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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Wiley
2013-01-01
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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. |
format | Article |
id | doaj-art-601a2e7d645742db9becefb78f2820d8 |
institution | Kabale University |
issn | 0962-9351 1466-1861 |
language | English |
publishDate | 2013-01-01 |
publisher | Wiley |
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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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