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Abstract Details

Clinical Significance of Kelch-like Protein 11 Antibodies
Autoimmune Neurology
S16 - Autoimmune Neurological Disorders: Diagnosis, Biomarkers, and Epidemiology (2:24 PM-2:36 PM)
008
To report the clinical and oncological associations of antibodies against Kelch-like 11 protein (KLHL11-abs) and to analyze the value of routine immunohistochemistry in the screening of these antibodies.
KLHL11-abs have been recently proposed as a new biomarker of paraneoplastic brainstem/cerebellar syndrome and testicular seminoma. However, the spectrum of neurological syndromes and tumors related to these antibodies is unknown.
KLHL11-abs were determined using a cell-based assay (CBA) with HEK293T cells transfected with a human KLHL11 clone and immunoprecipitation. Positive samples were assessed by immunohistochemistry on rat brain sections. Overall, we examined sera of 420 patients with the following disorders: 157 paraneoplastic syndromes, 172 autoimmune neurological disorders, 63 neurological symptoms in the setting of teratomas, 28 isolated small-cell lung cancer (SCLC), and 12 healthy subjects.
KLHL11-abs were detected in 32 patients by CBA (all confirmed by immunoprecipitation). Immunohistochemistry on rat brain only detected 7 (21%) of the KLHL11-ab-positive samples. Patients’ median age was 28.5 years (range 9 -76), 16 (50%) were women. Fourteen (44%) patients had benign teratomas (10 ovarian, 4 testicular) and 7 (22%) testicular or thymic seminomas or mixed germ-cell tumors, 1 ovarian carcinoma and 1 SCLC. Neurological syndromes included: 7 (22%) cases with cerebellar ataxia, 6 (19%) widespread encephalitis with predominant involvement of brainstem and cerebellum, 7 (22%) anti-NMDAR encephalitis (5 with ovarian teratoma), 5 (16%) opsoclonus-myoclonus, 3 (9%) limbic encephalitis, and 4 (12%) diverse neurological symptoms (3 associated with teratomas). Thirteen (41%) patients had concomitant antibodies (7 anti-NMDAR, and 6 onconeuronal).
KLHL11-abs associate with a much broader spectrum of neurological syndromes and tumors than previously reported. They may also occur in well-defined autoimmune encephalitis (anti-NMDAR encephalitis or Ma2 encephalitis) without clinically modifying their phenotype. Routine rat brain immunohistochemistry is not useful for the screening of these antibodies.
Authors/Disclosures

PRESENTER 		No disclosure on file
No disclosure on file
No disclosure on file
Thais Armangue, MD (IDIBAPS-HClinic) Dr. Armangue has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. The institution of Dr. Armangue has received research support from ISCIII(Spanish institute of health) -PI21/00316, Marato TV3, La Caixa Research Foundadion, Pablove Foundation (689368), Torrons Vicens Foundation (PFNR0144), 2021 Invest AEP.
Albert Saiz (Hospital Clinico De Barcelona) Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janseen. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for novartis. Albert Saiz has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen.
Francesc R. Graus, MD (IDIBAPS) Dr. Graus has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for MedLink. Dr. Graus has received intellectual property interests from a discovery or technology relating to health care.
Josep O. Dalmau, MD, PhD, FAAN Dr. Dalmau has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astellas Research Institute of America. Dr. Dalmau has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen Research & Development . Dr. Dalmau has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for 好色先生. An immediate family member of Dr. Dalmau has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer Nature. The institution of Dr. Dalmau has received research support from Sage Therapeutics. The institution of Dr. Dalmau has received research support from Edmond J.Safra Foundation . The institution of Dr. Dalmau has received research support from La Caixa Foundation. The institution of Dr. Dalmau has received research support from Spanish Ministry of Health (ISCIII). The institution of Dr. Dalmau has received research support from Euroimmun, Inc. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care.
No disclosure on file