POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy: a case report

POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy: a case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy: a case report xuan Zou, Yan Hong, Guanen Zhou This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4789563/v1 This work is licensed under a CC BY 4.0 License Status: Under Review Version 1 posted 11 You are reading this latest preprint version Abstract Background POEMS syndrome is a rare paraneoplastic syndrome caused by abnormal proliferation of plasma cells. It can cause multiple peripheral neuropathy, but the specific mechanism is still unclear. we describe a case of POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy. Case presentation A 66-year-old male patient developed flaccid paralysis of the limbs after diarrhea two months ago. He was diagnosed with POEMS syndrome 4 years ago. The titer of the IgG antibody against contact associated protein1 (Caspr1) in the patient's serum was 1:100, and the IgG and IgM antibodies against GM1 were weakly positive. Brain MRI shows left paraventricular infarction, accompanied by cerebrovascular stenosis, severe heart failure, and pleural effusion. The patient's condition improved after receiving plasma exchange (PE) and methylprednisolone treatment. Conclusion There is report of POEMS syndrome combined with anti-GM1 antibody1, but there has been no report of POEMS syndrome combined with anti-Caspr1 antibody. Abnormal plasma cell proliferation in POEMS syndrome may be the culprit behind the production of antibodies. Figures Figure 1 Figure 2 Figure 3 Figure 4 Background POEMS syndrome is a rare paraneoplastic syndrome caused by abnormal proliferation of plasma cells. 100% of POEMS syndromes are complicated with multiple peripheral neuropathy, which is also the first condition for diagnosing POEMS. The literature reports that POEMS syndrome can be accompanied by anti-ganglioside antibodies [ 1 ], but the specific mechanism is unclear. we describe a case of POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy. There has been no relevant literature report before. Abnormal plasma cell proliferation in POEMS syndrome may be the main culprit in producing abnormal peripheral nerve antibodies. Case presentation A 66-year-old male patient experienced worsening of flaccid paralysis in the limbs after diarrhea two months ago, accompanied by pain below the wrist and knee joints. He was diagnosed with “Guillain-Barre syndrome” 7 years ago and left with symptoms of numbness and weakness in his limbs. Five years ago, the patient was diagnosed with hypothyroidism due to decreased Free Triiodothyronine (FT3) and increased Thyroid-stimulating hormone (TSH). Four years ago, the patient was hospitalized again due to numbness in his hands and feet. During the hospitalization, the doctor found that the patient had symptoms such as splenomegaly, skin pigmentation (Fig. 1 ), and pulmonary arterial hypertension. The plasma vascular endothelial growth factor (VEGF) was elevated to 820.02pg/ml (reference value: 0-142pg/ml) upon examination, and serum immune electrophoresis revealed IgA-λ type monoclonal protein (MP). The patient was ultimately diagnosed with POEMS syndrome. Meanwhile, the expression of anti-GM1 IgG antibody in the patient's serum was weakly positive, with anti-SOX1 antibody (++) in serum and anti-SOX1 antibody (+) in the cerebrospinal fluid (CSF). Anti-nuclear antibody and anti-histone antibody are also positive in serum. The patient's condition improved after receiving intravenous immune globulin (IVIG) and methylprednisolone treatment, and later received Rd regimen (lenalidomide + low-dose dexamethasone) treatment in the hematology department. This time, the patient was readmitted due to a subacute onset of numbness and weakness in the limbs. After admission, we tested the patient's serum peripheral nerve antibodies. The titer of the IgG antibody against contact associated protein1 (Caspri1) in the patient's serum was 1:100 (Fig. 2 ), and the IgG and IgM antibodies against GM1 were weakly positive. The electromyography shows a decrease in the amplitude and conduction velocity of peripheral nerves in the limbs. The amplitude of motor and sensory conduction waves of the bilateral tibial nerves was not elicited. The occurrence rate of upper limb F-waves was low, and lower limb F-waves were not extracted. In addition, the patient simultaneously exhibits some characteristic manifestations of POEMS syndrome. The patient's brain magnetic resonance imaging (MRI) showed left paraventricular