, age at onset, associated seizure type), or pragmatic groupings (e. g., epileptic encephalopathies, self-limited electroclinical syndromes) may serve as the basis for organizing knowledge about recognized forms of epilepsy and facilitate identification of new forms.

Summary Purpose: The aim of this study was to assess behavioral aspects of visual function and visuoperceptual abilities in patients with Panayiotopoulos syndrome (PS), and their possible associations with clinical and electroencephalography (EEG) findings in order to establish the possible effect of interictal paroxysmal activity on visual performance. Methods: The cohort included 28 patients (14 male and 14 female) of ages ranging between 4 and 15 years. All patients underwent serial videopolygraphic studies and a detailed battery of tests assessing visual abilities, including assessment of acuity, stereopsis, visual fields, and visuoperceptual abilities; tests included the Movement Assessment Battery for Children, the Visuo Motor Integration tests, and evaluation of motion and form coherence threshold. Results: On the assessment of visual function, only 4 of the 28 (15%) had abnormal crowding acuity and one had abnormal stereopsis. On the visuoperceptual assessment, one patient had abnormal results on the Visuo Motor Integration tests, and one on the Movement Assessment Battery for Children, whereas 4 (15%) had abnormal results for form coherence threshold and one for motion threshold. Discussion: Our results suggest that, although most of our patients had focal or diffuse EEG abnormalities involving the occipital regions, abnormalities of visual and visuoperceptual function were relatively uncommon. Age at onset of seizure

孤発性片麻痺性片頭痛（SHM）で発症したPanayiotopolos症候群（PS）の女児例を経験した。4歳1カ月から8カ月間に、早朝睡眠中あるいは、朝起床直後に発症する4回の発作を認めた。最初2回の発作は、激しい頭痛と嘔吐、一過性左片麻痺といったSHMに合致する症状で、3回目は反復性嘔吐と軽い頭痛、4回目は眼球左方偏倚、四肢脱力、意識障害、反復性嘔吐と頭痛といったPSに合致する症状であった。4回目の発作後に初めて、脳波で右後頭部優位の棘波が確認され、PSと診断された。 特発性てんかんの一部と片麻痺性片頭痛では、共通の神経イオンチャネル遺伝子の異常が知られている。こうした異常を背景に、片麻痺性片頭痛とPSが並存する可能性もあり得ると思われる。

Panayiotopoulos syndrome is a common multifocal autonomic childhood epileptic disorder with significant clinical, pathophysiological and management implications. It affects otherwise normal children with onset at around 3-6 years. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms and mainly ictal vomiting. EEG shows shifting and/or multiple foci, often with occipital dominance. Despite characteristic clinical and EEG manifestations Panayiotopoulos syndrome is often confused with occipital epilepsy and acute non-epileptic disorders such as encephalitis, syncope, cyclic vomiting or atypical migraine. This review aims to describe Panayiotopoulos syndrome on the basis of independent major studies and provide clinical clues for diagnosis and management.

In its first description (1989), Panayiotopoulos syndrome was defined as an idiopathic epilepsy syndrome with an excellent prognosis, characterized by a clinical ictal triad of nocturnal seizures, tonic deviation of the eyes, and vomiting. The electroencephalographic and clinical features of this condition were highly suggestive of occipital lobe involvement. Subsequently, the concept of this benign age-related focal epilepsy has been expanded over the years, including a wider and larger spectrum of seizure manifestations far beyond the occipital manifestations, and for which the eponym of Panayiotopoulos syndrome (PS) has been adopted. However, many theoretical and practical points, including diagnostic, genetic, and pathophysiologic issues remain still unresolved for PS.

