Clinical Report: Diverse Clinical Presentation and Treatment Strategies in Anti-KLHL11 Encephalitis
Overview
This report details two cases of anti-KLHL11 encephalitis, highlighting the clinical heterogeneity and varying treatment responses. Notably, efgartigimod emerged as a promising therapeutic option for refractory cases.
Background
Anti-KLHL11 encephalitis is a recently identified autoimmune encephalitis subtype associated with paraneoplastic syndromes, particularly in males with testicular germ cell tumors. Its clinical manifestations can vary widely, complicating diagnosis and treatment. Understanding the diverse presentations and treatment responses is crucial for improving patient outcomes.
High serum anti-KLHL11 titer (1:320) may correlate with treatment refractoriness.
Immunotherapy, including IVIG and rituximab, is a cornerstone of management.
Efgartigimod demonstrated marked clinical improvement in a refractory case.
Comprehensive tumor screening is essential in managing affected patients.
Clinical Implications
Clinicians should be aware of the diverse presentations of anti-KLHL11 encephalitis and the potential for refractory cases. Early identification and treatment with immunotherapy, including novel agents like efgartigimod, may improve outcomes. Regular tumor surveillance is critical, especially in male patients.
Conclusion
The findings underscore the importance of individualized treatment strategies in anti-KLHL11 encephalitis, particularly in cases with high antibody titers. Continued research is needed to validate these observations and optimize management protocols.
