Scalp EEG-recorded high-frequency oscillations can predict seizure activity in Panayiotopoulos syndrome.

OBJECTIVE: We studied the relationship between the clinical course of Panayiotopoulos syndrome (PS) and high-frequency oscillations (HFOs) captured during interictal scalp electroencephalography (EEG) to determine the feasibility of using HFOs to detect seizure activity in PS. METHODS: We analyzed the interictal scalp EEGs of 18 children with PS. Age parameters, seizure frequencies, and antiepileptic drugs were compared between the HFO-positive (HFOPG) and HFO-negative (HFONG) groups. RESULTS: Thirteen patients (72.2%) had HFOs while five patients (27.8%) had no HFOs in 194 interictal EEG records. We found no statistically significant differences in the mean age of epilepsy onset and last seizure, seizure frequency, or frequency of status epilepticus. However, the seizure activity period of the HFOPG was significantly longer than that of the HFONG. Patients with an HFO duration longer than 2 years were intractable to treatment. In most cases, seizures did not occur in the absence of HFOs, even when the spikes remained. CONCLUSIONS: HFOs are related to the seizure activity period in patients with PS. SIGNIFICANCE: We propose that HFOs are a biomarker of epileptogenicity and an indicator for drug reduction because seizures did not occur if HFOs disappeared even if the spikes remained.

A reappraisal of interictal EEG characteristics in self-limited epilepsy with autonomic seizures, formerly known as Panayiotopoulos syndrome or early-onset benign occipital epilepsy.

PURPOSE: In the 2022 New International Classification of Epilepsy Syndromes, self-limited epilepsy with autonomic seizures (SeLEAS), formerly known as Panayiotopoulos syndrome is recognized as an electroclinical syndrome that is clinically characterized by autonomic seizures and electroencephalographically by multifocal EEG foci. EEG studies were reviewed herein and the suitability of the EEG definition to characterize SeLEAS was assessed. METHODS AND RESULTS: The EEG findings of SeLEAS studies published to date were reviewed and typical sites of EEG foci and their evolutionary changes with age were analyzed. Although previous studies investigated the details of interictal EEG characteristics in a sufficient number of SeLEAS cases, there were few systematically analyzing cross sectional and longitudinal EEG changes except one study. Despite these limited evidence, I propose the following practical and useful EEG definition. The interictal EEG characteristics of SeLEAS are multifocal EEG foci with age-dependent predominant locations; occipital (O) at 2-5 years old, and occipital and frontopolar (synchronous and independent O and Fp spikes) at 4-7 years old and centro-parieto-temporal (CPT) at 6-10 years old. O EEG foci evolve to multifocal EEG foci with a O-Fp or CPT predominance with age and disappear by 12∼16 years old. O-Fp EEG foci may further evolve to generalized spike-wave complexes and rarely to spike-wave activated in sleep. In rare cases, the EEGs do not have epileptic EEG foci. CONCLUSION: Interictal EEG foci in SeLAES may have different EEG patterns in terms of location and the mode of appearance depending on the age at which EEG is recorded. O-Fp EEG foci may be a specific EEG pattern indicating a diagnosis of SeLEAS.

A Case Report of a 5-Year-Old Girl with Self-Limited Epilepsy with Autonomic Seizures.

BACKGROUND: Self-limited epilepsy with autonomic seizures (SeLEAS), formerly known as Panayiotopoulos syndrome (PS), is a common multifocal autonomic childhood epileptic syndrome. SeLEAS affects 6% of children in between the ages of 1 and 15 years who have had one or more afebrile seizures in their lifetime. CASE: A 5-year-old girl was admitted to the paediatric emergency room (ER) of our hospital due to a reported episode of vomiting during her sleep, followed by central cyanosis perorally of sort duration (<5'), a right turn of her head, and gaze fixation with right eye deviation. She was dismissed after a one-day hospitalization free of symptoms. A month later, the patient was admitted to the paediatric ER of a tertiary health unit due to a similar episode. The patient underwent EEG, which revealed pathologic paroxysmal abnormalities of high-amplitude sharp waves and spike-wave complexes in temporal-occipital areas of the left hemisphere, followed by enhancement of focal abnormalities in temporal-occipital areas of the left hemisphere during sleep. The patient was diagnosed with SeLEAS and started levetiracetam. CONCLUSIONS: SeLEAS can be easily misdiagnosed as many physicians may not be very familiar with this disease, and, on the other hand, the autonomic manifestations can be easily disregarded as seizures. The physician must always be alert and search beneath the symptoms to find the cause rather than only treat them.