infarction (Fig. 3 ), while vascular ultrasound showed severe stenosis of the left internal carotid artery and middle cerebral artery. At the same time, the patient is accompanied by severe heart failure, bilateral massive pleural effusion (Fig. 4 ), and hypoalbuminemia. After receiving methylprednisolone treatment, the patient's limb weakness improved, and the upper limbs were able to hold objects while the lower limbs were able to stand. Both anti-Caspri1 antibody and anti-GM1 antibody turned negative. Discussion and conclusions POEMS syndrome [ 2 ] is caused by abnormal proliferation of plasma cells and is a rare paraneoplastic syndrome. In addition to causing multiple peripheral-neuropathy, it can also cause organ enlargement, endocrine disorders, monoclonal proteinemia, and skin changes. In addition to the typical symptoms mentioned above, this patient also presents with characteristic manifestations such as cerebral infarction and pleural effusion. The electrophysiological changes in POEMS syndrome have a relatively uniform slowing of conduction velocity (NCV), mainly in the proximal end, usually without conduction block and abnormal waveform dispersion. The patient in this case has a subacute onset, with a course of more than 8 weeks. And the patient had a history of infection before the onset of the disease. Limb weakness and sensory abnormalities are mainly located at the distal end, and the electromyography shows conduction block. It is considered that the responsibility antibody for the worsening of the disease is anti-Caspri1 antibody. Caspri1 is located in the vicinity of the Ranvier node. It forms a complex with contact-1 (CNTN1) and neurofascin 155 (NF155), participating in nerve conduction. Patients with positive anti-Caspri1 antibody exhibit acute/subacute neuropathy, often accompanied by ataxia, neuropathic pain, cranial nerve involvement, and poor response to IVIg. Anti-Caspri1 antibody disease was initially classified as chronic inflammatory demyelinating polyneuropathy (CIDP) variants, but due to its unique clinical features such as ataxia, tremors, etc., they are currently classified as autoimmune nodo-paranodopathy [ 3 ]. After reviewing the literature, we found relevant report of POEMS syndrome combined with anti-GM1 antibody [ 1 ], but there is no report of POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy. Abnormal plasma cell proliferation in POEMS syndrome may be the culprit behind the production of anti-Caspri1 antibody. The generation of abnormal antibodies is not limited to the peripheral nerves, but may also affect the central nervous system or connective tissue. The presence of anti-SOX antibody, anti-nuclear antibody, and anti-histone antibody in the patient's previous serum or cerebrospinal fluid confirms this. The antibodies in patients with autoimmune nodo-paranodopathy are mostly IgG4 subtype, and their effectiveness in IVIg is often poor. Effective methods for eliminating antibodies, such as PE and rituximab, are the preferred treatment options. Unfortunately, in this case, due to the presence of cachexia and concerns about the worsening of infection and heart failure, we did not provide treatment with PE or rituximab. However, simple glucocorticoid therapy has also achieved good therapeutic effects. The more fundamental treatment method is chemotherapy or radiation therapy to eliminate abnormally proliferating plasma cells and prevent the production of abnormal antibodies. It is worth noting that this type of plasma cell proliferation disorder is often confused with autoimmune peripheral neuropathy in the early stages, which delays the optimal treatment time. In clinical practice, we should pay attention to differential diagnosis by observing clinical symptoms, combining autoimmune peripheral nerve antibodies and immunoelectrophoresis results. Early diagnosis and control may prolong the survival of POEMS syndrome patients. However, it should also be noted that patients with POEMS syndrome who experience acute/subacute progressive peripheral nerve injury need to undergo comprehensive immune peripheral nerve antibody testing. Declarations Funding Tianjin Key Medical Discipline (Specialty) Construction Project (No. TJYXZDXK-052B). Author information Authors and Affiliations Department of Neurology, Huanhu hospital, Nankai University, Tianjin 300350, China Xuan Zou, Yan Hong, Guanen Zhou Contributions Xuan Zou designed and drafted the manuscript. Yan Hong contributed to analysis and interpretation of data. Guanen Zhou revised the manuscript for intellectual content. Corresponding author Guanen Zhou, email: [email protected] Ethics declarations Conflicts of interest The authors declare that there is no conflict of interest relevant to this paper. Ethical standard Patient ’ s informed consent and permission to publish his information have been obtained. Informed consent Written informed consent was collected from the patient for the inclusion of anonymized clinical data in a scientific publication, in agreement with the Declaration of Helsinki. References Nobile-Orazio E, Giannotta C, Briani C. Anti-ganglioside complex IgM antibodies in multifocal motor neuropathy and chronic immune-mediated neuropathies. J Neuroimmunol. 