Background: The underlying neurologic disorders of status epilepticus (SE) in childhood remain poorly characterized. Methods: We reviewed 249 consecutive patients with SE, aged 1 month to 18 years, who were referred to Tottori University Hospital from 1984 to 2002. After exclusion of SE patients with acute symptomatic etiology and progressive encephalopathy, such as acute encephalitis/encephalopathy, meningitis, head trauma, or metabolic disorders, we analyzed 112 patients, aged 3 months to 14 years, and focused on the epilepsy classification and perinatal brain damage in these patients. Results: Major underlying neurologic disorders were non-symptomatic epilepsy (41 patients, 36.6%), perinatal brain damage (15 patients, 13.4%), non-syndromic mental retardation (17 patients, 15.2%), and congenital disorders including chromosomal abnormalities (13 patients, 11.6%). In non-symptomatic epilepsy, childhood epilepsy with occipital paroxysms (Panayiotopoulos syndrome, 11 patients) and severe myoclonic epilepsy in infancy (SMEI, 6 patients) were common and had high recurrence rates (81.8% and 66.7%, respectively). In patients with a history of perinatal brain damage, preterm birth, neonatal seizure, asphyxia, and neonatal hypoglycemia were frequent. Neonatal hypoglycemia and neonatal seizure were related to the recurrence of SE (100% and 87.5%, respectively). They were mostly diagnosed as symptomatic occipital lobe epilepsy. Parieto-occipital paroxysms were associated with a high recurrence rate of SE (80.6%). Conclusions: Although the underlying neurologic disorders of SE are heterogeneous, three specific epileptic syndromes (Panayiotopoulos syndrome, SMEI, and symptomatic occipital lobe epilepsy secondary to neonatal hypoglycemia and neonatal seizure) were the most common causes of SE and were associated with higher recurrence rates.

Background Electroencephalogram (EEG) activity in normal subjects and epileptic patients is often closely related to the eye's status such as eye opened (EO), eye closure (ECL) and eyes closed (EC). ECL is the period immediately after closing of the eyes and only lasts for less than 3 seconds if the eyes remain closed. EC is the period as long as the eyes are closed. Epileptiform changes on EEG induced by ECL or EC are called the changes of ECL sensitivity (ECLS) or EC sensitivity (ECS). ECLS occurs mainly but not exclusively in photosensitive patients and ECS has been seen rarely in photosensitive patients. This study aimed to investigate the relationships among ECLS, ECS, photosensitivity and epilepsy syndromes in children. Methods EEG records from child patients in the EEG Department of Peking University First Hospital during the period of May 2005 to May 2007 were examined for the presence of ECLS or ECS. Open-close eye tests and intermittent photic stimulations were carried out during video-EEG monitoring for examining ECLS, ECS and photosensitivity. Results Based on ECLS and ECS on their EEGs, 30 patients were divided into ECLS group (16 cases) and ECS group (14 cases). There were more boys than girls in the two groups. The mean age of initial detection of ECLS and ECS was 10 years, and the average onset age of seizures was 9 years. The epilepsy syndromes in the ECLS group included idiopathic photosensitive occipital lobe epilepsy, Panayiotopoulos syndrome, symptomatic occipital lobe epilepsy, juvenile myoclonic epilepsy, juvenile absence epilepsy, eyelid myoclonia with absences, epilepsy with grand mal on awakening and pure photosensitive epilepsy with mainly generalized tonic clonic seizures. Those in the ECS group were juvenile myoclonic epilepsy, idiopathic photosensitive occipital lobe epilepsy, Panayiotopoulos syndrome and Gastaut type-idiopathic children occipital epilepsy. Photosensitivity was detected in 88% of patients with ECLS and 29% of patients with ECS. Conclusions ECLS and ECS are relatively common in females. Comparing with ECS, ECLS is found in more epilepsy syndromes. However, ECS and ECLS could exist in the same epilepsy syndrome. ECLS and ECS can be associated or dissociated with photosensitivity. The rate of ECLS with photosensitivity is higher than that of ECS with photosensitivity, suggesting that mechanisms for ECLS, ECS and photosensitivity may be different but correlated.