Analysis of spatio-temporal propagation of occipito-frontal spikes in childhood epilepsies by 3D sequential voltage mapping and dipole localization.

OBJECTIVE: We sought to study the spatio-temporal propagation of occipito-frontal spikes in childhood epilepsies by voltage mapping and dipole localization and identify types of occipito-frontal spikes based on onset, propagation, and stability of their dipoles. METHODS: Sleep EEG data of children, aged 1-14 years, with a minimum 1 h of recording from June 2018 to June 2021, were analyzed to identify occipito-frontal spikes. In total, 150 successive occipito-frontal spikes were manually selected from each EEG and using a source localization software were averaged using automated pattern matching with a threshold of 80%, and sequential 3D voltage maps of averaged spike were analyzed. Stability quotient (SQ) was calculated as the total number of averages/150. Stable dipole was defined as SQ ≥ .8. Dipole analysis was performed with principal component analysis using an age-appropriate template head model. RESULTS: Ten children with occipito-frontal spikes were identified; five with self-limited epilepsy with autonomic seizures (SeLEAS) and five with non-SeLEAS epilepsies. Three types of occipito-frontal spikes were identified: (1) narrow occipito-frontal spikes with stable dipoles seen in all five children with SeLEAS which were "apparently" synchronous and bilateral clone-like with an occipito-frontal interval of 10-30 ms and a homogeneous propagation pattern from a unilateral medial parieto-occipital region to an ipsilateral mesial frontal region; (2) wide occipito-frontal spikes with stable dipoles seen in one child with non-SeLEAS and developmental and/or epileptic encephalopathy with spike-wave activation in sleep (D/EE-SWAS) with an occipito-frontal interval of 45 ms, caused by focal spike propagation from a deeper temporal focus to ipsilateral peri-rolandic cortex; and (3) wide occipito-frontal spikes with unstable dipoles seen in four children with non-SeLEAS lesional epilepsies with an occipito-frontal latency of >50 ms and heterogeneous propagation patterns with poor intra-individual dipole stability. SIGNIFICANCE: We successfully identified different types of occipito-frontal spikes in childhood epilepsies. Although the term "occipito-frontal" is used to describe these spikes on the 10-20 EEG system, true propagation from occipital to frontal regions is not necessary. It is possible to differentiate idiopathic from symptomatic cases by analyzing the stability quotient and the occipito-frontal interval of occipito-frontal spikes.

Self-limited epilepsy with autonomic seizures: A case report.

Self-limited epilepsy with autonomic seizures, formerly known as benign occipital epilepsy of childhood or Panayiotopoulos syndrome is a focal epilepsy that is part of the epileptic syndromes with onset during childhood. The objective of this report is to raise awareness about its importance and describe the clinical manifestations, timely diagnosis, and treatment. A pediatric patient admitted with gastrointestinal manifestations is presented. The autonomic manifestations must be considered as part of the clinical spectrum that includes this disease and the digestive and autonomic manifestations that mask the diagnosis, sometimes even in the absence of motor seizures themselves. Electroencephalographic confirmation was performed, avoiding cataloging it in other differential diagnoses.

The Generation of Human iPSC Lines from Three Individuals with Dravet Syndrome and Characterization of Neural Differentiation Markers in iPSC-Derived Ventral Forebrain Organoid Model.

Dravet syndrome (DRVT) is a rare form of neurodevelopmental disorder with a high risk of sudden unexpected death in epilepsy (SUDEP), caused mainly (>80% cases) by mutations in the SCN1A gene, coding the Nav1.1 protein (alfa-subunit of voltage-sensitive sodium channel). Mutations in SCN1A are linked to heterogenous epileptic phenotypes of various types, severity, and patient prognosis. Here we generated iPSC lines from fibroblasts obtained from three individuals affected with DRVT carrying distinct mutations in the SCN1A gene (nonsense mutation p.Ser1516*, missense mutation p.Arg1596His, and splicing mutation c.2589+2dupT). The iPSC lines, generated with the non-integrative approach, retained the distinct SCN1A gene mutation of the donor fibroblasts and were characterized by confirming the expression of the pluripotency markers, the three-germ layer differentiation potential, the absence of exogenous vector expression, and a normal karyotype. The generated iPSC lines were used to establish ventral forebrain organoids, the most affected type of neurons in the pathology of DRVT. The DRVT organoid model will provide an additional resource for deciphering the pathology behind Nav1.1 haploinsufficiency and drug screening to remediate the functional deficits associated with the disease.

