Kelch-like protein 11 antibody-associated paraneoplastic neurological syndrome : A state-of-the-art review

Copyright © 2022 Elsevier Inc. All rights reserved.

Bibliographische Detailangaben
Veröffentlicht in:Clinical immunology (Orlando, Fla.). - 1999. - 241(2022) vom: 25. Aug., Seite 109074
1. Verfasser: Li, Er-Chuang (VerfasserIn)
Weitere Verfasser: Lai, Qi-Lun, Cai, Meng-Ting, Zheng, Yang, Fang, Gao-Li, Fang, Wei, Du, Bing-Qing, Shen, Chun-Hong, Ding, Mei-Ping, Zhang, Yin-Xi
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2022
Zugriff auf das übergeordnete Werk:Clinical immunology (Orlando, Fla.)
Schlagworte:Journal Article Review Immunotherapy Kelch-like protein 11 antibody Oncotherapy Paraneoplastic neurological syndrome Rhombencephalitis Testicular germ cell tumor Autoantibodies
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520 |a The Kelch-like protein 11 antibody-associated paraneoplastic neurological syndrome (KLHL 11-PNS) was first identified in 2019. This novel antibody, targeting the intracellular KLHL 11 antigen, can be detected in serum and cerebrospinal fluid using tissue-based and cell-based assays. It is thought to be a biomarker for a T-cell autoimmunity response. The most likely immunopathogenesis of KLHL 11-PNS appears to be linked to cytotoxic T-cell-mediated neuronal injury and loss. Patients have adult-male predilection, rhombencephalitis (brainstem and / or cerebellar involvement), and a robust oncological correlation with testicular germ cell tumors (predominately seminoma). Brain magnetic resonance imaging demonstrated T2 / fluid-attenuated inversion recovery hyperintensities and atrophy of the temporal lobe, cerebellum, and brainstem. Most patients responded poorly to immunotherapy and oncotherapy and thus had a poor long-term prognosis. We review the literature and provide an update of current knowledge regarding KLHL 11-PNS, including epidemiology, underlying mechanism, clinical presentations, paraclinical and oncological findings, diagnostic workup, and treatment approaches 
650 4 |a Journal Article 
650 4 |a Review 
650 4 |a Immunotherapy 
650 4 |a Kelch-like protein 11 antibody 
650 4 |a Oncotherapy 
650 4 |a Paraneoplastic neurological syndrome 
650 4 |a Rhombencephalitis 
650 4 |a Testicular germ cell tumor 
650 7 |a Autoantibodies  |2 NLM 
700 1 |a Lai, Qi-Lun  |e verfasserin  |4 aut 
700 1 |a Cai, Meng-Ting  |e verfasserin  |4 aut 
700 1 |a Zheng, Yang  |e verfasserin  |4 aut 
700 1 |a Fang, Gao-Li  |e verfasserin  |4 aut 
700 1 |a Fang, Wei  |e verfasserin  |4 aut 
700 1 |a Du, Bing-Qing  |e verfasserin  |4 aut 
700 1 |a Shen, Chun-Hong  |e verfasserin  |4 aut 
700 1 |a Ding, Mei-Ping  |e verfasserin  |4 aut 
700 1 |a Zhang, Yin-Xi  |e verfasserin  |4 aut 
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