RESUMO
Epileptic spasms were defined by the International League Against Epilepsy Task Force on Classification and Terminology in 2001 as a specific seizure type. Epileptic spasms without hypsarrhythmia have been described in some series of patients, occurring either in infancy or childhood. More prolonged epileptic spasms without hypsarrhythmia were previously defined as a different seizure type, and referred to as "tonic spasm seizures". Here, we present a 5-year-old boy who started having epileptic spasms without hypsarrhythmia at 8 months of age, effectively treated with oxcarbazepine. With the withdrawal of medication, epileptic spasms returned. Video-EEG monitoring revealed high-voltage slow waves superimposed by low-voltage fast activity, followed by an electrodecremental phase and a burst of asymmetric fast activity, time-locked to clinical tonic spasm seizures. Brain MRI showed left temporal atrophy with temporal pole grey/white matter junction blurring and ictal PET-CT showed left basal frontal hypermetabolism. Seizures were refractory to several AEDs and vigabatrin was introduced with seizure cessation. Despite efforts to classify epileptic spasms, these are still considered as part of the group of unknown seizure types. In some cases, a focal origin has been suggested, leading to the term "periodic spasms" and "focal spasms". In this case, epileptic spasms without hypsarrhythmia, associated with tonic spasms, may be a variant of focal spasms and might be considered as an epileptic syndrome. [Published with video sequence].
Assuntos
Epilepsias Parciais/fisiopatologia , Espasmos Infantis/fisiopatologia , Anticonvulsivantes/administração & dosagem , Anticonvulsivantes/farmacologia , Carbamazepina/administração & dosagem , Carbamazepina/análogos & derivados , Carbamazepina/farmacologia , Pré-Escolar , Epilepsias Parciais/classificação , Epilepsias Parciais/tratamento farmacológico , Epilepsias Parciais/patologia , Humanos , Lactente , Masculino , Oxcarbazepina , Espasmos Infantis/classificação , Espasmos Infantis/tratamento farmacológico , Espasmos Infantis/patologia , Vigabatrina/administração & dosagemRESUMO
PURPOSE: A national study was performed in Colombia to determine the general and regional prevalence of epilepsy, clinical profiles, seizure types, and clinical syndromes. METHODS: Based on the National Epidemiological Study of Neurological Diseases (EPINEURO), we evaluated and followed up for 1 year all the subjects with epilepsy from the National Sample. Clinical profiles were further assessed. Seizure types and epilepsy syndromes were established according to the international classifications. RESULTS: General prevalence was found to be 11.3 per 1,000, with little variation among regions, except the eastern region, where prevalence was 23 per 1,000; prevalence for active epilepsy was 10.1 per 1,000. Women have a slightly greater (not statistically significant) risk. Most seizures are focal (partial), frequently with secondary generalization. The most frequent epilepsy syndrome encountered was partial symptomatic/cryptogenic (80%). Epilepsy onset in Colombia occurs most frequently in childhood. CONCLUSIONS: Prevalence rates of epilepsy in Colombia are similar to those reported in nations with comparable developmental status and have diminished over time. The study presents the distribution of seizures and syndromes. The most frequent types are focal syndromes.
