Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy

作者：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^*

来源：Neurology, 2014, 82(18): 1578-1586.

DOI：10.1212/WNL.0000000000000383

Objective:To evaluate a trial of immunotherapy as an aid to diagnosis in suspected autoimmune epilepsy.Method: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.Results: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%).Conclusions:These retrospective findings justify consideration of a trial of immunotherapy in patients with suspected autoimmune epilepsy.Classification of evidence:This study provides Class IV evidence that in patients with suspected autoimmune epilepsy, IVMP, IVIg, or both improve seizure control.

• 出版日期2014-5-6