2010;219:119–22. 10.1016/j.jneuroim.2009.11.012 . BardwickPA ZNJ, GillGN, et al. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. Med (Baltim). 1980;59:311–22. 10.1097/00005792-198007000-00006 . Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint Task Force–Second revision. Eur J Neurol. 2021;28:3556–83. 10.1111/ene.15225 . Additional Declarations No competing interests reported. Cite Share Download PDF Status: Under Review Version 1 posted Editorial decision: Revision requested 07 Oct, 2024 Reviewers agreed at journal 16 Sep, 2024 Reviews received at journal 16 Sep, 2024 Reviewers agreed at journal 09 Sep, 2024 Reviews received at journal 09 Sep, 2024 Reviewers agreed at journal 02 Sep, 2024 Reviewers invited by journal 02 Sep, 2024 Editor invited by journal 02 Aug, 2024 Editor assigned by journal 01 Aug, 2024 Submission checks completed at journal 01 Aug, 2024 First submitted to journal 23 Jul, 2024 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4789563","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":345082809,"identity":"2d89bc0f-a6d4-4de6-9a39-e23ea7a5be12","order_by":0,"name":"xuan Zou","email":"","orcid":"","institution":"Tianjin Huanhu Hospital","correspondingAuthor":false,"prefix":"","firstName":"xuan","middleName":"","lastName":"Zou","suffix":""},{"id":345082816,"identity":"bcbfe2e8-e874-44bc-a780-0ec2a5ff4393","order_by":1,"name":"Yan Hong","email":"","orcid":"","institution":"Tianjin Huanhu Hospital","correspondingAuthor":false,"prefix":"","firstName":"Yan","middleName":"","lastName":"Hong","suffix":""},{"id":345082819,"identity":"8bfadf0b-7ee3-49a2-aea3-715286d1b7bb","order_by":2,"name":"Guanen Zhou","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAAqElEQVRIiWNgGAWjYFCCA0BcISEnT7QGHrCWMxbGhg3EawECxraKRLBWooA94/HHLz7Ok0hgbGB++OgGkQ5Ls5y5TSKPnYHN2DiHSC3HjHm3SRQzNvCwSROp5WCb8d85EokNB4jXcpj5MWMDSVoOHGNj7DkmYWzYTKxf2Gccf/zhR02dnDx788PHRGlhkDjAJgFmMBOlHAT4G5g/EK14FIyCUTAKRiYAAL4jMApY01SOAAAAAElFTkSuQmCC","orcid":"","institution":"Tianjin Huanhu Hospital","correspondingAuthor":true,"prefix":"","firstName":"Guanen","middleName":"","lastName":"Zhou","suffix":""}],"badges":[],"createdAt":"2024-07-23 14:32:25","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4789563/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4789563/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":64009883,"identity":"63154bde-d923-46bb-8291-2d22d78ec697","added_by":"auto","created_at":"2024-09-04 23:22:23","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":220635,"visible":true,"origin":"","legend":"\u003cp\u003eThe patient's skin has pigmentation\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-4789563/v1/649050684ca44fbd6e55cab0.png"},{"id":64009884,"identity":"ae5498fc-a83a-4798-a9b3-2bdca72fe9b8","added_by":"auto","created_at":"2024-09-04 23:22:23","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":232504,"visible":true,"origin":"","legend":"\u003cp\u003eThe transfection cell method detected that the patient's serum anti-Caspr1 antibody IgG was positive, and the transfected cells showed green fluorescence（× 400)\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-4789563/v1/417ab5e94af73ac67120ac6a.png"},{"id":64010197,"identity":"724387cd-631a-4d36-b721-5a2b558fdf3f","added_by":"auto","created_at":"2024-09-04 23:30:23","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":46674,"visible":true,"origin":"","legend":"\u003cp\u003eThe patient's brain MRI shows left paraventricular infarction\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-4789563/v1/a12e6e0451191290782229d2.png"},{"id":64009886,"identity":"586db474-dad6-4161-9d51-1790abff8456","added_by":"auto","created_at":"2024-09-04 23:22:23","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":79344,"visible":true,"origin":"","legend":"\u003cp\u003eThe patient's lung CT shows a large amount of pleural effusion on both sides\u003c/p\u003e","description":"","filename":"4.png","url":"https://assets-eu.researchsquare.com/files/rs-4789563/v1/b6f672e7a37856351de2b9b3.png"},{"id":64010198,"identity":"377c1fc3-9959-47cd-8b8d-2cebc2e53ff2","added_by":"auto","created_at":"2024-09-04 23:30:27","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":890241,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4789563/v1/9dbbc1c7-7308-496a-bdcc-862e8272ccb0.