-The objective of this study was to evaluate the safety and efficacy of clobazam in children with refractory focal epilepsy. We investigated 100 consecutive patients concerning etiology of epilepsy, previously used antiepileptic drugs, seizure frequency and adverse events. Clobazam was introduced as add-on therapy in patients with previous failure of at least two monotherapies. Mean age was eight yearsold and 39 patients were girls. Clobazam mean dosage was 23.6 mg/day. Mean use of clobazam was 18.6 months. Twenty-two patients had adverse events. Twenty-six patients became seizure-free, 11 had an improvement of >75% and in 58 there was no modification in seizure frequency. Five patients had an increase in seizure frequency. Clobazam efficacy lasted for more than one year in 42% of the seizure-free patients. Clobazam seems to be safe and effective in the treatment of focal epilepsy in childhood and should be considered in patients with refractory seizures.KEY WORDS: clobazam, focal epilepsy, childhood. Eficácia do clobazam como terapia adjuvante em crianças com epilepsia focal refratáriaRESUMO -O objetivo deste estudo foi avaliar a segurança e eficácia do clobazam em crianças com epilepsia focal refratária. Nós investigamos 100 pacientes consecutivos em relação à etiologia da epilepsia, uso prévio de drogas anti-epilépticas, freqüência de crises e eventos adversos. Clobazam foi introduzido como terapia adjuvante em pacientes que não responderam a pelo menos duas monoterapias. A idade média foi 8 anos e 39 pacientes eram do sexo feminino. A dose média de clobazam foi 23,6 mg/dia. O uso médio de clobazam foi por 18,6 meses. Vinte e dois pacientes tiveram eventos adversos. Vinte e seis pacientes tornaram-se livres de crises, 11 tiveram melhora > 75% e em 58 não houve modificação na freqüência de crises. Cinco pacientes tiveram aumento na freqüência de crises. A eficácia do clobazam permaneceu por mais de um ano em 42% dos pacientes sem crises. Clobazam parece ser seguro e eficaz no tratamento de epilepsia focal na infância e deve ser considerado em pacientes com crises refratárias. PALAVRAS-CHAVE: clobazam, epilepsia focal, infância.

AbSTRACT:SIRPIDs, an acronym for stimulus-induced rhythmic, periodic, or ictal discharges, were first named in 2004. This is a pattern observed in continuous electroencephalogram (CEEG) consistently elicited by stimulation in comatose patients. The pathophysiology of SIRPIDs probably involves dysregulation of subcortico-cortical projections, particularly thalamocortical circuit, in a markedly abnormal brain with hyperexci table cortex. This may explain some studies found an association of prolonged periodic epileptiform discharges (PEDs) activity and a higher incidence of concurrent electrographic seizures and SIRPIDs. An association of SIRPIDs and poor prognosis has already been described. However, it is not yet possible to assert whether these discharges can cause neuronal injury or if they are simply a marker of severe brain injury. Objective of this paper is to review clinical relevance and pathophysiology of SIRPIDs, as well as its role as a brain response in the critically ill patient.

This article aims to expand the understanding of how it is possible to alleviate suffering and enable a dignified life trajectory for patients with progressive neurological diseases or with severe and permanent neurological impairment. The four most common disease trajectories described for people with chronic and progressive disease used to advance care planning, Brazilian normative ethical resolutions, evidence-based benefits of palliative care (PC), as well as particularities of PC in neurology, such as neurological symptom control, caring for existential and psychological suffering, care provider’s needs and particularities of pediatric neurologic PC are reviewed.

Introdução: A hérnia diafragmática congênita (HDC) causa hipoplasia e hipertensão pulmonar e em geral leva a alta morbidade e mortalidade neonatal. Traqueo-oclusão fetal (TO) e corticoterapia pré-natal são alternativas para acelerar o crescimento pulmonar fetal e diminuir a hipoplasia na HDC. A produção de VEGF (Vascular Endothelial Growth Factor) está relacionada com a maturidade pulmonar e sofre alterações na HDC ainda não elucidadas. Materiais e métodos: Seis grupos de 12 fetos de ratos Spreague-Dawley foram comparados: TO, Sham, Controle, TO+Dex, Sham+Dex e Controle+Dex. No dia 18,5º foi realizada TO com e sem corticoterapia utilizando dexametasona. No 21,5º dia gestacional os pesos corporal e pulmonar foram mensurados. Realizou-se imunohistoquímica para VEGFR-1 (Flt-1), VEGFR-2 (Flk-1), seguida de morfometria. Resultados: O VEGFR-1 estava aumentado no TO (p<0.05)e na TO+Dex (p<0.05). O VEGFR-2 teve aumento significativo quando comparamos TO e TO+Dex com o controle (p<0,05). Conclusão: A TO associada ao uso de corticoesteróide aumentou o número de VEGFR-1 e VEGFR-2 em pulmões de fetos de ratas.