Ten of 17 patients with Rasmussen’s syndrome receiving IV methylprednisolone and/or oral predisolone, and eight of nine patients receiving immunoglobulins showed some short-term reduction in seizure frequency in a multicenter international report. Side effects included fluid retention, psychotic symptoms and behavior problems. The authors propose a central register and standardized protocol with initial trial of IV immunoglobulin (400 mg/kg/d on 3 successive days) followed by monthly one day treatments if improvement occurs. Steroid therapy (IV methylprednisolone, 400 mg/m2 on 3 alternate days followed by monthly single infusions for one year or longer, and oral predisolone starting at 2 mg/kg/d) is recommended for patients not responding to IVIG. [1]

COMMENT. The patients were treated at the Montreal Neurological and Children’s Hospitals; the National Hospital, London; Great Ormond Street Hospital, London; Hospital for Sick Children, Toronto; and Hospital Juan Garrahan, Buenos Aires. The diagnosis was confirmed by biopsy in all but three of the patients. As with infantile spasms, the earlier the therapy the better the results. Since ACTH is usually considered superior to prednisone in the treatment of infantile spasms, it is surprising that only one of the 17 patients with Rasmussen’s syndrome received ACTH and ACTH is omitted from the recommended treatment protocol for further trials. If a comparison with infantile spasms is carried further, patients benefited by steroids or ACTH usually respond within 4 to 8 weeks and prolongation of therapy in non-responders is generally ineffective and is accompanied by serious toxicity. The authors recommend frequent monitoring of patients on high-dose steroids when continued for the suggested periods of 1 to 2 years or longer and caution that improvements may be delayed for several months.