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Immunoadsorption therapy in autoimmune encephalitides
Müjgan Dogan Onugoren1, Kristin S Golombeck1, Corinna Bien1
1Epilepsy Center Bethel (M.D.O., C.B., M.B.-H., C.G.B.), Krankenhaus Mara, Bielefeld; Department of Neurology (M.D.O.), University Hospital Erlangen; Department of Neurology (K.S.G., H.L., H.W., N.M.), University of Münster; Department of Nephrology (M.A.-T., R.V.), Bethel-EvKB, Bielefeld; Department of Medicine D (M.B., H.P., G.T.), Division of General Internal Medicine, Nephrology and Rheumatology, University Hospital of Münster; and Laboratory Krone (D.M.), Bad Salzuflen, Germany.
Objective:
It was hypothesized that in encephalitides with autoantibodies directed to CNS surface antigens an antibody-removing intervention might speed up recovery.
Methods:
The outcome of autoimmune encephalitis in 19 patients with antibodies against surface antigens (leucine-rich, glioma inactivated 1 [LGI1], n = 3; contactin-associated protein-2 [CASPR2], n = 4; NMDA receptor [NMDAR], n = 7) and intracellular antigens (glutamic acid decarboxylase [GAD], n = 5) after immunoadsorption in addition to corticosteroid therapy was evaluated retrospectively. Modified Rankin scale (mRS) scores and data on seizures, memory, and antibody titers directly after immunoadsorption (early follow-up) and after a median of 4 months (late follow-up) were compiled.
Results:
Immediately after immunoadsorption, 9 of 14 patients with antibodies against LGI1, CASPR2, or NMDAR (64%), but none with GAD antibodies, had improved by at least one mRS point. Five of the 7 patients with LGI1 or CASRP2 antibodies had become seizure-free, and 2 patients with NMDAR antibodies had a memory improvement of more than 1 SD of a normal control population. At late follow-up, 12 of 14 patients with surface antibodies had improved (86%), and none of the patients with GAD antibodies.
Conclusions:
It is suggested that addition of immunoadsorption to immunosuppression therapy in patients with surface antibodies may accelerate recovery. This supports the pathogenic role of surface antibodies.
Classification Of Evidence:
This study provides Class IV evidence that immunoadsorption combined with immunosuppression therapy is effective in patients with autoimmune encephalitis with surface antibodies.
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