ABSTRACT
Resumen Este artículo no tiene como objetivo el presentar una descripción detallada de cada una de las encefalopatías epilépticas y del desarrollo, sino más bien discutir cam bios recientes en la terminología y criterios diagnósticos de ciertas encefalopatías, en base a una revisión actua lizada de los últimos 10 años. Se analizan cambios importantes en definiciones de síndromes específicos y nuevos tratamientos que han demostrado eficacia en el manejo de crisis convulsivas en estos pacientes. En conclusión: Las nuevas terapias de modulación genética, contribuirán no solo a reducir la carga de crisis epilépticas, sino también a mejorar el pronóstico cognitivo, y por lo tanto la calidad de vida.
Abstract It is not the intend of this article to present a de tailed description of each developmental and epileptic encephalopathy, but to discuss recent changes in the terminology and diagnostic criteria of specific disorders, based on an updated review of the last 10 years. Important changes in the definitions of specific syn dromes and new treatments that have shown efficacy in the management of seizures in these patients are analyzed. In conclusion: New gene modulation therapy will likely improve not only seizure frequency, but also cog nitive outcome and therefore quality of life.
ABSTRACT
Absence seizures (AS) are generalized non-convulsive seizures characterized by a brief loss of consciousness and spike-and-wave discharges (SWD) in an electroencephalogram (EEG). A number of animal models have been developed to explain the mechanisms of AS, and thalamo-cortical networks are considered to be involved. However, the cortical foci have not been well described in mouse models of AS. This study aims to use a high density EEG in pathophysiologically different AS models to compare the spatiotemporal patterns of SWDs. We used two AS models: a pharmacologically induced model (gamma-hydroxybutyric acid, GHB model) and a transgenic model (phospholipase beta4 knock-out, PLCβ4 model). The occurrences of SWDs were confirmed by thalamic recordings. The topographical analysis of SWDs showed that the onset and propagation patterns were markedly distinguishable between the two models. In the PLCβ4 model, the foci were located within the somatosensory cortex followed by propagation to the frontal cortex, whereas in the GHB model, a majority of SWDs was initiated in the prefrontal cortex followed by propagation to the posterior cortex. In addition, in the GHB model, foci were also observed in other cortical areas. This observation indicates that different cortical networks are involved in the generation of SWDs across the two models.
Subject(s)
Animals , Mice , Electroencephalography , Epilepsy, Absence , Frontal Lobe , Models, Animal , Prefrontal Cortex , Seizures , Somatosensory Cortex , UnconsciousnessABSTRACT
Objective To investigate the clinical effect of large dosage of Methylprednisolone on epilespy combined with electrical status epilepticus during sleep(ESES) in children.Methods Forty-six epielpsy patients with ESES were treated with additive large dosage of Methylprednisolone.The seizures and video electroencephalogram discharges were observed before and after using Methylprednisolone.The Methylprednisolone effect in eliminating the methylprednisolone of ESES and controlling of clinical seizures and improving cognitive function were analyzed.Results Two cases were lost and 44 cases were included,male 30,female 14,the age at onset was (5.37 ±2.52) (2-9) years old.The duration of follow-up was (4.12 ± 2.00)(1-9) years old.The age at diagnosis of ESES was (8.17 ± 2.09) (4.0-12.5) years old.The efficacy of Methylprednisolone on seizures was 72.7％ (32/44 cases),while the efficacy of Methyl prednisolone on electroencephalograph (EEG) was 59.1％ (26/44 cases).For patients who were resistant to Levetiracetam or Clonazepam,Methylprednisolone was still effective.Intelligence quotient had no significant changes before and after treatment(P ＞ 0.05).The earlier onset age,the worse effect of Methylprednisolone.The efficacy of methylprednisolone for atypical benign epilepsy with cento-temporal spike(BECT) was higher than other syndromes.Conclusions Large dosage of Methyl prednisolone therapy for children with ESES,especially for those resistant to traditional or new antiepileptic drug for ESES,was effective and safe.The onset age and syndrome classification may have a certain value for prognosis and prediction of the effect of Methylprednisolone treatment.
ABSTRACT
Possible interactions between the sleep-wakefulness cycle and a new kind of spontaneous epilepsy, expressed as absence-like seizures and spike-wave bursts in FMUSP rats, are evaluated. The electro-oscillograms of some cortical and subcortical regions of the brain were recorded, as well as head, rostrum/vibrissae and eye movements. Recordings were performed uninterruptedly during 24 hours. The seizures were mostly concentrated in the wakefulness state but they could occur in any other phase, including paradoxical sleep. After the seizure, the rats usually returned to the same phase that was interrupted, although they often returned to wakefulness. There was an intense fragmentation of the sleep-wakefulness cycle. The incidence of each cycle phase was significantly reduced, except S III of synchronized sleep and paradoxical sleep, thus maintaining the overall duration and architecture of the sleep -wakefulness cycle. The fragmentation of the cycle seems to be due to an impairment of the very processes that generate sleep and wakefulness. Electrophysiological and behavioral profiles of the FMUSP rats recommend accurate and comprehensive study of the animal model owing to its resemblance to seizures in humans and also to discrepancies with existing genetic or experimental epilepsy models.
