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      Anti-CV2/CRMP5 antibody-positive paraneoplastic neurological syndromes with chronic intestinal pseudo-obstruction in a small-cell lung cancer patient: a case report and literature review

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          Abstract

          Patients with anti-CV2/collapsin response mediator protein (CRMP)5 antibodies present with more frequent chorea, cerebellar ataxia, uveo/retinal symptoms, and Lambert–Eaton myasthenic syndrome or myasthenia gravis. Chronic intestinal pseudo-obstruction (CIPO) is an intestinal motility dysfunction disease dysmotility that is caused by a neuromuscular disease with recurrent or persistent intestinal obstruction in the absence of mechanical obstruction. We report the case of a patient with CRMP5 antibody-positive paraneoplastic neurological syndrome (PNS) that is associated with autonomic dysfunction (presenting most remarkably as CIPO). CIPO is one of the rarest forms of PNS. Some PNS patients who are positive for anti-CV2/CRMP5 antibodies may have fatal complications such as CIPO. To detect if PNS patients are at risk for CIPO, a timely diagnosis and appropriate treatment are required.

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          Most cited references34

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          Recommended diagnostic criteria for paraneoplastic neurological syndromes.

          Paraneoplastic neurological syndromes (PNS) are defined by the presence of cancer and exclusion of other known causes of the neurological symptoms, but this criterion does not separate "true" PNS from neurological syndromes that are coincidental with a cancer. To provide more rigorous diagnostic criteria for PNS. An international panel of neurologists interested in PNS identified those defined as "classical" in previous studies. The panel reviewed the existing diagnostic criteria and recommended new criteria for those in whom no clinical consensus was reached in the past. The panel reviewed all reported onconeural antibodies and established the conditions to identify those that would be labelled as "well characterised". The antibody information was obtained from published work and from unpublished data from the different laboratories involved in the study. The panel suggest two levels of evidence to define a neurological syndrome as paraneoplastic: "definite" and "possible". Each level can be reached combining a set of criteria based on the presence or absence of cancer and the definitions of "classical" syndrome and "well characterised" onconeural antibody. The proposed criteria should help clinicians in the classification of their patients and the prospective and retrospective analysis of PNS cases.
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            Paraneoplastic syndromes of the CNS.

            Major advances in the management of paraneoplastic neurologic disorders (PND) include the detection of new antineuronal antibodies, the improved characterisation of known syndromes, the discovery of new syndromes, and the use of CT and PET to reveal the associated tumours at an early stage. In addition, the definition of useful clinical criteria has facilitated the early recognition and treatment of these disorders. In this article, we review some classic concepts about PND and recent clinical and immunological developments, focusing on paraneoplastic cerebellar degeneration, opsoclonus-myoclonus, and encephalitides affecting the limbic system.
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              Paraneoplastic neurological syndromes.

              Paraneoplastic neurological syndromes are immune-mediated erroneous attacks on the central or peripheral nervous systems, or both, directed originally against the tumour itself. They have been known for more than 40 years, but recently the discovery of new subgroups of paraneoplastic encephalitis syndromes with a remarkably good response to immune therapy has ignited new clinical and scientific interest. Knowledge of these subgroups and their associated autoantibodies is important in therapeutic decision-making. However, the abundance of new autoantibodies and syndromes can be confusing. This review paper summarizes current knowledge and new developments in the field of paraneoplastic neurological syndromes, their classification, pathophysiology and treatment. © 2013 British Society for Immunology.
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                Author and article information

                Journal
                J Int Med Res
                J Int Med Res
                IMR
                spimr
                The Journal of International Medical Research
                SAGE Publications (Sage UK: London, England )
                0300-0605
                1473-2300
                11 December 2020
                December 2020
                : 48
                : 12
                : 0300060520974466
                Affiliations
                [1-0300060520974466]Department of Neurology and Neuroscience Center, First Hospital of Jilin University, Changchun, China
                Author notes
                [*]

                These authors contributed equally to this work.

                [*]Yang Zhao, Department of Neurology and Neuroscience Center, First Hospital of Jilin University, 21 Xinmin Street, Changchun 130000, China. Email: youngzhao.jlu@ 123456foxmail.com
                Author information
                https://orcid.org/0000-0001-5442-5221
                https://orcid.org/0000-0002-9143-671X
                Article
                10.1177_0300060520974466
                10.1177/0300060520974466
                7734506
                33305627
                ea12b319-da2c-4987-9a09-ee2486af4582
                © The Author(s) 2020

                Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 7 April 2020
                : 26 October 2020
                Funding
                Funded by: National Natural Science Foundation of China, FundRef https://doi.org/10.13039/501100001809;
                Award ID: NO.81600924
                Funded by: the National Key Research and Development Project of China ;
                Award ID: NO.2018YFC1312301
                Categories
                Case Report
                Custom metadata
                ts2

                collapsin response mediator protein,cv2/crmp5 antibodies,chronic intestinal pseudo-obstruction,small-cell lung cancer,paraneoplastic neurological syndrome,intestinal motility dysfunction disease,dysmobility,neuromuscular disease

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