A big advance in epileptology has been the recognition of syndromes with distinct aetiology, clinical and EEG features, treatment and prognosis. A prime and common example of this is rolandic epilepsy that is well known by the general paediatricians for over 50 years, thus allowing a precise diagnosis that predicts an excellent prognosis. However, rolandic is not the only benign childhood epileptic syndrome. Converging evidence from multiple and independent clinical, EEG and magnetoencephalographic studies has documented Panayiotopoulos syndrome (PS) as a model of childhood autonomic epilepsy, which is also common and benign. Despite high prevalence, lengthy and dramatic features, PS as well as autonomic status epilepticus had eluded recognition because emetic and other ictal autonomic manifestations were dismissed as non-epileptic events of other diseases. Furthermore, PS because of frequent EEG occipital spikes has been erroneously considered as occipital epilepsy and thus confused with the idiopathic childhood occipital epilepsy of Gastaut (ICOE-G), which is another age-related but rarer and of unpredictable prognosis syndrome. Encephalitis is a common misdiagnosis for PS and migraine with visual aura for ICOE-G. Pathophysiologically, the symptomatogenic zone appears to correspond to the epileptogenic zone in rolandic epilepsy (sensory-motor symptomatology of the rolandic cortex) and the ICOE-G (occipital lobe symptomatology), while the autonomic clinical manifestations of PS are likely to be generated by variable and widely spread epileptogenic foci acting upon a temporarily hyperexcitable central autonomic network. Rolandic epilepsy, PS, ICOE-G and other possible clinical phenotypes of benign childhood focal seizures are likely to be linked together by a genetically determined, functional derangement of the systemic brain maturation that is age related (benign childhood seizure susceptibility syndrome). This is usually mild but exceptionally it may diverge to serious epileptic disorders such as epileptic encephalopathy with continuous spike and wave during sleep. Links with other benign and age-related seizures in early life such as febrile seizures, benign focal neonatal and infantile seizures is possible. Overlap with idiopathic generalized epilepsies is limited and of uncertain genetic significance. Taking all these into account, benign childhood focal seizures and related epileptic syndromes would need proper multi-disciplinary re-assessment in an evidence-based manner.

The aim of this study was to analyse the epidemiological, clinical and evolutional characteristics of Panayiotopoulos syndrome (PS) in order to facilitate the diagnosis in daily clinical practice. Thirty-seven medical records of patients diagnosed with PS were reviewed and the epidemiological and clinical features, results of complementary studies and evolutional data were collected. Mean age at diagnosis was 5.4 years. Female/male ratio was 2.1. The majority of seizures occurred during sleep (67.2%). They usually begin with autonomic manifestations or vomiting (70.1%). Ictal eye and/or cephalic deviation and progression to partial or generalized convulsions were also quite frequent. EEG showed occipital spikes in 28 patients (75.7%). Besides, 71.3% of recurrences were observed in the first 6 months after diagnosis, and 82.9% of the patients had no seizures beyond 2 years from diagnosis. One patient with an atypical development was recorded. The PS is a relatively frequent epileptic syndrome in paediatric age, especially in pre-school children. Although its semiological sequence is fairly typical, the unspecific clinical and neurological characteristics complicate the diagnostic suspicion. Prognosis is excellent; however, it would be convenient to keep a strict evolutional follow-up in these patients as an atypical evolution might occur.

Focal spike activities in Panayiotopoulos syndrome involve all brain regions in electroencephalography, and commonly reveal multiple foci, often through occipital predominance. To investigate correlations between developmental brain maturation and spike origin in Panayiotopoulos syndrome, we evaluated age-related or duration-related magnetoencephalographic spike localization in 25 patients with Panayiotopoulos syndrome. Regarding age at examination, patients with frontal spikes were significantly older than patients with spikes on rolandic, parieto-occipital, or calcarine sulci. Occipital spikes were classified into two subgroups, located at the calcarine sulcus and parieto-occipital sulcus. Both calcarine and parieto-occipital localizations were seen in patients around the same age. Follow-up magnetoencephalography was performed on three patients, and demonstrated shifting localization or disappearance of magnetoencephalographic spikes. These results suggest that the location of spike discharges is not directly related to seizure symptoms, but instead indicates maturation-related cortical hyperexcitability in patients with Panayiotopoulos syndrome.

Purpose: We have studied the clinical differences between early-onset benign epilepsy with centro-temporal spikes (early-onset BECT) and Panayiotopoulos syndrome (PS) to investigate the hypothesis that BECT and PS nosologically constitute age-dependent benign childhood seizure susceptibility syndromes based on a common etiopathogenesis. Subjects and Methods: The subjects were 24 patients with BECT and 62 patients with PS, who satisfied the following definitions: 1) onset of epilepsy before 5 years of age; 2) the BECT and PS seizures started mainly with orofacial focal motor attacks and emetic symptoms followed by focal seizures, respectively; 3) follow-up examinations for longer than 2 years. We compared the various clinical features between these two groups. Results: In children with early-onset BECT, the seizures at times manifested with hypersalivation, vomiting, and focal motor seizures, but the vomiting that developed in the middle of seizures was different from the initial vomiting observed in patients with PS. Although the seizures recurred more frequently in patients with early-onset BECT, the incidence of status epilepticus as well as prolonged seizures was higher in those with PS. The patients demonstrating below borderline IQ scores and mild developmental behavioral disorders were more frequently seen in early-onset BECT than PS, accounting for 37.5 and 14.6% (P

