Autoimmune Epilepsy: Clinical Characteristics and Response to Immunotherapy
JAMA Neurology, 04/06/2012
Clinical Article
Quek AML et al. – When clinical and serological clues suggest an autoimmune basis for medically intractable epilepsy, early–initiated immunotherapy may improve seizure outcome.
Methods- 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.
- Immunotherapy with intravenous methylprednisolone; intravenous immune globulin; and combinations of intravenous methylprednisolone, intravenous immune globulin, plasmapheresis, or cyclophosphamide.
- Seizure frequency.
- 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.
