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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.

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.

SUBMITTER: Yan J

PROVIDER: S-EPMC7734506 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature

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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.

Yan Jinhua J Chen Zhongbo Z Liang Yumei Y Yang Huijia H Cao Lizhi L Zhou Yuling Y Zhao Yang Y Zhang Ying Y

The Journal of international medical research 20201201 12

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 ant ...[more]

PMID: 33305627

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Project description:ImportanceLittle is known of glutamic acid decarboxylase antibodies (GAD-abs) in the paraneoplastic context. Clinical recognition of such cases will lead to prompt tumor diagnosis and appropriate treatment.ObjectiveTo report the clinical and immunological features of patients with paraneoplastic neurological syndromes (PNS) and GAD-abs.Design, setting, and participantsRetrospective case series study and immunological investigations conducted in February 2014 in a center for autoimmune neurological disorders. Fifteen cases with GAD65-abs evaluated between 1995 and 2013 who fulfilled criteria of definite or possible PNS without concomitant onconeural antibodies were included in this study.Main outcomes and measuresAnalysis of the clinical records of 15 patients and review of 19 previously reported cases. Indirect immunofluorescence with rat hippocampal neuronal cultures and cell-based assays with known neuronal cell-surface antigens were used. One hundred six patients with GAD65-abs and no cancer served as control individuals.ResultsEight of the 15 patients with cancer presented as classic paraneoplastic syndromes (5 limbic encephalitis, 1 paraneoplastic encephalomyelitis, 1 paraneoplastic cerebellar degeneration, and 1 opsoclonus-myoclonus syndrome). When compared with the 106 non-PNS cases, those with PNS were older (median age, 60 years vs 48 years; P = .03), more frequently male (60% vs 13%; P < .001), and had more often coexisting neuronal cell-surface antibodies, mainly against γ-aminobutyric acid receptors (53% vs 11%; P < .001). The tumors more frequently involved were lung (n = 6) and thymic neoplasms (n = 4). The risk for an underlying tumor was higher if the presentation was a classic PNS, if it was different from stiff-person syndrome or cerebellar ataxia (odds ratio, 10.5; 95% CI, 3.2-34.5), or if the patient had coexisting neuronal cell-surface antibodies (odds ratio, 6.8; 95% CI, 1.1-40.5). Compared with the current series, the 19 previously reported cases had more frequent stiff-person syndrome (74% vs 13%; P = .001) and better responses to treatment (79% vs 27%; P = .005). Predictors of improvement in the 34 patients (current and previously reported) included presentation with stiff-person syndrome and the presence of a thymic tumor.Conclusions and relevancePatients with GAD-abs must be screened for an underlying cancer if they have clinical presentations different from those typically associated with this autoimmunity or develop classic PNS. The risk for cancer increases with age, male sex, and the presence of coexisting neuronal cell-surface antibodies.

Dataset Information

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.

Publications

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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