Summary: Objectives: To characterize the electroclinical features and evolution of Panayiotopoulos Syndrome (PS). Methods: Children with electroclinical criteria of PS were prospectively identified and followed-up clinically, and with sleep and awake EEGs between February 1990 and 2006. Results: We identified 192 patients with PS. In the same length of time 398 children with benign childhood epilepsy with centro-temporal spikes (BCECTS) were registered. PS had a peak age at onset of 5 years. Autonomic manifestations were one of the most common ictal event. Ictal deviation of the eyes and progression to generalized convulsions were also quite frequent. Approximately one third had partial status epilepticus. In all patients except five, the seizures occurred during sleep. One-third also had fits while awake. Sixteen children had concomitant symptoms of rolandic epilepsy and eight developed rolandic seizures after remission of PS seizures. Prognosis was excellent. Eighty-four (44.2%) had a single seizure, 79 (41.2%) had 2–5 fits, and 28 (14.6%) had frequent seizures. Conclusion: PS is less common than BCECTS, but is well defined and easily recognizable by clinical and EEG features, with autonomic manifestations as one of the most common ictal event.

Panayiotopoulos syndrome is a relatively frequent and benign epileptic syndrome, characterised by predominantly autonomic symptoms and/or simple motor focal seizures followed or not by impairment of consciousness. Interictal EEG shows occipital spikes although multifocal spikes with high amplitude sharp-slow wave complexes at various locations can be present. This syndrome can imitate gastroenteritis, encephalitis, syncope, migraine, sleep disorders or metabolic diseases. The peculiar aspects should be known not only by epileptologists but also by general doctors because a correct diagnosis would avoid aggressive interventions and concerns on account of its benign outcome. This review focuses on the main clinical and EEG features of this epilepsy underlining its typical and atypical symptoms and its management.

To evaluate epileptic children with occipital lobe epilepsy (OLE) in the light of the characteristics of Panayiotopoulos syndrome and late-onset occipital lobe epilepsy of Gastaut (OLE-G).Patients were categorized into six groups: primary OLE with autonomic symptoms (Panayiotopoulos syndrome), primary OLE with visual symptoms (OLE-G), secondary OLE with autonomic symptoms (P-type sOLE), secondary OLE with visual symptoms (G-type sOLE), and non-categorized primary OLE and non-categorized secondary OLE according to characteristic ictal symptoms of both Panayiotopoulos syndrome and OLE-G, as well as aetiology (primary or secondary). Patients were compared with regards to seizure symptoms, aetiology, cranial imaging, EEG, treatment and outcome.Of 108 patients with OLE (6.4±3.9 years of age), 60 patients constituted primary groups (32 with Panayiotopoulos syndrome, 11 with OLE-G, and 17 with non-categorized primary OLE); the other 48 patients constituted secondary groups (eight with P-type sOLE, three with G-type sOLE, and 37 with non-categorized sOLE). Epileptiform activity was restricted to the occipital area in half of the patients. Generalized epileptiform activity was observed in three patients, including a patient with Panayiotopoulos syndrome (PS). Only one patient had refractory epilepsy in the primary groups while such patients made up 29% in the secondary groups.In OLE, typical autonomic or visual ictal symptoms of Panayiotopoulos syndrome and OLE-G do not necessarily indicate primary (i.e. genetic or idiopathic) aetiology. Moreover, primary OLE may not present with these symptoms. Since there are many patients with OLE who do not exhibit the characteristics of Panayiotopoulos syndrome or OLE-G, additional definitions and terminology appear to be necessary to differentiate between such patients in both clinical practice and studies.

