RESUMO
La Epilepsia Rolándica (ER) o Epilepsia con espigas centrotemporales, es la epilepsia idiopática más frecuente, focal autolimitada, de buena evolución. Debuta entre los 3 a 13 años, con crisis estereotipadas durante el sueño, al inicio o al despertar, focales motoras y somatosensoriales. El electroencefalograma (EEG) muestra una base normal con actividad epileptiforme interictal centrotemporal, con espigas con escaso desarrollo de onda lenta, uni o bilaterales. Se describe en los últimos años una forma atípica de ER (ERA), asociando otros tipos de crisis, que responden peor a tratamiento, manteniendo crisis, con complicaciones neuropsicológicas asociadas y deterioro del EEG, observándose en algunos casos, actividad, continua o casi continua en sueño. El objetivo de este trabajo es caracterizar a los pacientes con ER que se controlan en Red Salud UC-Christus y describir la presencia de posibles factores de riesgo asociados a la evolución atípica de la ER. 16 pacientes con ER típica y otros 6 con ERA, ambos grupos edad promedio 6 años al debut presentación, de predominio sexo masculino, la mayoría con buen rendimiento escolar y examen neurológico normal. En ER, crisis de predominio focales y base de EEG 62% normal. EEG entre ocasional y muy frecuentes descargas epileptiformes. En ERA, solo un 33% base normal, y predominio crisis generalizadas. Mayoría evoluciona con descargas EEG contínuas o casi continuas en sueño No-REM. Hubo peor respuesta a FAEs en ERA. Concluimos que este estudio es concordante con lo descrito en la literatura actual para pacientes con ER y ERA, por lo que recomendamos considerar la presencia de posibles factores indicadores, iniciales o durante la evolución, de formas atípicas de ER.
Abstract: Rolandic Epilepsy (ER) or epilepsy with centrotemporal spikes, is a focal and selflimited epilepsy and it is the most frequent and well-recognized idiopathic epilepsy. It is characterized by an age of presentation between 3 and 13 years, with stereotyped seizures during sleep, at the beginning or on awakening, which can be focal, motor and sensory. The electroencephalogram shows a normal base with interictal epileptiform activity, centrotemporal spikes with poor slow wave development, which can be unilateral o bilateral. In recent years, an atypical presentation of Rolandic Epilepsy (ERA) has been described, with presence of other types of seizures, with poor response to treatment, continued seizures and associated neuropsychological complications and deterioration of the electroencephalogram, in some cases with continuous or almost continuous activity during sleep. Our work aims to characterize the patients that are controlled in the Red Salud UC-Christus and to describe the presence of possible risk factors associated with the atypical evolution of Rolandic Epilepsy. We studied 16 patients with typical ER and other 6 with ERA, both groups with an age average of 6 years at time of debut presentation, more frequent in males, most with good school performance and normal neurological examination. In ER there were focal prevalence seizures and 62% had an EEG with a normal base. The EEG showed occasional to very frequent epileptiform discharges. In ERA, only 33% of the EEG had a normal base, and it most frequently showed generalized seizure. Most of the patients followed up with continuous or nearly continuous discharges in the EEG during REM sleep. There was worse response to FAEs in ERA. We conclude that this study is consistent with that described in the current literature for patients with ER and ERA, we recommend physicians to consider the presence of possible initial of belated indicators of atypical forms of ER.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Epilepsia Rolândica/diagnóstico por imagem , Eletroencefalografia/métodos , Fatores de Risco , Epilepsia Rolândica/epidemiologiaRESUMO
PURPOSE: The effect of etiology on the relationship between epilepsy and sleep during childhood has not been studied in detail. The aim of this study was to evaluate differences in sleep structure in drug-resistant epilepsies with different underlying causes. METHODS: We studied 31 patients with drug-resistant epilepsies with or without a structural lesion (lesional and nonlesional) and compared their sleep architecture with that of normal controls and with that of a group of children with benign epilepsy with rolandic spikes (BERS). Subjects underwent a single-night polysomnographic recording. Sleep recordings were scored according to the American Academy of Sleep Medicine (AASM) and cyclic alternating pattern (CAP) criteria. KEY FINDINGS: Compared to normal controls, patients with drug-resistant epilepsy showed a significant reduction of time in bed, total sleep time, rapid eye movement (REM) sleep, sleep stage N3, and sleep efficiency, and a significant increase in wake after sleep onset. The lesional subgroup showed a reduction in total sleep time and sleep latency and an increase in REM latency and wake after sleep onset. No significant differences, however, were found comparing the lesional and nonlesional subgroups. When compared to BERS, patients with drug-resistant epilepsy showed a significant reduction in sleep stage N3, REM sleep, and sleep efficiency. Regarding CAP analysis, when compared to controls, the drug-resistant group had an increased A1% and a decreased A2%, with a decrease of A1 index in N3 and a global decrease of A2 and A3 indexes. The lesional subgroup showed a slight increase of A1% with a decrease of A1 index in N3 and a global decrease of A2 and A3 indexes. Drug-resistant epilepsy, compared to benign epilepsy showed an increase of CAP rate in N2 and of A1 index in N1 and N2 but not in N3; A2 and A3 indexes were similar in both, but patients with drug-resistant epilepsy showed a significant reduction of A3 index in N1. SIGNIFICANCE: Our findings suggest that the presence of structural cerebral abnormalities may play an important role in disrupting sleep architecture.
