Abstract
High serum carbamazepine-epoxide concentrations were correlated with unexpected seizure exacerbation and partial status epilepticus in 6 young adults reported from the Marshfield Clinic, WI, and the Mayo Clinic, MN.
Highlights
Steroid therapy (IV methyl¬ prednisolone, 400 mg/m^ 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
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
Summary
High serum carbamazepine-epoxide concentrations were correlated with unexpected seizure exacerbation and partial status epilepticus in 6 young adults reported from the Marshfield Clinic, WI, and the Mayo Clinic, MN. Steroid therapy (IV methyl¬ prednisolone, 400 mg/m^ 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. (Hart YM, Cortez M, Andermann F et al Medical treatment of Rasmussen's syndrome (chronic encephalitis and epilepsy): Effect of high-dose steroids or immunoglobulins in 19 patients.
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