Epilepsy syndromes in cerebral palsy: varied, evolving and mostly self-limited.

Seizures occur in approximately one-third of children with cerebral palsy. This study aimed to determine epilepsy syndromes in children with seizures and cerebral palsy due to vascular injury, anticipating that this would inform treatment and prognosis. We studied a population-based cohort of children with cerebral palsy due to prenatal or perinatal vascular injuries, born 1999-2006. Each child's MRI was reviewed to characterize patterns of grey and white matter injury. Children with syndromic or likely genetic causes of cerebral palsy were excluded, given their inherent association with epilepsy and our aim to study a homogeneous cohort of classical cerebral palsy. Chart review, parent interview and EEGs were used to determine epilepsy syndromes and seizure outcomes. Of 256 children, 93 (36%) had one or more febrile or afebrile seizures beyond the neonatal period and 87 (34%) had epilepsy. Children with seizures were more likely to have had neonatal seizures, have spastic quadriplegic cerebral palsy and function within Gross Motor Function Classification System level IV or V. Fifty-six (60%) children with seizures had electroclinical features of a self-limited focal epilepsy of childhood; we diagnosed these children with a self-limited focal epilepsy-variant given the current International League Against Epilepsy classification precludes a diagnosis of self-limited focal epilepsy in children with a brain lesion. Other epilepsy syndromes were focal epilepsy-not otherwise specified in 28, infantile spasms syndrome in 11, Lennox-Gastaut syndrome in three, genetic generalized epilepsies in two and febrile seizures in nine. No epilepsy syndrome could be assigned in seven children with no EEG. Twenty-one changed syndrome classification during childhood. Self-limited focal epilepsy-variant usually manifested with a mix of autonomic and brachio-facial motor features, and occipital and/or centro-temporal spikes on EEG. Of those with self-limited focal epilepsy-variant, 42/56 (75%) had not had a seizure for >2 years. Favourable seizure outcomes were also seen in some children with infantile spasms syndrome and focal epilepsy-not otherwise specified. Of the 93 children with seizures, at last follow-up (mean age 15 years), 61/91 (67%) had not had a seizure in >2 years. Children with cerebral palsy and seizures can be assigned specific epilepsy syndrome diagnoses typically reserved for normally developing children, those syndromes commonly being age-dependent and self-limited. Compared to typically developing children with epilepsy, self-limited focal epilepsy-variant occurs much more commonly in children with cerebral palsy and epilepsy. These findings have important implications for treatment and prognosis of epilepsy in cerebral palsy, and research into pathogenesis of self-limited focal epilepsy.

Gastrointestinal and Autonomic Symptoms-How to Improve the Diagnostic Process in Panayiotopoulos Syndrome?

One of the most common epileptic disorders in the pediatric population is Panayiotopoulos syndrome. Clinical manifestations of this idiopathic illness include predominantly autonomic symptoms and dysfunction of the cardiorespiratory system. Another feature constitutes prolonged seizures that usually occur at sleep. It is crucial to differentiate the aforementioned disease from other forms of epilepsy, especially occipital and structural epilepsy and non-epileptic disorders. The diagnostic process is based on medical history, clinical examination, neuroimaging and electroencephalography-though results of the latter may be unspecific. Patients with Panayiotopoulos syndrome (PS) do not usually require treatment, as the course of the disease is, in most cases, mild, and the prognosis is good. The purpose of this review is to underline the role of central autonomic network dysfunction in the development of Panayiotopoulos syndrome, as well as the possibility of using functional imaging techniques, especially functional magnetic resonance imaging (fMRI), in the diagnostic process. These methods could be crucial for understanding the pathogenesis of PS. More data arerequired to create algorithms that will be able to predict the exposure to various complications of PS. It also concerns the importance of electroencephalography (EEG) as a tool to distinguish Panayiotopoulos syndrome from other childhood epileptic syndromes and non-epileptic disorders.

International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions.

