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http://hdl.handle.net/2445/184091
Title: | Clinical significance of Kelch-like protein 11 antibodies |
Author: | Maudes, Estíbaliz Landa, Jon Muñoz Lopetegi, Amaia Armangué, Thaís Alba, Mercedes Saiz Hinarejos, Albert Graus Ribas, Francesc Dalmau Obrador, Josep Sabater, Lidia |
Keywords: | Càncer de pulmó Immunohistoquímica Malalties del sistema nerviós central Lung cancer Immunohistochemistry Central nervous system diseases |
Issue Date: | 17-Jan-2020 |
Publisher: | American Academy of Neurology |
Abstract: | Objective: To report the clinical and oncologic associations of antibodies against Kelch-like protein 11 (KLHL11-ab), recently suggested as biomarkers of a paraneoplastic brainstem cerebellar syndrome associated with testicular seminoma, and to determine the value of immunohistochemistry as a screening technique. Methods: Studies included 432 sera or CSF from 329 patients with paraneoplastic (157) or autoimmune neurologic syndromes (172); 63 with neurologic symptoms and benign teratomas; 28 with small-cell lung cancer, and 12 healthy subjects. KLHL11-abs were examined using a cell-based assay (CBA) with HEK293 cells transfected with a human KLHL11 clone. The CBA specificity was confirmed by immunoprecipitation. All positive samples were examined by immunohistochemistry on rat brain sections. Results: KLHL11-abs were detected in 32 patients by CBA, and patients' antibodies immunoprecipitated KLHL11. Using rat brain immunohistochemistry, only 7 samples (22%) were positive. Patients' median age was 28 years (range 9-76 years), and 16 (50%) were women. Tumors were identified in 23/32 (72%) patients, including 14 teratomas and 7 seminomas or mixed germ cell tumors. Thirteen (41%) patients had cerebellar ataxia (7) or encephalitis with brainstem cerebellar symptoms (6), 7 (22%) anti-NMDA receptor (NMDAR) encephalitis (5 with ovarian teratoma), 5 (16%) opsoclonus-myoclonus, 3 (9%) limbic encephalitis, and 4 (12%) diverse neurologic symptoms (3 with benign teratomas). Concurrent autoantibodies occurred in 14 (44%) patients (7 anti-NMDAR, 6 Ma2, and 1 Hu). Conclusions: KLHL11-abs associate with a spectrum of syndromes and tumors wider than those previously reported; 44% of patients have concurrent neuronal antibodies, some of them (anti-NMDAR) pathogenically relevant. Brain immunostaining is not useful for routine screening of KLHL11-abs. |
Note: | Reproducció del document publicat a: https://doi.org/10.1212/NXI.000000000000066 |
It is part of: | Neurology. Neuroimmunology & Neuroinflammation, 2020, vol. 7, num. 3, p. e666 |
URI: | http://hdl.handle.net/2445/184091 |
Related resource: | https://doi.org/10.1212/NXI.000000000000066 |
ISSN: | 2332-7812 |
Appears in Collections: | Articles publicats en revistes (Medicina) Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer) |
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