Sleep and epilepsy have a close relationship. About 20% of patients suffer seizures only during the night, approximately 40% only during the day and approximately 35% during the day and night. In certain epilepsy syndromes, the occurrence of seizures is strongly related to sleep or awakening. Infantile spasms appear predominately on awakening, and hypsarrhythmia is sometimes visible only in sleep. Children with Panayiotopoulos syndrome or benign epilepsy with centrotemporal spikes (BECTS) have seizures mostly when asleep, and in both syndromes interictal spike waves are markedly accentuated in slow wave sleep. Electrical status epilepticus during slow sleep/continuous spike wave discharges during sleep (ESES/CSWS), atypical benign partial epilepsy, and Landau–Kleffner syndrome are epileptic encephalopathies with substantial behavioral and cognitive deficits, various seizures, and continuous spike–wave activity during non–rapid eye movement (NREM) sleep. The hallmark of juvenile myoclonic epilepsy and grand mal seizures on awakening are seizure symptoms within 2 hours after awakening, often provoked by sleep deprivation. Nocturnal frontal lobe epilepsy is sometimes mistaken for parasomnia. Differentiation is possible when the clinical symptoms and the frequency of the paroxysmal events per night and month are carefully observed and nocturnal video electroencephalography (EEG) performed. Sleep EEG recordings may be helpful in patients with suspected epilepsy and nonconclusive awake EEG. Depending on the clinical question, sleep recordings should be performed during nap (natural sleep or drug induced), during the night, or after sleep deprivation.

Introdução: O Síndrome de Panayiotopoulos é a segunda epilepsia focal idiopática infantil mais comum, com um pico de incidência aos 4-5 anos. A sua incidência é subestimada devido às manifestações clínicas atípicas, caracterizando-se por crises com predomínio de sintomas autonómicos. Caso clínico: Descreve-se um caso clássico de Síndrome de Panayiotopoulos, com crises recorrentes, com boa resposta à terapêutica antiepiléptica e remissão após alguns anos. Conclusão: Salienta-se a importância da suspeição clínica deste síndrome epiléptico e a utilidade do registo electroencefalográfico que inclua um período de sono. Na maioria dos casos o prognóstico é bom, apesar de ser necessário manter o tratamento por um período de tempo variável. Introduction: Panayiotopoulos syndrome is the second commonest idiopathic focal childhood epilepsy, with a peak of age at onset 4-5 years. Its incidence is usually underestimated, because of the typical unusual clinical manifestations of the seizures with predominant autonomic changes. Case report: We describe a classical case of Panayiotopoulos syndrome, with recurrent seizures, but good response to antiepileptic drug treatment and remission after a few years. Conclusion: The importance of the clinical suspicion of this epileptic syndrome and the usefulness of an electroencephalographic recording including a period of sleep is underlined. In most diagnosed cases prognosis is good, although therapy is usually necessary for a variable period of time.

SummaryWe describe herein a patient with Panayiotopoulos syndrome (PS) showing an atypical course. The patient initially had seizures typical of this syndrome from 3 to 5 years of age. EEG showed right occipital high-amplitude sharp and slow-wave complexes followed by brief generalized discharges of slow waves. Sequential EEGs obtained from 5 to 11 years of age showed both multifocal discharges and generalized spike and wave complexes. With these changes in EEG findings, the patient experienced various types of seizures. The seizures were frequent and showed oculocephalic deviation followed by absence, atonic seizures, generalized tonic clonic convulsions and clonic seizures of the eyelids, which were observed between 7 and 10 years of age. Antiepileptic drugs were only partially effective for these seizures. Ictal EEG recorded at 8 years of age revealed high-voltage slow waves from the bilateral frontal and occipital regions prior to diffuse high-amplitude spike–wave bursts. At 9 years of age, magnetoencephalography (MEG) revealed the calculated dipoles of the preceding bifrontal spike–wave discharges to be in the frontal areas, while those of the following generalized spike–wave bursts were in the bilateral mid-temporal areas. In PS, reportedly, dipoles of multifocal epileptic discharges are usually located in the occipital and Rolandic areas. The unique clinical evolution in our case may be associated with the unusual frontal localization of dipoles detected by MEG.

Summary: Purpose: Panayiotopoulos syndrome (PS) is a type of benign childhood partial epilepsy that is frequently associated with abundant multifocal spikes other than main occipital spikes on the EEG. In this study, we investigated the characteristic features of dipoles in PS. Methods: We performed dipole analysis of the interictal occipital spike discharges seen in 10 children with PS (group A) and in 10 children with other types of symptomatic localization-related epilepsy (group B). We analyzed the dipoles of the averaged spike in each patient. Results: In group A, the averaged occipital spikes in each patient showed dense dipole locations in the mesial occipital area; in group B, widely scattered dipole locations were observed. In Group A, the geometric centers of the dipoles at each time point (such as at the main negative peak and before or after the main peak) were estimated in the neighboring locations. In contrast, they tended to be scattered in group B. Conclusions: Our study reveals that PS has high dipole stability, similar to that of rolandic epilepsy. From the electroencephalographic view, this seems to indicate a close link between these two syndromes.