The 2017 International League Against Epilepsy classification has defined a three-tier system with epilepsy syndrome identification at the third level. Although a syndrome cannot be determined in all children with epilepsy, identification of a specific syndrome provides guidance on management and prognosis. In this paper, we describe the childhood onset epilepsy syndromes, most of which have both mandatory seizure type(s) and interictal electroencephalographic (EEG) features. Based on the 2017 Classification of Seizures and Epilepsies, some syndrome names have been updated using terms directly describing the seizure semiology. Epilepsy syndromes beginning in childhood have been divided into three categories: (1) self-limited focal epilepsies, comprising four syndromes: self-limited epilepsy with centrotemporal spikes, self-limited epilepsy with autonomic seizures, childhood occipital visual epilepsy, and photosensitive occipital lobe epilepsy; (2) generalized epilepsies, comprising three syndromes: childhood absence epilepsy, epilepsy with myoclonic absence, and epilepsy with eyelid myoclonia; and (3) developmental and/or epileptic encephalopathies, comprising five syndromes: epilepsy with myoclonic-atonic seizures, Lennox-Gastaut syndrome, developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep, hemiconvulsion-hemiplegia-epilepsy syndrome, and febrile infection-related epilepsy syndrome. We define each, highlighting the mandatory seizure(s), EEG features, phenotypic variations, and findings from key investigations.

Differentiating early clinical features of Panayiotopoulos syndrome from acute encephalopathy.

BACKGROUND: Panayiotopoulos syndrome (PS) is a common benign epilepsy in childhood, characterized by predominantly autonomic symptoms such as emesis, pallor, and seizures, which are often prolonged. In an emergency room (ER), particularly when unconsciousness is prolonged, differentiating PS from acute encephalopathy is challenging. In this study, we aimed to elucidate the differences in clinical features of patients with PS and acute encephalopathy who visited our ER. METHODS: We retrospectively reviewed 18 patients who were transferred to our ER because of status epilepticus later diagnosed as PS, and 30 patients with acute encephalopathy, between July 2012 and July 2017. We compared patient demographics, clinical characteristics, and treatment. RESULTS: Most patients (90%) with acute encephalopathy had convulsive seizures of greater than or equal to 15 min, whereas only three patients (17%) with PS had convulsive seizures of greater than or equal to 15 min (P < 0.001). In addition, seizures were treatable in all patients with PS with a small dose of midazolam (0.1 mg/kg), but all patients with acute encephalopathy required midazolam at 0.3 mg/kg or more (P < 0.001). More patients with PS had autonomic symptoms compared to those with acute encephalopathy (e.g., vomiting [78% vs. 3%, P < 0.001]). Non-convulsive status epilepticus was observed in 22% of PS patients, but not in any acute encephalopathy patients. In contrast, fever was observed in all patients with acute encephalopathy (100%), but less frequently in those with PS (11%, P < 0.001). CONCLUSION: PS was characterized by 1) convulsive seizures shorter than 15 min, 2) seizures treatable with small doses of midazolam, and 3) autonomic symptoms. PS could be differentiated from acute encephalopathy in the early stages of the syndrome.

Panayiotopoulos syndrome: Unusual clinical manifestations.

PURPOSE: We retrospectively analyzed the electroclinical features, treatment, and outcome of patients with Panayiotopoulos syndrome (PS) who presented with unusual clinical manifestations. METHOD: A retrospective, descriptive, multicenter study was conducted evaluating 44 patients with PS who had seizures with an unusual semiology. Data from patients with PS seen at eight Argentine centers between April 2000 and April 2019 were collected. RESULTS: Twelve patients (29.2%) had ictal syncope or syncope-like epileptic seizures. Three children (7.3%) had recurrent episodes of vomiting. Four patients (9.7%) presented with urinary incontinence associated with autonomic signs and consciousness impairment. One child had hiccups with autonomic manifestations followed by eye deviation. One boy had episodes of laughter with autonomic symptoms followed by loss of consciousness. Six patients (14.6%) had hyperthermia without acute febrile illness with autonomic symptoms as the first manifestation. Six others (14.6%) had focal motor seizures characterized by eye and head deviation in four and eyelid blinking in two. Four patients (9.7%) had ictal headache as the initial manifestation followed by nausea and vomiting. Two children (4.8%) had their first seizure while asleep associated with cardiorespiratory arrest. Two children (4.8%) had oral automatisms, such as sucking and chewing. In two children (4.8%) coughing was the initial manifestation followed by emetic symptoms. One patient (2.3%) had vertigo with a sensation of fear, with eye deviation and unresponsiveness. One child started with continuous spikes and waves during slow sleep, behavior disturbances, and emetic symptoms. CONCLUSION: In this study, evidence of the existence of unusual clinical cases of PS with typical EEG patterns was found. Outcome was excellent.

