�Ferrie replies on behalf of the Internal Consortium who produced the consensus view� (original) (raw)

Benign childhood focal epilepsies: assessment of established and newly recognized syndromes

Brain, 2008

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 evidencebased manner.

Benign childhood seizure susceptibility syndrome: three case reports

Epileptic disorders : international epilepsy journal with videotape, 2011

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).

Pediatric Epilepsy Syndromes: An Update and Critical Review

Epilepsia, 1996

Epilepsy syndromes occupy an important position in the current nosology of the epilepsies, describing and classifying seizure disorders with shared clinical and EEG features. Increasingly, this schema is being refined as new information becomes available and our understanding of etiology and presentation of each syndrome widens. Advances in neuroimaging and neurogenetics have been particularly important and are likely to fundamentally change our concepts of syndrome classification. At present, the International League Against Epilepsy classification of epilepsy syndromes according to presumed localization (partial, generalized, undetermined) and etiology (idiopathic, cryptogenic, symptomatic). In clinical practice, it is often useful to conceptualize epilepsy syndromes according to their usual age at presentation, which greatly facilitates syndrome identification in new patients and recognizes the age-related expression of many childhood epilepsies. Definitional problems exist for many pediatric epilepsy syndromes, particularly the epileptic encephalopathies of early infancy, the benign epilepsies of infancy and childhood, the myoclonic epilepsies of infancy and early childhood, and the idiopathic generalized epilepsies of childhood and adolescence. It is likely that further input from the fields of molecular genetics and neuroimaging will enable the classification of epilepsies to become more etiologically oriented and disease specific.

Relationships within the idiopathic generalized epilepsies

Journal of Clinical Neuroscience, 1997

The relationships between the various epileptic syndromes, which comprise the idiopathic generalized epilepsies that commence after infancy, were explored in 451 patients by comparing the incidences of various clinical and electroencephalographic features in subsets of the patients, determined primarily by their clinical patterns of seizure phenomena (absences, myoclonic and generalized tonic-clonic seizures), and secondarily by their ages at the onset of their seizures. The approach showed a bimodal distribution of ages of onset in the absence epilepsy group only. More subjects whose absences had begun under 10 years of age experienced very frequent episodes (52.0% vs 26.9%) and fewer experienced generalized tonic-clonic seizures (44.0% vs 71.2%). These differences were consistent with the distinction made in the International League Against Epilepsy's Classification between childhood and adolescent onset absence epilepsy. There were no tmambiguons age of onset related differences in the incidences of the features studied within each of the myoclonic seizure and the generalized tonic-clonic seizure only groups, although there were differences in the incidences of the features between the two groups. Thus the idiopathic generalized epilepsies studied appeared to comprise a number of discrete entities rather than a smooth continuum of age of onset related epileptic phenomena.

Is ″benign Childhood Epilepsy with Centrotemporal Spikes″ Always Benign?

Iranian Journal of Child Neurology, 2014

Objective To determine the prevalence of associated behavioral problems and prognosis with Benign Childhood Epilepsy with CentroTemporal Spikes (BCECTS). Descriptive, Cross Sectional study that was conducted from October 2009 to April 2013 in the Department of Pediatric Neurology, the Children’s Hospital Taif, KSA. Material & Methods This study was conducted after approval from the Ethics Committee of the Children’s Hospital Taif, Saudi Arabia. Thirty-two patients from the age of 3 to 10 years old were recruited from the pediatric neurology clinic over a period of 4 years. All the patients were selected based on history, EEGs, and neuropsychological and neurological examinations. EEGs were performed for all the patients while in awake and sleep states. Those who had centrotemporal discharges were included in the study. All the patients also underwent a brain MRI. Only two patients had mild cortical atrophy but developmentally they were normal. Results In our study, prevalence of BRE...

