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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.

Author information

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

Abstract

IMPORTANCE:

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.

OBSERVATIONS:

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.

CONCLUSIONS AND RELEVANCE:

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.

PMID:
24842754
PMCID:
PMC4477824
DOI:
10.1001/jamaneurol.2014.463
[Indexed for MEDLINE]
Free PMC Article
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