Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?

Paraneoplastic neurological syndromes (PNSs) occur in patients with cancer and can cause clinical symptoms and signs of dysfunction of the nervous system that are not due to a local effect of the tumor or its metastases. Most of these clinical syndromes in adults are associated with lung cancer, esp...

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Main Authors: Hongliang Zhang, Chunkui Zhou, Limin Wu, Fengming Ni, Jie Zhu, Tao Jin
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Mediators of Inflammation
Online Access:http://dx.doi.org/10.1155/2013/172986
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author Hongliang Zhang
Chunkui Zhou
Limin Wu
Fengming Ni
Jie Zhu
Tao Jin
author_facet Hongliang Zhang
Chunkui Zhou
Limin Wu
Fengming Ni
Jie Zhu
Tao Jin
author_sort Hongliang Zhang
collection DOAJ
description Paraneoplastic neurological syndromes (PNSs) occur in patients with cancer and can cause clinical symptoms and signs of dysfunction of the nervous system that are not due to a local effect of the tumor or its metastases. Most of these clinical syndromes in adults are associated with lung cancer, especially small cell lung cancer (SCLC), lymphoma, and gynecological tumors. The finding of highly specific antibodies directed against onconeural antigens has revolutionized the diagnosis and promoted the understanding of these syndromes and led to the current hypothesis of an autoimmune pathophysiology. Accumulating data strongly suggested direct pathogenicity of these antibodies. The field of PNS has expanded rapidly in the past few years with the discovery of limbic encephalitis associated with glutamic acid decarboxylase (GAD) 65, the voltage (VGKC-gated potassium channel) complex, the methyl (N-NMDA-D-aspartate), alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA), and gamma aminobutyric acid (GABA) (B) receptors, and so forth. Despite this, the clinical spectrum of these diseases has not yet been fully investigated. The clinical importance of these conditions lies in their frequent response to immunotherapies and, less commonly, their association with distinctive tumors. This review provides an overview on the pathogenesis and diagnosis of PNS, with emphasis on the role of antibodies in limbic encephalitis.
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series Mediators of Inflammation
spelling doaj-art-601a2e7d645742db9becefb78f2820d82025-02-03T01:31:23ZengWileyMediators of Inflammation0962-93511466-18612013-01-01201310.1155/2013/172986172986Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?Hongliang Zhang0Chunkui Zhou1Limin Wu2Fengming Ni3Jie Zhu4Tao Jin5Department of Neurology, The First Bethune Hospital of Jilin University, Jilin University, Xinmin Street 71, Changchun 130021, ChinaDepartment of Neurology, The First Bethune Hospital of Jilin University, Jilin University, Xinmin Street 71, Changchun 130021, ChinaDepartment of Neurology, The First Bethune Hospital of Jilin University, Jilin University, Xinmin Street 71, Changchun 130021, ChinaDepartment of Radiotherapy, The First Bethune Hospital of Jilin University, Xinmin Street 71, Changchun 130021, ChinaDepartment of Neurology, The First Bethune Hospital of Jilin University, Jilin University, Xinmin Street 71, Changchun 130021, ChinaDepartment of Neurology, The First Bethune Hospital of Jilin University, Jilin University, Xinmin Street 71, Changchun 130021, ChinaParaneoplastic neurological syndromes (PNSs) occur in patients with cancer and can cause clinical symptoms and signs of dysfunction of the nervous system that are not due to a local effect of the tumor or its metastases. Most of these clinical syndromes in adults are associated with lung cancer, especially small cell lung cancer (SCLC), lymphoma, and gynecological tumors. The finding of highly specific antibodies directed against onconeural antigens has revolutionized the diagnosis and promoted the understanding of these syndromes and led to the current hypothesis of an autoimmune pathophysiology. Accumulating data strongly suggested direct pathogenicity of these antibodies. The field of PNS has expanded rapidly in the past few years with the discovery of limbic encephalitis associated with glutamic acid decarboxylase (GAD) 65, the voltage (VGKC-gated potassium channel) complex, the methyl (N-NMDA-D-aspartate), alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA), and gamma aminobutyric acid (GABA) (B) receptors, and so forth. Despite this, the clinical spectrum of these diseases has not yet been fully investigated. The clinical importance of these conditions lies in their frequent response to immunotherapies and, less commonly, their association with distinctive tumors. This review provides an overview on the pathogenesis and diagnosis of PNS, with emphasis on the role of antibodies in limbic encephalitis.http://dx.doi.org/10.1155/2013/172986
spellingShingle Hongliang Zhang
Chunkui Zhou
Limin Wu
Fengming Ni
Jie Zhu
Tao Jin
Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?
Mediators of Inflammation
title Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?
title_full Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?
title_fullStr Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?
title_full_unstemmed Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?
title_short Are Onconeural Antibodies a Clinical Phenomenology in Paraneoplastic Limbic Encephalitis?
title_sort are onconeural antibodies a clinical phenomenology in paraneoplastic limbic encephalitis
url http://dx.doi.org/10.1155/2013/172986
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