Assuntos
Epilepsia/epidemiologia , Fases do Sono/fisiologia , Transtornos do Sono-Vigília/diagnóstico , Transtornos do Sono-Vigília/epidemiologia , Adolescente , Criança , Pré-Escolar , Epilepsia/fisiopatologia , Epilepsia Rolândica/diagnóstico , Epilepsia Rolândica/epidemiologia , Epilepsia Rolândica/fisiopatologia , Feminino , Humanos , Lactente , Masculino , Polissonografia/métodos , Transtornos do Sono-Vigília/fisiopatologiaRESUMO
Typical benign rolandic epilepsy (BRE) is a frequent and well-delineated epileptic syndrome in childhood. Mild cognitive and behavioral difficulties are increasingly recognized in the course of BRE and should not be considered as atypical features. Atypical features are recognized on electroclinical grounds. These features, particularly early age at onset and frequent spikes or spike-wave discharges, seem to be risk factors for neuropsychological deficits but also for an atypical evolution of BRE. Atypical evolutions of BRE are defined by the appearance of severe neuropsychological impairments and continuous spike-and-waves during slow sleep (CSWSS). The clinical expressions of these situations correspond to the syndromes known as atypical benign focal epilepsy of childhood (ABFEC), status of BRE, Landau-Kleffner syndrome (LKS), and CSWSS syndrome, which may be part of a continuum related to BRE.
Assuntos
Transtornos Cognitivos/epidemiologia , Epilepsias Parciais/epidemiologia , Epilepsia Rolândica/epidemiologia , Síndrome de Landau-Kleffner/epidemiologia , Sono/fisiologia , Idade de Início , Transtornos Cognitivos/diagnóstico , Comorbidade , Eletroencefalografia , Epilepsias Parciais/diagnóstico , Epilepsia Rolândica/diagnóstico , Seguimentos , Humanos , Síndrome de Landau-Kleffner/diagnóstico , Testes NeuropsicológicosRESUMO
PURPOSE: To characterize the electroclinical features and evolution of childhood occipital epilepsy of Gastaut (COE-G). METHODS: Children with electroclinical criteria of COE-G were retrospectively identified and followed-up clinically, and with sleep and awake EEGs between 1990 and 2007. RESULTS: We identified 33 patients with COE-G. In the same length of time, 201 children with Panayiotopoulos syndrome and 410 children with benign childhood epilepsy with centrotemporal spikes were registered. COE-G had a peak age at onset of 8.5 years. Visual manifestations were the most common ictal event. Ictal deviation of the eyes was frequent. Approximately half of the patients had migraine-like symptoms. In all patients the seizures occurred while awake, and 11 also had seizures during sleep. The majority of the patients had occipital spike-wave discharges when the eyes were closed that disappeared or attenuated when the eyes were opened. Prognosis was excellent in 80% of the cases. CONCLUSION: This study confirms the existence of COE-G, a rare but well-defined syndrome within the group of idiopathic focal epilepsies in childhood.
Assuntos
Eletroencefalografia/estatística & dados numéricos , Adolescente , Idade de Início , Córtex Cerebral/fisiopatologia , Criança , Pré-Escolar , Epilepsias Parciais/diagnóstico , Epilepsias Parciais/epidemiologia , Epilepsias Parciais/fisiopatologia , Epilepsia Rolândica/diagnóstico , Epilepsia Rolândica/epidemiologia , Epilepsia Rolândica/fisiopatologia , Feminino , Seguimentos , Humanos , Masculino , Transtornos de Enxaqueca/diagnóstico , Transtornos de Enxaqueca/epidemiologia , Transtornos de Enxaqueca/fisiopatologia , Lobo Occipital/fisiopatologia , Prognóstico , Estudos Retrospectivos , Sono/fisiologia , Síndrome , Vigília/fisiologiaRESUMO
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.
Assuntos
Epilepsias Parciais/diagnóstico , Idade de Início , Doenças do Sistema Nervoso Autônomo/diagnóstico , Doenças do Sistema Nervoso Autônomo/fisiopatologia , Córtex Cerebral/fisiopatologia , Criança , Pré-Escolar , Ritmo Circadiano/fisiologia , Comorbidade , Eletroencefalografia/estatística & dados numéricos , Epilepsias Parciais/epidemiologia , Epilepsias Parciais/fisiopatologia , Epilepsia Rolândica/diagnóstico , Epilepsia Rolândica/epidemiologia , Epilepsia Rolândica/fisiopatologia , Epônimos , Feminino , Seguimentos , Humanos , Lactente , Masculino , Prognóstico , Estudos Prospectivos , Convulsões/diagnóstico , Convulsões/epidemiologia , Convulsões/fisiopatologia , Sono/fisiologia , Estado Epiléptico/diagnóstico , Estado Epiléptico/fisiopatologia , Síndrome , Vigília/fisiologiaRESUMO
PURPOSE: To emphasize that, in some patients, different atypical evolutions occur in the course of so-called benign focal epilepsies of childhood (BFEC) and to promote interest in finding clinical and/or electroencephalographic (EEG) clues to which patients might be prone to these risky evolutions. METHODS: Twenty-six patients who started with the typical clinical and EEG features of benign childhood epilepsy with centrotemporal spikes (BCECTS) but who had reversible or persistent, serious epileptic events including status epilepticus and language, cognitive, or behavioral impairments were followed for