Kelch-Like Protein 11 Antibody, IgG by CBA-IFA With Reflex to Titer, Serum

IgG antibodies to KLHL11 are associated with paraneoplastic neurologic syndromes with phenotypes most often including a combination of brainstem and cerebellar encephalitis as well as sensorineural hearing loss. Patients with anti-KLHL11 syndromes should be thoroughly evaluated for cancer, including testicular cancer, as neurologic symptoms often precede cancer diagnosis. Consider sending testing in CSF as well as serum to improve diagnostic yield. Coexisting and clinically relevant antineural antibodies have been reported; consider ordering a phenotype-specific panel to assess for these. Results (positive or negative) should be interpreted in the context of the patient's complete clinical picture, as false positives may occur, and a negative result does not exclude the diagnosis of immune-mediated neurologic disease.