Autoimmune Epilepsy Clinical Characteristics and Response to Immunotherapy

This is a very interesting article about patients treated with immunotherapy for autoimmune epilepsy.

• Online Features  This Article  • Full text  • PDF  • Author Interview  • eTables and eFigure  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Contact me when this article is cited  Related Content  • Related article  • Similar articles in this journal  Topic Collections  • Neurology  • Epilepsy  • Prognosis/ Outcomes  • Drug Therapy  • Immunologic Disorders  • Immunology, Other  • Alert me on articles by topic  Social Bookmarking          What's this? ONLINE FIRST Autoimmune EpilepsyClinical Characteristics and Response to Immunotherapy Amy M. L. Quek, MBBS; Jeffrey W. Britton, MD; Andrew McKeon, MD; Elson So, MD; Vanda A. Lennon, MD, PhD; Cheolsu Shin, MD; Christopher J. Klein, MD;Robert E. Watson Jr, MD, PhD; Amy L. Kotsenas, MD; Terrence D. Lagerlund, MD; Gregory D. Cascino, MD; Gregory A. Worrell, MD, PhD; Elaine C. Wirrell, MD;Katherine C. Nickels, MD; Allen J. Aksamit, MD; Katherine H. Noe, MD; Sean J. Pittock, MD  Arch Neurol. Published online March 26, 2012. 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. Author Affiliations: Departments of Laboratory Medicine and Pathology (Drs Quek, McKeon, Lennon, Klein, and Pittock), Neurology (Drs Britton, McKeon, So, Lennon, Shin, Klein, Lagerlund, Cascino, Worrell, Wirrell, Nickels, Aksamit, and Pittock), Immunology (Dr Lennon), and Radiology (Drs Watson and Kotsenas), Mayo Clinic, College of Medicine, Rochester, Minnesota; and Department of Neurology, Mayo Clinic, College of Medicine, Scottsdale, Arizona (Dr Noe). Abstract here