Rituximab in Rasmussen's encephalitis: A single center experience and review of the literature.

Abstract

Rasmussen's encephalitis (RE) is a rare chronic inflammatory disease of the brain resulting in unilateral hemispheric atrophy with drug-resistant focal epilepsy associated with a variable degree of progressive hemiparesis and cognitive decline. The precise etiology of RE is unknown but presumed to have a neuroinflammatory pathobiological basis. Only surgery halts progression of the disease, but may occur at the expense of a fixed but otherwise inevitable neurological deficit. Therefore, the question of medical management is an important consideration. Reports of rituximab use in patients with RE were presented at the American Epilepsy Society annual meeting in 2008. Good published evidence for its usage has been very slow to emerge since then. However, rituximab continues to be listed in discussions of treatment options for patients with RE, though other monoclonal antibodies have since been used with comparable outcomes. We describe a series of nine patients including two with adult-onset RE. Rituximab was used early in the disease course (range 1-108months; mean 32months). Of nine patients with RE, there was significant benefit in their seizure burden with rituxamab. Seizure freedom occurred in 3 patients. Epilepsia partialis continua (EPC) was present in 4/9 and no focal motor deficit noted in 4/9. No progression of a neurological deficit was present in 2/9 and evidence of progression with neuroimaging was terminated with rituxamab in 5/9 supporting early use of rituxamab in patients with RE.