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy: a case report","fulltext":[{"header":"Background","content":"\u003cp\u003ePOEMS syndrome is a rare paraneoplastic syndrome caused by abnormal proliferation of plasma cells. 100% of POEMS syndromes are complicated with multiple peripheral neuropathy, which is also the first condition for diagnosing POEMS. The literature reports that POEMS syndrome can be accompanied by anti-ganglioside antibodies [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e], but the specific mechanism is unclear. we describe a case of POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy. There has been no relevant literature report before. Abnormal plasma cell proliferation in POEMS syndrome may be the main culprit in producing abnormal peripheral nerve antibodies.\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eA 66-year-old male patient experienced worsening of flaccid paralysis in the limbs after diarrhea two months ago, accompanied by pain below the wrist and knee joints. He was diagnosed with \u0026ldquo;Guillain-Barre syndrome\u0026rdquo; 7 years ago and left with symptoms of numbness and weakness in his limbs. Five years ago, the patient was diagnosed with hypothyroidism due to decreased Free Triiodothyronine (FT3) and increased Thyroid-stimulating hormone (TSH). Four years ago, the patient was hospitalized again due to numbness in his hands and feet. During the hospitalization, the doctor found that the patient had symptoms such as splenomegaly, skin pigmentation (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e), and pulmonary arterial hypertension. The plasma vascular endothelial growth factor (VEGF) was elevated to 820.02pg/ml (reference value: 0-142pg/ml) upon examination, and serum immune electrophoresis revealed IgA-λ type monoclonal protein (MP). The patient was ultimately diagnosed with POEMS syndrome. Meanwhile, the expression of anti-GM1 IgG antibody in the patient's serum was weakly positive, with anti-SOX1 antibody (++) in serum and anti-SOX1 antibody (+) in the cerebrospinal fluid (CSF). Anti-nuclear antibody and anti-histone antibody are also positive in serum. The patient's condition improved after receiving intravenous immune globulin (IVIG) and methylprednisolone treatment, and later received Rd regimen (lenalidomide\u0026thinsp;+\u0026thinsp;low-dose dexamethasone) treatment in the hematology department.\u003c/p\u003e \u003cp\u003eThis time, the patient was readmitted due to a subacute onset of numbness and weakness in the limbs. After admission, we tested the patient's serum peripheral nerve antibodies. The titer of the IgG antibody against contact associated protein1 (Caspri1) in the patient's serum was 1:100 (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e), and the IgG and IgM antibodies against GM1 were weakly positive. The electromyography shows a decrease in the amplitude and conduction velocity of peripheral nerves in the limbs. The amplitude of motor and sensory conduction waves of the bilateral tibial nerves was not elicited. The occurrence rate of upper limb F-waves was low, and lower limb F-waves were not extracted. In addition, the patient simultaneously exhibits some characteristic manifestations of POEMS syndrome. The patient's brain magnetic resonance imaging (MRI) showed left paraventricular infarction (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e), while vascular ultrasound showed severe stenosis of the left internal carotid artery and middle cerebral artery. At the same time, the patient is accompanied by severe heart failure, bilateral massive pleural effusion (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003e), and hypoalbuminemia. After receiving methylprednisolone treatment, the patient's limb weakness improved, and the upper limbs were able to hold objects while the lower limbs were able to stand. Both anti-Caspri1 antibody and anti-GM1 antibody turned negative.