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231226s2022 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2022.109074
|2 doi
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|a pubmed24n1144.xml
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|a (DE-627)NLM343303418
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|a (NLM)35809856
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|a (PII)S1521-6616(22)00155-3
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|a DE-627
|b ger
|c DE-627
|e rakwb
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|a eng
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|a Li, Er-Chuang
|e verfasserin
|4 aut
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|a Kelch-like protein 11 antibody-associated paraneoplastic neurological syndrome
|b A state-of-the-art review
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|c 2022
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|a Text
|b txt
|2 rdacontent
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|a ƒaComputermedien
|b c
|2 rdamedia
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|a ƒa Online-Ressource
|b cr
|2 rdacarrier
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|a Date Completed 09.08.2022
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|a Date Revised 25.08.2022
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2022 Elsevier Inc. All rights reserved.
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|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
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|a Journal Article
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|a Review
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|a Immunotherapy
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|a Kelch-like protein 11 antibody
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|a Oncotherapy
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|a Paraneoplastic neurological syndrome
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|a Rhombencephalitis
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|a Testicular germ cell tumor
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|a Autoantibodies
|2 NLM
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1 |
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|a Lai, Qi-Lun
|e verfasserin
|4 aut
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1 |
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|a Cai, Meng-Ting
|e verfasserin
|4 aut
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|a Zheng, Yang
|e verfasserin
|4 aut
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1 |
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|a Fang, Gao-Li
|e verfasserin
|4 aut
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|a Fang, Wei
|e verfasserin
|4 aut
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|a Du, Bing-Qing
|e verfasserin
|4 aut
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|a Shen, Chun-Hong
|e verfasserin
|4 aut
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1 |
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|a Ding, Mei-Ping
|e verfasserin
|4 aut
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|a Zhang, Yin-Xi
|e verfasserin
|4 aut
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 241(2022) vom: 25. Aug., Seite 109074
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|x 1521-7035
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|g volume:241
|g year:2022
|g day:25
|g month:08
|g pages:109074
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|u http://dx.doi.org/10.1016/j.clim.2022.109074
|3 Volltext
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