Neuropsychological and behavioral profiles of self-limited epileptic syndromes of childhood: a cross-syndrome comparison.

Childhood epilepsy with centro-temporal spikes (CECTS), Childhood absence epilepsy (CAE) and Panayiotopoulos syndrome (PS) are some of the most common pediatric epileptic syndromes. Despite the relatively benign (self-limited) course of epilepsy, current evidence suggests that these conditions are associated with an increased risk of neuropsychological and behavioral comorbidities. This study provides a cross-epileptic syndromes' comparison reporting on the cognitive and behavioral profile of a cohort of 32 children with CECTS (n = 14), CAE (n = 10) and PS (n = 8), aged 6 to 15 years old. Frequent, although often subclinical cognitive difficulties involving attention, executive functions and academic abilities were found in children with CECTS and CAE, and to a lesser extent in PS. Internalizing symptoms (particularly anxiety) were more common in the PS group compared to CECTS and CAE based on parental reports. Correlational analysis revealed a significant correlation between phonemic fluency and seizure-free interval at the time of evaluation, suggesting a beneficial effect of epilepsy remission on this executive function measure in all the three groups. These results add to existing literature providing further detail on neuropsychological and behavioral peculiarities of children with CECTS, CAE, and PS. Moreover, the need for neuropsychological assessment as part of the standard childhood epilepsy evaluation is stressed. The results are discussed in the context of the current literature, highlighting areas of consensus and controversies related to the clinical management of these epileptic syndromes as well as directions for future research.

A Self-Limited Childhood Epilepsy as Co-Incidental in Cerebral Palsy.

BACKGROUND: Cerebral palsy is the most frequent motor disability in childhood and is associated with a higher incidence of seizure disorders. In many instances, it is recognized that motor difficulties, as well as seizures, are from the same underlying brain lesion. However, self-limited childhood epilepsies, being a common group of epilepsy syndromes, would be expected to occur in patients with cerebral palsy merely on chance association and be unrelated to the structural brain imaging abnormality causing the motor impairment. Differential diagnosis in this case is important determining the long-term prognosis and need for anticonvulsant treatment. CASE PRESENTATION: Here, we report two patients with cerebral palsy combined with epilepsy, whose age at onset, seizure semiology and electroclinical features were similar to children with self-limited childhood-specific seizure disorders (childhood epilepsy with centrotemporal spikes and Panayiotopoulos syndrome). CONCLUSION: These cases highlight the importance of comprehensive differential diagnosis of seizures in cerebral palsy. Co-existence of age-dependent focal epilepsies with an underlying brain pathology as white matter injury, not affecting the cerebral cortex, might take place in the case of children with impaired motor skills. With health systems increasingly utilizing clinical pathways, it is important to consider the possibility of a self-limited childhood epilepsy and avoid aggressive and unnecessary medication treatment in children with cerebral palsy.

Standard procedures for the diagnostic pathway of sleep-related epilepsies and comorbid sleep disorders: an EAN, ESRS and ILAE-Europe consensus review.

BACKGROUND AND PURPOSE: Some epilepsy syndromes (sleep-related epilepsies, SREs) have a strong link with sleep. Comorbid sleep disorders are common in patients with SRE and can exert a negative impact on seizure control and quality of life. Our purpose was to define the standard procedures for the diagnostic pathway of patients with possible SRE (scenario 1) and the general management of patients with SRE and comorbidity with sleep disorders (scenario 2). METHODS: The project was conducted under the auspices of the European Academy of Neurology, the European Sleep Research Society and the International League Against Epilepsy Europe. The framework entailed the following phases: conception of the clinical scenarios; literature review; statements regarding the standard procedures. For the literature search a stepwise approach starting from systematic reviews to primary studies was applied. Published studies were identified from the National Library of Medicine's MEDLINE database and Cochrane Library. RESULTS: Scenario 1: Despite a low quality of evidence, recommendations on anamnestic evaluation and tools for capturing the event at home or in the laboratory are provided for specific SREs. Scenario 2: Early diagnosis and treatment of sleep disorders (especially respiratory disorders) in patients with SRE are likely to be beneficial for seizure control. CONCLUSIONS: Definitive procedures for evaluating patients with SRE are lacking. Advice is provided that could be of help for standardizing and improving the diagnostic approach of specific SREs. The importance of identifying and treating specific sleep disorders for the management and outcome of patients with SRE is underlined.