Methods: Thirteen PS patients with mean age 5 (3 to 14) years were studied. Magnetoencephlography (MEG) was done using whole-head 204-channel “Neuromag” with simultaneous EEG. The estimated locations of equivalent current dipoles (ECDs) of each peak of spike discharges were plotted on MRI images. Results: Most patients (84.6%) showed ECD concentrations in the areas alongside parieto-occipital sulcus (8/13, 61.5%) or calcarine sulcus (4/13, 30.8%), except two in rolandic areas only. Despite Fp-O synchronization of spike discharges in EEG in five patients, no frontal locations of ECD were found. All five patients (all males) with sylvian seizures, who also showed ECD concentrations in rolandic areas, had relatively earlier age of onset (3-4 years) and higher seizure-frequency (mostly >10 times). The directions of each ECD were almost regular, except one, the oldest patient (14 years), showing irregular and dispersed ECDs alongside bilateral calcarine sulci. In one patient, who has had frequent momentary atonic seizures in addition to several sylvian seizures, ECD concentrations in bilateral rolandic areas were relatively broad. Discussion and Conclusion: According to our results, occipital spikes in scalp EEG seem to be originating from the restricted cortical areas alongside parieto-occipital sulcus or calcarine sulcus. In addition, more patients (61.5%) showed ECD concentration in the areas alongside parieto-occipital sulcus. Therefore, it may be inappropriate to classify this clinical entity as occipital epilepsy. Five patients (38.5%) had both sylvian seizures and rolandic discharges, which were also confirmed in MEG. This evidence suggests that PS has a quite high affiliation with benign childhood epilepsy with centro-temporal spikes (BCECTS). One of these five patients was diagnosed as having atypical type of BCECTS by Aicardi. Although Panayiotopoulos proposed the concept of age-dependent diffuse cortical hyper-excitability, these results seem to suggest localized cortical hyper-excitability in the areas alongside major cortical sulci or fissures such as parieto-occipital sulcus or calcarine sulcus. However, the reason is unknown why those inter-ictal discharges from the areas alongside parietooccipital sulcus or calcarine sulcus do not cause directly typical seizure phenomena, while rolandic discharges themselves cause sylvian seizures. Thus, occipital spikes in scalp EEG in PS seem to be originating from the restricted cortical areas alongside parieto-occipital sulcus or calcarine sulcus. It may be inappropriate to classify PS as pure occipital epilepsy.

Panayiotopoulos syndrome (PS) is a type of benign childhood partial epilepsy, which has a good prognosis despite the fact that it is frequently associated with abundant multifocal spikes on the electroencephalography (EEG). We investigated whether stable dipoles, as seen in rolandic epilepsy, were also present in PS. We performed dipole analysis of the interictal spike discharges seen in the interictal EEGs of eight children with PS. We chose more than 10 spikes for each kind of spike, and investigated whether or not more than three of these spikes showed consistently stable dipole locations. (1) We observed 15 different kinds of spikes in various regions in the EEGs of the eight children. (2) Twelve of the 15 kinds of spikes had dipoles with a high goodness of fit. Furthermore, 14 of the 15 spikes had stable dipoles with similar locations for more than three individual spikes. (3) Fourteen of the 15 spikes, including frontal spikes, showed dense dipole locations in the mesial occipital area. Thirteen of these 14 spikes also showed other dipole locations in the rolandic area and/or the vertex (Cz). Our study revealed that the various types of spikes observed in PS have similar and stable dipole locations. The dipoles showing high stability, were located in the mesial occipital area, and were accompanied by dipoles located in the rolandic area. The stability and location of these dipoles indicate that there may be a pathogenetic link between PS and rolandic epilepsy.

