Clinical heterogeneity and treatment optimization in anti-KLHL11 encephalitis: two case reports and literature review

01 Jun 20264 min read0 viewsJournal Feed

GIST (Key Takeaways)

Objective. This study aimed to investigate the clinical features, differential treatment responses, and management strategies of encephalitis associated with anti-Kelch-like protein 11 (KLHL11) antibodies. Methods.
We report two cases of anti-KLHL11 encephalitis that improved following immunotherapy and present a literature review of cases published in the last six years. Results. Case 1 involved a 52-year-old woman who tested positive for serum anti-KLHL11 antibodies at a 1:100 titer.
The patient completely recovered following a treatment regimen comprising intravenous immunoglobulin administration at 400 mg/kg/day for 5 days, combined with low-dose rituximab (100 mg, weekly for three consecutive weeks). Extensive tumor screening revealed no underlying malignancies. In Case 2, a 65-year-old man tested positive for anti-KLHL11 antibodies in both serum (titer 1:320) and cerebrospinal fluid (titer 1:100).
Initial high-dose intravenous glucocorticoid pulse therapy, starting at 1000 mg dose and tapered by halving the dose every 3 days until discontinuation, combined with low-dose rituximab (100 mg weekly for 3 consecutive weeks), resulted in a suboptimal response, with symptoms recurring after one month. Subsequent administration of efgartigimod (10 mg/kg) led to marked clinical improvement.