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  • Utility of an immunotherapy...
    Toledano, M; Britton, J W; McKeon, A; Shin, C; Lennon, V A; Quek, A M L; So, E; Worrell, G A; Cascino, G D; Klein, C J; Lagerlund, T D; Wirrell, E C; Nickels, K C; Pittock, S J

    Neurology, 2014-May-6, Volume: 82, Issue: 18
    Journal Article

    To evaluate a trial of immunotherapy as an aid to diagnosis in suspected autoimmune epilepsy. We reviewed the charts of 110 patients seen at our autoimmune neurology clinic with seizures as a chief complaint. Twenty-nine patients met the following inclusion criteria: (1) autoimmune epilepsy suspected based on the presence of ≥ 1 neural autoantibody (n = 23), personal or family history or physical stigmata of autoimmunity, and frequent or medically intractable seizures; and (2) initiated a 6- to 12-week trial of IV methylprednisolone (IVMP), IV immune globulin (IVIg), or both. Patients were defined as responders if there was a 50% or greater reduction in seizure frequency. Eighteen patients (62%) responded, of whom 10 (34%) became seizure-free; 52% improved with the first agent. Of those receiving a second agent after not responding to the first, 43% improved. A favorable response correlated with shorter interval between symptom onset and treatment initiation (median 9.5 vs 22 months; p = 0.048). Responders included 14/16 (87.5%) patients with antibodies to plasma membrane antigens, 2/6 (33%) patients seropositive for glutamic acid decarboxylase 65 antibodies, and 2/6 (33%) patients without detectable antibodies. Of 13 responders followed for more than 6 months after initiating long-term oral immunosuppression, response was sustained in 11 (85%). These retrospective findings justify consideration of a trial of immunotherapy in patients with suspected autoimmune epilepsy. This study provides Class IV evidence that in patients with suspected autoimmune epilepsy, IVMP, IVIg, or both improve seizure control.