Rasmussen’s syndrome is a rare inflammatory brain disease characterized by severe intractable epilepsy and unilateral progressive motor deficit associated with contralateral hemispheric atrophy. It usually affects children although there are occasional reports of adult-onset.
This paper reports four patients in whom seizures began in adolescence or adulthood with clinical and radiological symptoms suggesting Rasmussen’s syndrome. The patients are compared with 33 cases found from the literature between 1987 and 2004.
Adult-onset Rasmussen’s can mimic the early-onset form but symptoms often progress more slowly and the associated neurological deficits are more variable. Occipital lobe involvement is more common than in the childhood form as are atypical features such as bilateral hemispheric involvement, temporal lobe epilepsy and movement disorders at onset. Surgical hemispheric disconnection, an effective treatment for controlling seizures in childhood, is not indicated for adults for obvious functional reasons. Based on recent pathogenetic concepts, several approaches to medical treatment can be proposed.
© 2007 Elsevier Masson SAS. Tous droits réservés.