Rituximab and Cyclophosphamide as an alternative in immunomodulatory therapy in Rasmussen encephalitis

Introduction: Rasmussen encephalitis (RE) is a rare, autoimmune inflammation of the cerebral cortex. Cytotoxic T-lymphocytes are involved in the inflammatory process which gradually advances to cortical atrophy. RE is characterized by severe therapy resistant seizures and progressive neurological deficits. In 85% of the cases, the children are younger than 10 years of age at disease manifestation. The therapy of RE comprises corticosteroids, immunoglobulins and Tacrolimus. On failure of this treatment, hemispherectomy is conducted as a last resort therapy. The effects of Rituximab as an alternative immunomodulatory treatment were investigated in single case studies. In 9 of 10 cases betterment of the clinical symptoms was observed. We report of two children with RE who were treated with Rituximab and Cyclophosphamide.

The diagnosis of RE was confirmed in two female patients (P1 and P2) at the age of 4 (P1) and 12 (P2) years respectively. Both of them were being treated with immunoglobulins and dexamethasone over a period of 4 years. Under this treatment, the disease advanced and severe medicamentous side effects occurred. Therefore, the therapy was switched to Rituximab and low-dose cyclophosphamide. Both drugs were administered intermittently in blocks. Thereupon, a reduction in severity and frequency of epilepsy in P1 and improvement in psychomotor skills in P2 were observed.

Conclusion: By treating two juvenile patients with rituximab and cyclophosphamide, we achieved an intermittent improvement of epilepsy respectively of psychomotorics lacking side effects. However, recurrent epileptic status could not be prevented in the long term. Immunomodulatory treatment probably delays the progress of cortical atrophy. Thus, this treatment leads to slowdown of neurological functional waste which can result in reduction of severity and frequency of epilepsy and improvement of psychomotor function.