Autonomic seizures and autonomic status epilepticus in children have a high prevalence, manifest with dramatic clinical symptoms, and have important clinical and management implications. They probably affect approximately 13% of children aged 3-6 years with one or more nonfebrile seizures, or 6% in the age group 1-15. The primary cause is an idiopathic age-dependent epileptogenic susceptibility (Panayiotopoulos syndrome), but 10-20% are due to cerebral pathology. Autonomic seizures and autonomic status epilepticus have been best studied in Panayiotopoulos syndrome, which has been confirmed worldwide in more than 800 cases and recently recognized in the new classification scheme of the International League Against Epilepsy. Seizures start with autonomic symptoms, mainly emesis, while the child is usually fully conscious. Other more conventional seizure manifestations often ensue, but autonomic manifestations commonly predominate to the end of the seizure. Ictal syncope (transient loss of consciousness and postural tone) is an intriguing common symptom. Half of the seizures last longer than 30 minutes, constituting autonomic status epilepticus. Prognosis is invariably excellent except for the symptomatic cases. The interictal EEG shows great variability from normal to severely epileptogenic, often with multifocal spikes. Pathophysiology of Panayiotopoulos syndrome is unknown, but it is likely that they are due to diffuse maturation-related epileptogenicity activating susceptible-for-children emetic centers and the hypothalamus. Thus, Panayiotopoulos syndrome is not occipital epilepsy, with which it is often erroneously equated. Autonomic seizures and autonomic status epilepticus are frequently misdiagnosed and often treated as encephalitis, atypical migraine, cardiogenic syncope, or other unrelated medical conditions such as gastroenteritis. This review examines the existing evidence, provides a means of improving diagnostic yield, and proposes practice parameters and guidelines for the diagnosis and management of autonomic seizures and autonomic status epilepticus in children.

Panayiotopoulos syndrome is an idiopathic epilepsy syndrome presenting with a large variety of autonomic symptoms. The mechanism of autonomic symptoms is still not well understood. A neurologically normal 13-month-old boy presented on 2 occasions with complex partial status epilepticus that included significant ictal priapism. Inpatient brain magnetic resonance imaging (MRI) scan showed restricted diffusion-weighted imaging within his left temporal lobe and electroencephalograms (EEGs) left temporal slowing only. An outpatient EEG 6 months later showed abundant multifocal, predominantly posterior, as well as irregular generalized spike-and-slow-wave discharges on normal background consistent with the diagnosis of Panayiotopoulos syndrome. Ictal priapism is a previously undescribed phenomenon that is consistent with parasympathetic manifestations noted in this young boy. Acute postictal MRI and EEG findings suggest that this undeniably fascinating seizure semiological sign may be localized to the left temporal region.

Introduction Autonomic nervous system (ANS) involvement in children with paroxysmal events can be seen at multiple levels, including epileptic ictal and postictal symptoms, status epilepticus, non-epileptic spells, side effects of antiepileptic drugs and vagus nerve stimulation. Autonomic testing combines a battery of tests serving to define the severity of the problem. Aim The aim of the study was to present current knowledge regarding the involvement of ANS in children with paroxysmal disorders. Material and methods Using key words ANS, epilepsy and syncope the search was performed in collections of Elsevier and Springer Journals, as well as PubMed. Results Manifestation of ANS dysfunctions may comprise a clinical spectrum of symptoms, including apnea, arrhythmia, hypotension and aspiration. Certain pediatric epilepsy syndromes present with a unique prominence of autonomic signs and symptoms (Panayiotopoulos syndrome). Autonomic dysfunction has been hypothesized in children with channelopathies such as Dravet syndrome. In adolescents with syncope, autonomic testing can help to stratify the risk of future episodes and to guide treatment decisions. Cardiovagal testing has been demonstrated in a number of paroxysmal disorders to be a marker of autonomic dysfunction. Conclusions Recognition of the diverse autonomic manifestations of epilepsy and non-epileptic spells would enhance early diagnosis and appropriate management, ultimately improving quality the of life of the affected patients. Symptoms of ANS dysregulation may warning as for life threatening conditions, so they should be included as a part of medical history and physical examination.

Panayiotopoulos syndrome is encompassed in the classification of the ILAE in idiopathic focal epilepsies. Mutations in the SCN1A gene have been associated with the development of this syndrome. We present two cases of Panayiotopoulos syndrome in two monozygotic twins, who underwent a molecular analysis of SCN1A, but no alteration was found. These cases suggest a genetic origin, and SCN1A appears to be associated with the outcome but not with the development of this syndrome.