Assuntos
Epilepsia/epidemiologia , Convulsões/epidemiologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Colômbia/epidemiologia , Epilepsias Parciais/classificação , Epilepsias Parciais/diagnóstico , Epilepsias Parciais/epidemiologia , Epilepsia/classificação , Epilepsia/diagnóstico , Feminino , Seguimentos , Humanos , Masculino , Programas de Rastreamento , Pessoa de Meia-Idade , Prevalência , Fatores de Risco , Convulsões/classificação , Convulsões/diagnóstico , Fatores Sexuais , SíndromeRESUMO
PURPOSE: To characterize the clinical and EEG findings and evolution of the syndrome of benign focal seizures of adolescence (BFSA), as described by Loiseau et al. METHODS: A prospective study was performed in adolescents with normal clinical and neurologic examinations and normal neuroradiologic studies who had focal seizures that occurred isolated or in a cluster, with or without secondary generalization in the first 24 to 48 h after onset. None of the patients was treated with antiepileptic drugs (AEDs). RESULTS: Between January 1996 and January 2002, 15 patients with BFSA were enrolled in the study. Median age at onset of BFSA was 14 years. Thirteen patients had focal sensory or motor seizures. In two patients, the ictal manifestation was motion arrest associated with oral automatisms. Eight of them evolved to generalized tonic-clonic seizures. Thirteen patients had seizures only when awake, and the other two, both when awake and during sleep. Repeated interictal EEGs were normal, but in four of the patients who had seizures in a cluster, we were able to record an EEG within 8 h after seizure onset. Two of these four patients had focal seizures, and their waking EEG showed focal centroparietal theta activities. The other two patients had secondarily generalized seizures, and their waking EEG showed bilateral theta activities instead. Prognosis was excellent. CONCLUSIONS: BFSA is a well-defined seizure syndrome, recognizable by clinical and EEG features, as described by Loiseau et al. In teenagers with these electroclinical features with a normal neurologic examination and normal neuroradiologic findings, AEDs should be avoided.
Assuntos
Epilepsias Parciais/classificação , Epilepsias Parciais/diagnóstico , Adolescente , Fatores Etários , Idade de Início , Córtex Cerebral/fisiopatologia , Ritmo Circadiano/fisiologia , Eletroencefalografia/estatística & dados numéricos , Epilepsias Parciais/fisiopatologia , Epilepsia Parcial Complexa/classificação , Epilepsia Parcial Complexa/diagnóstico , Epilepsia Parcial Complexa/fisiopatologia , Epilepsia Generalizada/classificação , Epilepsia Generalizada/diagnóstico , Epilepsia Generalizada/fisiopatologia , Feminino , Humanos , Masculino , Prognóstico , Estudos Prospectivos , Sono/fisiologia , Vigília/fisiologiaRESUMO
INTRODUCTION. The last proposal for a classification of epileptic seizures and syndromes of the International League Against Epilepsy (ILAE) includes recognition of several groups of epileptic syndromes and among them one of idiopathic focal epilepsies of infancy and childhood, and another of familial (autosomal dominant) focal epilepsies. The syndromes here described belong to these two groups and are: benign familial infantile seizures, benign infantile seizures (nonfamilial), benign childhood epilepsy with centrotemporal spikes (BCECTS), idiopathic occipital epilepsy of childhood Gastaut type (IOEC) and benign occipital epilepsy of childhood Panayiotopoulos type (BOEC). Atypical evolutions of the cases with BCECTS, IOEC and BOEC diagnosed and followed in our group are described, showing that these conditions are not always so benign. Finally, the so called benign focal convulsions of adolescence are considered, even when they were not included in ILAE s last proposal.
Assuntos
Epilepsias Parciais/classificação , Adolescente , Criança , Pré-Escolar , Diagnóstico Diferencial , Eletroencefalografia , Epilepsias Parciais/diagnóstico , Epilepsias Parciais/terapia , Humanos , LactenteRESUMO
OBJECTIVE: In this paper we wish to review the main characteristics of idiopathic partial epilepsy syndromes recognized in the International Classification of epilepsies and epileptic syndromes and of other syndromes which have been described but not yet included in this classification, with special emphasis on the clinical condition, electroencephalographic aspects and progress. DEVELOPMENT: The idiopathic partial epileptic syndromes are characterized by the absence of a subjacent cerebral lesion and neurological or intellectual deficit. In most cases there is a definite genetic predisposition. There is often a family history of benign epilepsy. The seizures usually start after the age of 18 months. The interictal EEG shows basically normal activity but focal epileptic discharges are often seen. CONCLUSIONS: There are many idiopathic partial epileptic syndromes. However, most of them are not included in the International Classification of the epilepsies and epileptic syndromes. We believe that further study is necessary and consider that when more cases are reported and with continual advance of genetic studies there will be a consequent improvement in understanding of these syndromes and they will be included in the International Classification of epilepsies and epileptic syndromes.
