Early onset Rasmussen's syndrome: A malignant, often bilateral form of the disorder

Early onset of Rasmussen's syndrome, chronic encephalitis and epilepsy, is associated with a greater tendency to bilateral disease. A cluster of such children is described. This form has a poor prognosis and is usually fatal. This may be related to immaturity of the immune system. Bilateral involvement, as distinct from bilateral epileptic discharges, has also been described in adults but seems to have a more favourable prognosis. Isolated patients treated with immune modulating agents may also have bilateral disease but the significance of the treatment accounting for the contralateral spread requires further observations.