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JAMA Neurol. 2014 Jul 1;71(7):896-900. doi: 10.1001/jamaneurol.2014.463.

Effect of rituximab in patients with leucine-rich, glioma-inactivated 1 antibody-associated encephalopathy.

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  • 1Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, England2Multiple Sclerosis and Neuroinflammation Center, Department of Neurology, University of California, San Francisco.
  • 2Multiple Sclerosis and Neuroinflammation Center, Department of Neurology, University of California, San Francisco.
  • 3Memory and Aging Center, Department of Neurology, University of California, San Francisco.
  • 4Department of Neurology, University of California, San Francisco.



This observational study describes the efficacy and safety of rituximab in 5 patients with voltage-gated potassium channel (VGKC)-complex/leucine-rich, glioma-inactivated 1 (LGI1) antibody-associated encephalopathy. Rituximab is a monoclonal antibody that targets CD20 and is used to treat other neurologic and nonneurologic diseases.


This case series reports sequential seizure frequencies, modified Rankin Scale scores, and VGKC-complex antibody titers in 5 adult patients (median age, 65 years; range, 48-73 years) treated with rituximab. Median time from symptom onset to rituximab initiation was 414 days (range, 312-851 days). One patient showed a rapid clinical improvement after treatment with rituximab alone and experienced a rituximab-responsive clinical relapse. Another showed possible improvement on neuropsychometric memory indexes after rituximab therapy. In contrast, all patients showed robust responses to treatment with glucocorticoids, intravenous immunoglobulins, and/or plasma exchange at some point in their illness. Treatment with glucocorticoids-less so with intravenous immunoglobulins and plasma exchange-was associated with the most marked reductions in VGKC-complex antibodies. The only patient who did not receive glucocorticoids showed the poorest clinical and serologic responses.


Rituximab was well tolerated in this predominantly older adult patient population and may be an effective option for some patients with LGI1 antibody-associated encephalopathy. Glucocorticoid therapy appears particularly efficacious. Earlier rituximab administration and randomized trials are required to formally assess efficacy.

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