Prognosis of Benign Childhood Epilepsy with Centrotemporal Spikes: A Foliow-Up Study of 168 Patients

Epilepsia, 1988

Summary: A spontaneous and complete recovery of benign childhood epilepsy with centrotemporal or rolandic spikes (BECT) is taken for granted. However, some authors have reported the occurrence of generalized tonic-clonic seizures in a few adult patients and in some children who have seizures after a long period without problems. The aims of this study were (a).to search for early predictors of outcome and (b) to ascertain the long-term prognosis of BECT in a large group of patients. An attempt to relocate 268 patients born between 1941 and 1967 and consecutively seen as outpatients was undertaken. The outcome after age 20 is known for only 168. Being adults and cured, the others are no longer in touch with their clinics or have moved. Only one indicator of short-term prognosis was found: The earlier the onset of BECT, the longer the period with seizures. Of the 168 patients, 165 are seizure-free with follow-up ranging from 7 to 30 years. Three patients experienced generalized tonic-clonic seizures at age 18, 22–24, and 35. Two apparently had an isolated seizure. The occurrence of such seizures after recovery from BECT is a rare event (∼2% of cases) and a relapse with partial seizures is quite uncommon. These patients do not differ from patients remaining seizure-free.Summary: A spontaneous and complete recovery of benign childhood epilepsy with centrotemporal or rolandic spikes (BECT) is taken for granted. However, some authors have reported the occurrence of generalized tonic-clonic seizures in a few adult patients and in some children who have seizures after a long period without problems. The aims of this study were (a).to search for early predictors of outcome and (b) to ascertain the long-term prognosis of BECT in a large group of patients. An attempt to relocate 268 patients born between 1941 and 1967 and consecutively seen as outpatients was undertaken. The outcome after age 20 is known for only 168. Being adults and cured, the others are no longer in touch with their clinics or have moved. Only one indicator of short-term prognosis was found: The earlier the onset of BECT, the longer the period with seizures. Of the 168 patients, 165 are seizure-free with follow-up ranging from 7 to 30 years. Three patients experienced generalized tonic-clonic seizures at age 18, 22–24, and 35. Two apparently had an isolated seizure. The occurrence of such seizures after recovery from BECT is a rare event (∼2% of cases) and a relapse with partial seizures is quite uncommon. These patients do not differ from patients remaining seizure-free.RÉSUMÉLa guérison spontanée et compléte de l'épilepsie partielle bÉnigne à pointes centro-temporales (rolandiques) (EPCT) est une notion classique. Toutefois la survenue de críses géneAralisées convulsives chez quelques adultes ayant eu une EPCT a été signalée. De plus, les crises sont trop fréquentes ou se répetent pendant trop longtemps chez un certain nombre d'enfants pour ne pas poser des problémes. Les objectifs de cette étude étaient (1) de rechercher des facteurs précoces de pronostic à court terme, (2) d'apprécier le pronostic eloigné de l';EPCT chez un nombre suffisant de sujets. Sur 268 patients, nés entre 1941 et 1967, et vus consécutivement, 168 seulement ont pu être retrouvés. Les autres ne consultaient plus ou, avaient changé de domicile, voire de nom, et leur médecin avaient disparu. Le seul facteur de pronostic immédiat a été l'âge de la premiére crise: plus il était précoce, plus les crises avaient de chance de se répéter pendant une longue période. 165 patients, âgés de plus de 20 ans, n'ont jamais refait de crise, avec un recul allant de 7 à 30 ans. Trois patients ont eu des crises généralisées tonico-cloniques à 18, 22 et 24 et 35 ans. Deux d'ent re-eux n'ont fait jusqu'ici qu'une seule crise. La survenue de crises Grand Mai aprés rémission d'une EPCT est rare (environ 2% des cas) et une rechute sous la forme de crises partielles est encore plus rare. Rien ne distingue ces patients de ceux qui resteront en rémission.La guérison spontanée et compléte de l'épilepsie partielle bÉnigne à pointes centro-temporales (rolandiques) (EPCT) est une notion classique. Toutefois la survenue de críses géneAralisées