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2014 ; 71
(7
): 896-900
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Effect of rituximab in patients with leucine-rich, glioma-inactivated 1
antibody-associated encephalopathy
#MMPMID24842754
Irani SR
; Gelfand JM
; Bettcher BM
; Singhal NS
; Geschwind MD
JAMA Neurol
2014[Jul]; 71
(7
): 896-900
PMID24842754
show ga
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.