A razão principal desta investigação foi estudar a arquitetura do ciclo vigília-sono numa cepa de ratos Wistar (FMUSP-rats) portadores de epilepsia espontânea tipo ausência. Foram utilizados 10 ratos Wistar adultos, que receberam eletrodos em regiões corticais e subcorticais, nos músculos trapézios e nos epicantos oculares, pelos quais registramos os eletroscilogramas continuamente por 24 horas, dos quais foram analisados os registros eletroscilográficos e demais parâmetros da arquitetura do ciclo vigília-sono. As crises ocorriam preferencialmente durante o período escuro, coincidindo com a maior prevalência de estados de vigília. O ciclo vigília-sono sofreu intensa fragmentação nos ratos epilépticos, e a duração média de algumas fases do sono foi mais prolongada nos ratos epilépticos do que nos sadios. As manifestações eletrofisiológicas das crises assumiram várias formas, predominando, porém, os complexos espícula-onda (de 7 a 9,5 Hz) o que se assemelha muito à faixa de oscilação das ondas teta. As características eletrofisiológicas e comportamentais da epilepsia que estudamos recomendam o estudo acurado e abrangente desse modelo de síndrome epiléptica, por sua semelhança com as crises encontradas em humanos, mas também por algumas discrepâncias em relação a modelos de epilepsia genética ou experimental já existentes.
Subject(s)
Rats , Epilepsy , Seizures , Sleep , WakefulnessABSTRACT
Introducción. El síndrome de Landau-Kleffner se caracteriza por afasia adquirida y anormalidades electroencefalográficas durante la vigilia y el sueño. El tratamiento con anticonvulsivos controla las crisis convulsivas pero en los problemas de lenguaje y comportamiento su eficacia es menor. Algunos reportes señalan mejoría de lenguaje con el uso de corticoesteroides en etapas tempranas y a dosis altas. Otra opción terapéutica es el uso de calcioantagonistas. Caso clínico. Paciente de 5 años de edad con evolución normal hasta los 2 años hasta que los padres observaron la pérdida espontánea del lenguaje previamente adquirido. En la evaluación neurológica se encontraron abundantes ecolalias e indiferencia a estímulos externos. El electroencefalograma mostró brotes intermitentes de ondas agudas y complejos punta-onda lenta de 3-4 Hz generalizados durante el sueño. Se inició tratamiento con prednisona por un mes y ácido valproico. Se continuó con el ácido valproico y después de 4 meses se añadió flunarizina, con lo que se observó mejoría en el lenguaje. Conclusiones. Este caso presenta los hallazgos clínicos y electroencefalográficos del síndrome; se observó que la mejor respuesta al tratamiento se obtuvo al agregar flunarizina. Esta evidencia contribuye a apoyar su uso y fundamenta la realización posterior de estudios controlados para concluir certeramente sobre su utilidad en el padecimiento.
Background. Landau-Kleffner syndrome is characterized by acquired aphasia and electroencephalographic abnormalities during wake-fulness and sleep. These abnormalities can be solved with anticonvulsive medications, but speech and behavioral problems cannot be treated using this therapy. Instead, there are reports that indicate that treatment with high-dose corticosteroids during early stages of the disease improves the speech difficulties. Use of calcium antagonists has also been proposed as possible treatment. Case report. We report the case of a 5-year-old patient with normal development until the age of 2 years. At that time, the parents observed loss of spontaneous acquired speech. During neurological evaluation, the child showed abundant echolalia and indifference to external stimuli. Electroencephalogram showed sharp waves and generalized slow spike-wave complexes of 3-4 Hz during sleep. We began treatment with prednisone and valproic acid for 1 month; flunarizine was added. After 4 months of treatment, the patient showed speech improvement. Conclusions. Our case has the characteristic clinical and electroencephalographic findings of Landau-Kleffner syndrome. We observed significant symptom improvement when flunarizine was added to the treatment. This evidence offers support for the use of a calcium antagonist as possible therapy, which may help setting the way for future controlled studies in order to finally establish its utility with this illness.
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PURPOSE: This study was undertaken to determine the incidence and the meaning of epileptiform discharges accompanied by chronic recurrent headaches. METHODS: We selected 449 cases of children who visited Dong-A University Hospital because of recurrent headaches from January 1999 to July 2005. The headaches were classified by the criteria established by the International Headache Research Committee in 2004. The electroencephalography was performed in 336 cases and 36 cases who showed epileptiform discharges were selected. We studied the characteristics of epileptiform discharges, MRI findings and the relationship with epilepsy. RESULTS: The incidence of interictal epileptiform discharges(ILEDs) of the patients with chronic headaches was 10.7%, which was higher than that in a normal population. In the location of ILEDs, focal areas(9.2%) were more common than general areas(1.5%). The incidence of the ILEDs was different according to the types of headaches(P<0.01). The focal ILEDs were concentrated at the central temporal areas. The most common type of ILEDs was frequent epileptiform discharges(63.9%). For the wave forms of ILEDs, focal spike activities were 83.3%, and bursts of slow waves mixed with spikes were 13.9%. Among the 36 cases that showed the ILEDs, 2 cases were associated with epilepsy and another 2 with AV malformation on the MRI images. CONCLUSION: The ILEDs of the patients with chronic recurrent headaches mainly occurred in the central temporal areas as focal spike wave forms, which shows a benign course. Because a few cases can accompany epilepsy and brain abnormalities, close observation and follow-up tests are needed.