Case Report: Four Cases of Panayiotopoulos Syndrome Evolving to Juvenile Myoclonic Epilepsy.

Panayiotopoulos syndrome (PS) is a self-limited focal epilepsy appearing in childhood. Seizures in PS are self-limiting and do not usually continue into adulthood. Juvenile myoclonic epilepsy (JME) is the most common type of idiopathic generalized epilepsy, developing around puberty and continuing throughout adulthood. We describe four cases of PS in childhood in which JME developed in adolescence. Age at onset ranged from 4 to 8 years for PS, and 11 to 14 years for JME. JME developed after PS subsided, with the interval between last PS seizure and first JME seizure ranging from 1 to 10 years. No link between the two conditions has previously been described. Since PS is considered to show good prognosis and to be self-limiting, long-term observation has been considered unnecessary. No definitive factors were found to predict future evolution to JME in our series, so longer-term follow-up may be warranted for all PS patients.

Encephalopathy related to status epilepticus during slow sleep (ESES) as atypical evolution of Panayiotopoulos syndrome: an EEG and neuropsychological study.

We report two patients with Panayiotopoulos syndrome (PS) who developed encephalopathy related to status epilepticus during slow sleep (ESES) at the peak of their clinical course. Clinical charts and EEG data were reviewed. The patients exhibited nocturnal autonomic seizures and occipital EEG foci, the latter of which later evolved into multifocal EEG foci with synchronous frontopolar and occipital spikes (Fp-O EEG foci), and finally into continuous spikes-waves during sleep (CSWS; spike-wave index >85% based on whole-night sleep recording) at eight years and seven years of age, respectively. The occipital spikes always preceded frontopolar spikes by 30∼50 mseconds based on the analysis of CSWS. Neuropsychological ability, including IQ, deteriorated during the CSWS period in both patients. The autonomic seizures and focal to bilateral tonic-clonic seizures were initially resistant to antiepileptic drugs (AEDs), and occurred more than 10 times in both patients. However, the seizures and EEG findings gradually resolved, and AEDs were successfully terminated in both patients. PS can progress to ESES if the clinical course exhibits atypical evolution. The initial autonomic symptom of the seizures and interictal Fp-O EEG foci should be carefully monitored in patients with CSWS or ESES.

Evolución atípica del síndrome de Panayiotopoulos a síndrome de punta-ondas continuas durante el sueño lento / Atypical evolution of Panayiotopoulos syndrome to continuous spikes and wave's syndrome during slow sleep

Introducción: Las epilepsias focales idiopáticas constituyen uno de los grupos de epilepsias más frecuentes en la infancia. Excepcionalmente los pacientes con este tipo de epilepsias tienen evoluciones atípicas que constituyen un reto diagnóstico y terapéutico. Objetivo: Ilustrar la evolución atípica de la epilepsia focal idiopática tipo Panayiotopoulos. Presentación del caso: Adolescente de 13 años que presentó su primera crisis epiléptica a los 5 años de edad, de breve duración, mientras dormía tuvo apertura ocular, desviación de los ojos a la izquierda, abundante salivación y presentó un vómito. En tres años tuvo solo tres crisis. No recibió tratamiento con fármacos antiepilépticos hasta después de la tercera crisis, que fue más prolongada. Tras iniciar tratamiento con carbamazepina comenzó a presentar dificultades en el aprendizaje y marcada hiperactividad. Un electroencefalograma interictal de sueño demostró descargas de punta-ondas continuas en el sueño lento. Después de dos años de tratamiento se alcanzó la normalidad en el estudio electroencefalográfico de sueño, con retirada inicial de la carbamazepina, e introducción progresiva de clobazam y valproato de magnesio. Evolutivamente el paciente mantuvo las dificultades en el aprendizaje, con mejoría notable de su hiperactividad, sin recurrencia de crisis epilépticas. Conclusiones: El caso presentado constituye un ejemplo infrecuente de un paciente con una epilepsia focal idiopática con evolución atípica, probablemente inducida por la carbamazepina, con cuadro clínico-electroencefalográfico de más de dos años de duración, con mejoría favorecida por el tratamiento finalmente empleado, la evolución natural del síndrome o el efecto de ambos (AU)

