RESUMO
PURPOSE: A limited number of cases of adult-onset Rasmussen's encephalitis (A-RE) have been reported, but the features of the syndrome are still unclear. The aim of this study was to verify the clinical features of A-RE, and outline a noninvasive approach that may allow its early diagnosis and treatment. METHODS: Retrospective evaluation of extensive noninvasive work-up of seven patients with A-RE, including repeat clinical, neurophysiological, and neuroimaging investigations. RESULTS: The study identified two distinct patterns of disease presentation, one characterized by focal motor epilepsy (the "epileptic" phenotype), and the other by focal cortical myoclonus (the "myoclonic" phenotype). Unilateral neurological deficits and brain atrophy were progressive in both phenotypes, but they were more prominent and were detected earlier in the "epileptic" phenotype. CONCLUSIONS: The anatomo-electroclinical features of these patients allowed a noninvasive diagnosis of A-RE and identification of two distinct disease phenotypes. Early noninvasive diagnosis can allow faster initiation of treatment.
Assuntos
Encefalite/diagnóstico , Adulto , Idade de Início , Atrofia , Encéfalo/patologia , Diagnóstico Diferencial , Eletroencefalografia/estatística & dados numéricos , Eletromiografia , Encefalite/classificação , Encefalite/genética , Epilepsia Parcial Contínua/classificação , Epilepsia Parcial Contínua/diagnóstico , Epilepsia Parcial Contínua/genética , Epilepsias Parciais/diagnóstico , Potenciais Somatossensoriais Evocados , Feminino , Fluordesoxiglucose F18 , Lateralidade Funcional/fisiologia , Humanos , Imageamento por Ressonância Magnética/estatística & dados numéricos , Pessoa de Meia-Idade , Fenótipo , Tomografia por Emissão de Pósitrons , Estudos RetrospectivosRESUMO
OBJECTIVES: Epilepsia partialis continua (Kojewnikow's syndrome) can be classified into 2 groups. The 1st group had stable neurological deficit, and the 2nd group had slowly progressive neurological deficit. The latter usually manifests not tetraplegia, but hemiplegia. We describe 3 patients with epilepsia partialis continua, rapid neurological and mental deterioration, resulting in tetraplegia and serious mental deficits within 2 to 3 years from the onset. RESULTS: Their interictal EEGs showed progressive findings of deterioration, which resulted in an inactive pattern or a pattern reminiscent of suppression-burst within several years. Their cranial CTs revealed rapid progressive atrophy of both hemispheres. Various screening tests failed to confirm a metabolic disease, a degenerative disease, or an infectious disease in the central nervous system. CONCLUSION: Frequent status epilepticus might contribute to the bilateral brain involvement and the serious neurological and mental outcomes in young patients with epileptia partialis continua.