Assuntos
Epilepsias Parciais/classificação , Epilepsias Parciais/genética , Epilepsias Parciais/fisiopatologia , Predisposição Genética para Doença , Humanos , SíndromeRESUMO
La epilepsia rolándica pertenece al grupo de síndromes epilépticos primarios, parciales benignos de la infancia, se caracteriza por tener un inicio entre los dos y los 13 años de edad; se presemnta en niños normales, cuya manifestación usual es una crisis parcial durante el sueño que puede llegar a generalizar. El hallazgo característico a nivel del elctroencefalograma está dado por descargas paroxísticas de punta-onda en la región centrotemporal. Este tipo de epilepsia remite espontáneamente antes de la edad adulta (1,2). Se ha visto que este desorden se presenta en un 15 por ciento a un 20 por ciento de los pacientes con epilepsia. Su presentación es más común en el sexo masculino con una relación 2:1, aunque hay autores que hablan de una relación 3:1. La edad de inicio está entre los dos y los 13 años, siendo su máxima aparición entre los 9 y los 10 años de edad. Las convulsiones casi siempre desaparecen espontáneamente después de los 16 años de edad (3,4). El propósito de esta revisión fue evaluar las diferentes manifestaciones clínicas, evolución y manejo que se realiza en este tipo de crisis, practicando un estudio descriptivo entre 1987 y 1994 de pacientes con diagnóstico de epilepsia rolándica en el servicio de neuropediatría del Hospital Militar Central
Assuntos
Humanos , Adolescente , Epilepsias Parciais/classificação , Epilepsias Parciais/congênito , Epilepsias Parciais/tratamento farmacológico , Epilepsias Parciais/etiologia , Epilepsias Parciais/enfermagem , Epilepsias Parciais/prevenção & controleRESUMO
To describe the epidemiological characteristics of epilepsy in a northern area of Chile, an investigation was conducted in four localities in the province of Copiapó (population of 17,694). Based on 314 cases of active epilepsy, the prevalence per 1000 at June 30, 1988 was 17.7. The average annual incidence for the period 1984-1988 was 113 per 100,000. Partial seizures were the most frequent type of seizure diagnosed (54.1%). Antecedents considered as possible etiological factors were found in 29.9% of cases. According to age of onset, 64.6% had their first attack before 15 years. Middle and low socioeconomic classes had higher prevalence rates of epilepsy. We compare our results with previous Latin-American studies.
Assuntos
Países em Desenvolvimento , Epilepsia/epidemiologia , Adolescente , Adulto , Criança , Pré-Escolar , Chile/epidemiologia , Estudos Transversais , Epilepsias Parciais/classificação , Epilepsias Parciais/diagnóstico , Epilepsias Parciais/epidemiologia , Epilepsia/classificação , Epilepsia/diagnóstico , Epilepsia Generalizada/classificação , Epilepsia Generalizada/diagnóstico , Epilepsia Generalizada/epidemiologia , Feminino , Humanos , Incidência , Lactente , Masculino , Pessoa de Meia-IdadeRESUMO
Intermittent rhythmic delta activity is reported in various disorders and is classified as a nonspecific abnormal electroencephalographic pattern. We have investigated its clinical and electroencephalographic features in childhood. Intermittent rhythmic delta activity was identified in 54 children over a period of 48 months. Epilepsy was present in 81%, 4% had only a single generalized tonic-clonic seizure, and 15% had no seizures. Generalized seizures were more common than partial seizures (83% versus 13%; 4% were mixed). The largest group of patients had idiopathic epilepsy. Epileptiform features were present in 70%. No patient identified prospectively has had a space-occupying lesion. Intermittent rhythmic delta activity should be considered an epileptiform pattern in children, most commonly occurring as an interictal pattern in primary generalized epilepsy.