Introduction: Idiopathic focal epilepsies are one of the most frequent epilepsy groups in childhood. Exceptionally, patients with this type of epilepsy have atypical evolutions that constitute a diagnostic and therapeutic challenge. Objective: To illustrate the atypical evolution of idiopathic focal epilepsy, type Panayiotopoulos. Case presentation: A 13-year-old adolescent who presented his first epileptic seizure at 5 years old, of short duration, while he slept had an eye opening, deviation of the eyes to the left, abundant salivation and vomiting. In three years he had only three seizures. He did not receive treatment with antiepileptic drugs until after the third seizure, which was longer. After starting treatment with carbamazepine, he began to have learning difficulties and marked hyperactivity. A sleep's interictal electroencephalogram showed continuous spikes and wave's discharges during the slow sleep. After two years of treatment, the normalization of the sleep electroencephalogram was achieved, with withdrawal of carbamazepine, and progressive introduction of clobazam and magnesium valproate. The patient remained evolutionarily with learning difficulties, with significant improvement in hyperactivity, without recurrence of seizures. Conclusions: The case presented is an infrequent example of a patient with idiopathic focal epilepsy with atypical evolution, probably induced by carbamazepine, with clinical-electroencephalographic symptoms during more than two years, with improvement favored by the final treatment used, the natural evolution of the syndrome or the effect of both(AU)

Ictal Epileptic Headache: When Terminology Is Not a Moot Question.

The relationship between headache and epilepsy is complex and despite the nature of this association is not yet clear. In the last few years, it has been progressively introduced the concept of the "ictal epileptic headache" that was included in the recently revised International Classification of Headaches Disorders 3rd edition (ICHD-3-revised). The diagnostic criteria for ictal epileptic headache (IEH) suggested in 2012 were quite restrictive thus leading to the underestimation of this phenomenon. However, these criteria have not yet been included into the ICHD-3 revision published in 2018, thus creating confusion among both, physicians and experts in this field. Here, we highlight the importance to strictly apply the original IEH criteria explaining the reasons through the analysis of the clinical, historical, epidemiological and pathophysiological characteristics of the IEH itself. In addition, we discuss the issues related to the neurophysiopathological link between headache and epilepsy as well as to the classification of these epileptic events as "autonomic seizure."

Panayiotopoulos syndrome and Gastaut syndrome are distinct entities in terms of neuropsychological findings.

BACKGROUND: Although the courses of self-limited focal epilepsies of childhood are considered as benign, a handful of studies suggested that these children may suffer from cognitive problems. Implementing tailor-made educational strategies would aid these children to reach their full potentials. Therefore, it is crucial to understand and differentiate the complete neuropsychological and behavioral profiles of these rather common syndromes. We aimed to examine the distinct cognitive and behavioral profiles of the Panayiotopoulos syndrome (PS) and the Gastaut syndrome (GS), comparatively. METHOD: Twenty patients with PS, 20 patients with GS, and 20 healthy controls have been recruited. The testing protocol included Wechsler Intelligence Scale for Children-Revised, Conner's Continuous Performance Test, Verbal Fluency Test, Stroop Color and Word Test, Color Trails Test, Tower of London Test, Symbol Digit Modalities Test, California Verbal Learning Test-Children's Version, Rey Complex Figure Test, Benton Face Recognition Test, Benton Judgment of Line Orientation, Peabody Picture Vocabulary Test, Reading and Writing Test, Child Behavior Checklist, Conner's Parent Rating Scale-48, and Behavior Rating Inventory of Executive Function. Demographical, clinical, electrophysiological data, and imaging findings have also been evaluated. RESULTS: With regard to intelligence, the patients with PS scored less in all scales compared to the healthy controls. However, only the performance IQ (intelligence quotient) scores differed significantly between the patient groups, with the patients with PS scoring lower than the patients with GS. Verbal memory problems were eminent in both of the patient groups; whereas, visual memory was impaired only in the group with PS. Psychomotor speed was affected in both groups. Reading problems were prominent only in the patients with PS. Writing and arithmetic skills were defective in both patient groups. There were no noteworthy behavioral problems in comparison to healthy subjects. CONCLUSION: Using neuropsychological profiles, this study demonstrated that the GS and the PS are two distinct entities. Cognitive dysfunction is a more prominent and widespread feature of the patients with PS; whereas, the patients with GS suffer only from milder and isolated cognitive problems.