Estado epiléptico como manifestação inicial de doença de Kawasaki / Status epilepticus as first manifestation of Kawasaki disease

A doença de Kawasaki é uma vasculite aguda de etiologia desconhecida com variações no quadro clínico. Estudos recentes têm demonstrado um aumento significativo da incidência nos últimos anos. A incidência de acometimento do SNC varia de 0,4 ­ 3,7%, incluindo convulsões, ataxia, infarto cerebral, coleção subdural, hemiplegia e paralisia do nervo facial. Neste estudo mostramos o caso de um paciente de dois anos de idade, branco, masculino, previamente hígido, com acometimento do SNC na forma de estado epiléptico antes da definição clínica da doença de Kawasaki. São relatados os desdobramentos clínicos, tanto durante a internação como durante o seguimento ambulatorial, achados laboratoriais, radiológicos e eletrofisiológicos, com a devida correlação com os dados de revisão da literatura. Conclusão: deve-se sempre pensar no diagnóstico diferencial ao acometimento súbito do SNC, na hipótese diagnóstica de doença de Kawasaki, mesmo antes da ocorrência de sinais e sintomas que compõem o quadro clássico da doença (AU)

Kawasaki disease is na acute vasculitis with unknown ethiology and large range of clinical findings. Recent studies showed higher incidence in the last years. Central nervous system is affected in 0,4 ­ 3,7% of patientes, and include seizures, ataxia, cerebral infarct, sudural effusions, hemiplegia and facial nerve palsy. In our study we show the case of a two years old male, previosly healthy, with occurrence of status epilepicus before the clinical diagnosis of Kawasaki disease. We report the clinical, laboratorial, neuroradiological and electrencephalogram findings comparing with data in medical literature. Conclusion: in the cases of sudden central nervous system involvement, it is necessary include Kawasaki disease in the differential diagnosis, even before the occurrence of classical clinical symptons (AU)

Cerebrovascular disease in pediatric patients

Although rare in childhood, stroke may have a serious impact when it happens in this stage of life. Also, it may be the first sign of a systemic disease. We report 12 cases of patients with stroke treated in the Neuropediatrics Unit of Hospital de ClÝnicas de Porto Alegre (HCPA) from March 1997 to March 2000. All patients, from term infants to 12-year-old children hospitalized in the Pediatrics Unit of HCPA, had clinical suspicion of stroke, which was later confirmed by radiological studies. Patient follow up ranged from 1 to 6 years (mean = 3.4 years). Presenting symptoms were hemiparesis in 9 patients, seizures in 7, deviation of labial commissure in 3, and loss of consciousness in 1. The increase in the number of cases of childhood stroke identified and later confirmed by noninvasive methods had helped in the determination of different ethiologies of stroke: the most frequent being hematologic, cardiac and genetic diseases. However, our study included 6 newborns with stroke whose ethiology was not identified. Seven children with seizures received phenobarbital. Six term infants had neonatal seizures secondary to stroke and restricted to the first 72 hours of life