convulsives chez quelques adultes ayant eu une EPCT a été signalée. De plus, les crises sont trop fréquentes ou se répetent pendant trop longtemps chez un certain nombre d'enfants pour ne pas poser des problémes. Les objectifs de cette étude étaient (1) de rechercher des facteurs précoces de pronostic à court terme, (2) d'apprécier le pronostic eloigné de l';EPCT chez un nombre suffisant de sujets. Sur 268 patients, nés entre 1941 et 1967, et vus consécutivement, 168 seulement ont pu être retrouvés. Les autres ne consultaient plus ou, avaient changé de domicile, voire de nom, et leur médecin avaient disparu. Le seul facteur de pronostic immédiat a été l'âge de la premiére crise: plus il était précoce, plus les crises avaient de chance de se répéter pendant une longue période. 165 patients, âgés de plus de 20 ans, n'ont jamais refait de crise, avec un recul allant de 7 à 30 ans. Trois patients ont eu des crises généralisées tonico-cloniques à 18, 22 et 24 et 35 ans. Deux d'ent re-eux n'ont fait jusqu'ici qu'une seule crise. La survenue de crises Grand Mai aprés rémission d'une EPCT est rare (environ 2% des cas) et une rechute sous la forme de crises partielles est encore plus rare. Rien ne distingue ces patients de ceux qui resteront en rémission.RESUMENSe acepta que la epilepsyía benigna infantil con puntas centro-encefÁlicas o rolándicas (BECT), mejora completa y espontáneamente. Sin embargo algunos autores han publicado la aparición de crisis generalizadas tónico-clónicas (GTLS) en algunos enfer-mos adultos y niños que han tenido ataques después de un largo periodo si problemas. Los propósitos de este estudio son: (a) la búsqueda de factores precoces que anticipen las posibles con-secuencias y (b) determinar el pronóstico a largo plazo de las BECT en un amplio grupo de enfermos. Se ha iniciado un intento de reclassificar 268 enfermos nacidos entre 1941 y 1967 y, pos-teriormente, seguidos como pacientes externos. Los resultados después de los 20 años de edad son sumamente conocidos en 168 enfermos. Los adultos jóvenes y curados no han mantenido contacto con las clínicas o se han cambiado de domicilio. Sólamente se ha encontrado un indicador vÁlido para el pronóstico a corto plazo: cuanto mÁs precoz sea el comienzo de las BECT mÁs largo será el periodo con ataques. De los 268 pacientes, 165 han permanecido sin ataques durante un periodo de seguimiento que ha variado entre 7 y 30 años. Tres enfermos experimentaron GTLS a las edades de 18, de 22 a 24 y a los 35. Dos, aparente-mente, tuvieron un ataque aislado. La aparición de GTLS después de una recuperación de BECT es un acontecimiento raro (alrededor del 2% de los casos) y la reaparición de ataques parciales es bastante infrecuente. Estos enfermos no se diferen-cian de los pacientes que permanecen sin ataques.Se acepta que la epilepsyía benigna infantil con puntas centro-encefÁlicas o rolándicas (BECT), mejora completa y espontáneamente. Sin embargo algunos autores han publicado la aparición de crisis generalizadas tónico-clónicas (GTLS) en algunos enfer-mos adultos y niños que han tenido ataques después de un largo periodo si problemas. Los propósitos de este estudio son: (a) la búsqueda de factores precoces que anticipen las posibles con-secuencias y (b) determinar el pronóstico a largo plazo de las BECT en un amplio grupo de enfermos. Se ha iniciado un intento de reclassificar 268 enfermos nacidos entre 1941 y 1967 y, pos-teriormente, seguidos como pacientes externos. Los resultados después de los 20 años de edad son sumamente conocidos en 168 enfermos. Los adultos jóvenes y curados no han mantenido contacto con las clínicas o se han cambiado de domicilio. Sólamente se ha encontrado un indicador vÁlido para el pronóstico a corto plazo: cuanto mÁs precoz sea el comienzo de las BECT mÁs largo será el periodo con ataques. De los 268 pacientes, 165 han permanecido sin ataques durante un periodo de seguimiento que ha variado entre 7 y 30 años. Tres enfermos experimentaron GTLS a las edades de 18, de 22 a 24 y a los 35. Dos, aparente-mente, tuvieron un ataque aislado. La aparición de GTLS después de una recuperación de BECT es un acontecimiento raro (alrededor del 2% de los casos) y la reaparición de ataques parciales es bastante infrecuente. Estos enfermos no se diferen-cian de los pacientes que permanecen sin ataques.