Test Catalog

Test ID: K11CC    
Kelch-Like Protein 11 Antibody, Cell Binding Assay, Spinal Fluid

Useful For Suggests clinical disorders or settings where the test may be helpful

Evaluating patients with paraneoplastic or autoimmune encephalitis (brainstem encephalitis or limbic encephalitis or cerebellar ataxia) using spinal fluid specimens

Testing Algorithm Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

If the cell binding antibody result is reactive, then the immunofluorescence titer assay will be performed at an additional charge.

Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

Kelch-like protein 11 (KLHL11, or Kelch-like family member 11) IgG is a biomarker of paraneoplastic encephalitis KLHL11 encephalitis is a unique paraneoplastic syndrome commonly associated with testicular germ cell tumors, mainly seminoma. Ataxia, diplopia, dysarthria, and vertigo are common presenting features of the rhombencephalitis phenotype. Hearing loss and tinnitus may precede other neurological signs and symptoms by weeks to months. A subset of patients also has clinical and magnetic resonance imaging (MRI) presentations consistent with limbic encephalitis. Most patients with this syndrome have inflammatory spinal fluid profiles (especially elevated oligoclonal bands). MRI brain demonstrates T2 fluid attenuated inversion recovery (T2/FLAIR) abnormalities involving the brainstem or limbic system. The accompanying neurological disorder is usually severe. Clinical improvement following treatment of cancer or immunotherapy has been reported.

Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.


Interpretation Provides information to assist in interpretation of the test results

Positivity for Kelch-like protein 11 (KLHL11)-IgG is indicative of a paraneoplastic neurological syndrome. Positivity indicates a high likelihood of finding a testicular cancer. A rigorous search for cancer should be initiated after KLHL11 autoimmunity is confirmed.

Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

A negative Kelch-like protein 11 (KLHL11) antibody test result does not exclude autoimmune neurological disease or cancer. 

Clinical Reference Recommendations for in-depth reading of a clinical nature

1. Mandel-Brehm C, Dubey D, Kryzer TJ, et al: Kelch-like protein 11 antibodies in seminoma-associated paraneoplastic encephalitis. N Engl J Med. 2019;381:47-54

2. Dubey D, Wilson MR, Clarkson B, et al: Expanded clinical phenotype, oncological associations, and immunopathologic insights of paraneoplastic Kelch-like protein-11 encephalitis. JAMA Neurol. 2020 Aug 3;77(11):1-10