Autoimmune epilepsy: clinical characteristics and response to immunotherapy

Arch Neurol. 2012 May;69(5):582-93. doi: 10.1001/archneurol.2011.2985.


Objective: To describe clinical characteristics and immunotherapy responses in patients with autoimmune epilepsy.

Design: Observational, retrospective case series.

Setting: Mayo Clinic Health System.

Patients: Thirty-two patients with an exclusive (n=11) or predominant (n=21) seizure presentation in whom an autoimmune etiology was suspected (on the basis of neural autoantibody [91%], inflammatory cerebrospinal fluid [31%], or magnetic resonance imaging suggesting inflammation [63%]) were studied. All had partial seizures: 81% had failed treatment with 2 or more antiepileptic drugs and had daily seizures and 38% had seizure semiologies that were multifocal or changed with time. Head magnetic resonance imaging was normal in 15 (47%) at onset. Electroencephalogram abnormalities included interictal epileptiform discharges in 20; electrographic seizures in 15; and focal slowing in 13. Neural autoantibodies included voltage-gated potassium channel complex in 56% (leucine-rich, glioma-inactivated 1 specific, 14; contactin-associated proteinlike 2 specific, 1); glutamic acid decarboxylase 65 in 22%; collapsin response- mediator protein 5 in 6%; and Ma2, N-methyl-D-aspartate receptor, and ganglionic acetylcholine receptor in 1 patient each.

Intervention: Immunotherapy with intravenous methylprednisolone; intravenous immune globulin; and combinations of intravenous methylprednisolone, intravenous immune globulin, plasmapheresis, or cyclophosphamide.

Main outcome measure: Seizure frequency.

Results: After a median interval of 17 months (range, 3-72 months), 22 of 27 (81%) reported improvement postimmunotherapy; 18 were seizure free. The median time from seizure onset to initiating immunotherapy was 4 months for responders and 22 months for nonresponders (P<.05). All voltage-gated potassium channel complex antibody-positive patients reported initial or lasting benefit (P<.05). One voltage-gated potassium channel complex antibody-positive patient was seizure free after thyroid cancer resection; another responded to antiepileptic drug change alone.

Conclusion: When clinical and serological clues suggest an autoimmune basis for medically intractable epilepsy, early-initiated immunotherapy may improve seizure outcome.

Publication types

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

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Autoantibodies / cerebrospinal fluid
  • Brain / diagnostic imaging
  • Brain / pathology
  • Cyclophosphamide / therapeutic use
  • Electroencephalography
  • Epilepsy* / etiology
  • Epilepsy* / immunology
  • Epilepsy* / therapy
  • Female
  • Fluorodeoxyglucose F18
  • Humans
  • Immunoglobulins / therapeutic use
  • Immunologic Factors / therapeutic use
  • Immunotherapy / methods*
  • Inflammation / cerebrospinal fluid
  • Inflammation / complications*
  • Magnetic Resonance Imaging
  • Male
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Nerve Tissue Proteins / immunology
  • Plasmapheresis / methods
  • Positron-Emission Tomography
  • Retrospective Studies
  • Treatment Outcome
  • Young Adult


  • Autoantibodies
  • Immunoglobulins
  • Immunologic Factors
  • Nerve Tissue Proteins
  • Fluorodeoxyglucose F18
  • Cyclophosphamide
  • Methylprednisolone