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

Neurology. 2014 May 6;82(18):1578-86. doi: 10.1212/WNL.0000000000000383. Epub 2014 Apr 4.


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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Autoantibodies
  • Child
  • Child, Preschool
  • Electroencephalography
  • Epilepsy / cerebrospinal fluid
  • Epilepsy / immunology*
  • Epilepsy / therapy*
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use*
  • Immunotherapy / methods*
  • Magnetic Resonance Imaging
  • Male
  • Methylprednisolone / therapeutic use*
  • Middle Aged
  • Nerve Tissue Proteins / immunology
  • Neuroprotective Agents / therapeutic use*
  • Retrospective Studies
  • Treatment Outcome
  • Young Adult


  • Autoantibodies
  • Immunoglobulins, Intravenous
  • Nerve Tissue Proteins
  • Neuroprotective Agents
  • Methylprednisolone