Miniatura

Fitxers

709137.pdf (308.53 KB)

Tipus de document

Article

Versió

Versió publicada

Data de publicació

2020-01-17

Llicència de publicació

cc-by-nc-nd (c) Maudes, Estíbaliz et al., 2020
Si us plau utilitzeu sempre aquest identificador per citar o enllaçar aquest document: https://hdl.handle.net/2445/184091

Clinical significance of Kelch-like protein 11 antibodies

Títol de la revista

Autors

Director/Tutor

ISSN de la revista

Títol del volum

Recurs relacionat

https://doi.org/10.1212/NXI.000000000000066

Resum

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.

Matèries

Càncer de pulmó, Immunohistoquímica, Malalties del sistema nerviós central

Matèries (anglès)

Lung cancer, Immunohistochemistry, Central nervous system diseases

Citació

Col·leccions

Articles publicats en revistes (Medicina)
Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)

Citació

MAUDES, Estibaliz, LANDA MEDRANO, Jon, MUÑOZ LOPETEGI, Amaia, ARMANGUÉ, Thaís, ALBA, Mercedes, SAIZ HINAREJOS, Albert, GRAUS RIBAS, Francesc, DALMAU OBRADOR, Josep, SABATER BAUDET, Lidia. Clinical significance of Kelch-like protein 11 antibodies. _Neurology. Neuroimmunology & Neuroinflammation_. 2020. Vol. 7, núm. 3, pàgs. e666. [consulta: 30 de gener de 2026]. ISSN: 2332-7812. [Disponible a: https://hdl.handle.net/2445/184091]

Exportar metadades

JSON - METS

Compartir registre