\u003c/p\u003e"},{"header":"Discussion and conclusions","content":"\u003cp\u003ePOEMS syndrome [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e] is caused by abnormal proliferation of plasma cells and is a rare paraneoplastic syndrome. In addition to causing multiple peripheral-neuropathy, it can also cause organ enlargement, endocrine disorders, monoclonal proteinemia, and skin changes. In addition to the typical symptoms mentioned above, this patient also presents with characteristic manifestations such as cerebral infarction and pleural effusion. The electrophysiological changes in POEMS syndrome have a relatively uniform slowing of conduction velocity (NCV), mainly in the proximal end, usually without conduction block and abnormal waveform dispersion. The patient in this case has a subacute onset, with a course of more than 8 weeks. And the patient had a history of infection before the onset of the disease. Limb weakness and sensory abnormalities are mainly located at the distal end, and the electromyography shows conduction block. It is considered that the responsibility antibody for the worsening of the disease is anti-Caspri1 antibody. Caspri1 is located in the vicinity of the Ranvier node. It forms a complex with contact-1 (CNTN1) and neurofascin 155 (NF155), participating in nerve conduction. Patients with positive anti-Caspri1 antibody exhibit acute/subacute neuropathy, often accompanied by ataxia, neuropathic pain, cranial nerve involvement, and poor response to IVIg. Anti-Caspri1 antibody disease was initially classified as chronic inflammatory demyelinating polyneuropathy (CIDP) variants, but due to its unique clinical features such as ataxia, tremors, etc., they are currently classified as autoimmune nodo-paranodopathy [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAfter reviewing the literature, we found relevant report of POEMS syndrome combined with anti-GM1 antibody [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e], but there is no report of POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy. Abnormal plasma cell proliferation in POEMS syndrome may be the culprit behind the production of anti-Caspri1 antibody. The generation of abnormal antibodies is not limited to the peripheral nerves, but may also affect the central nervous system or connective tissue. The presence of anti-SOX antibody, anti-nuclear antibody, and anti-histone antibody in the patient's previous serum or cerebrospinal fluid confirms this. The antibodies in patients with autoimmune nodo-paranodopathy are mostly IgG4 subtype, and their effectiveness in IVIg is often poor. Effective methods for eliminating antibodies, such as PE and rituximab, are the preferred treatment options. Unfortunately, in this case, due to the presence of cachexia and concerns about the worsening of infection and heart failure, we did not provide treatment with PE or rituximab. However, simple glucocorticoid therapy has also achieved good therapeutic effects. The more fundamental treatment method is chemotherapy or radiation therapy to eliminate abnormally proliferating plasma cells and prevent the production of abnormal antibodies.\u003c/p\u003e \u003cp\u003eIt is worth noting that this type of plasma cell proliferation disorder is often confused with autoimmune peripheral neuropathy in the early stages, which delays the optimal treatment time. In clinical practice, we should pay attention to differential diagnosis by observing clinical symptoms, combining autoimmune peripheral nerve antibodies and immunoelectrophoresis results. Early diagnosis and control may prolong the survival of POEMS syndrome patients. However, it should also be noted that patients with POEMS syndrome who experience acute/subacute progressive peripheral nerve injury need to undergo comprehensive immune peripheral nerve antibody testing.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eTianjin Key Medical Discipline (Specialty) Construction Project (No. TJYXZDXK-052B).\u003c/p\u003e\n\u003cp\u003eAuthor information\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAuthors and Affiliations\u003c/p\u003e\n\u003cp\u003eDepartment of Neurology, Huanhu hospital, Nankai University, Tianjin 300350, China\u003c/p\u003e\n\u003cp\u003eXuan Zou, Yan Hong, Guanen Zhou\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eContributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eXuan Zou designed and drafted the manuscript.\u003c/p\u003e\n\u003cp\u003eYan Hong\u0026nbsp;contributed to analysis and interpretation of data.\u003c/p\u003e\n\u003cp\u003eGuanen Zhou revised the manuscript for intellectual content.\u003c/p\u003e\n\u003cp\u003eCorresponding author\u003c/p\u003e\n\u003cp\u003eGuanen Zhou, email: [email protected]\u003c/p\u003e\n\u003cp\u003eEthics declarations\u003c/p\u003e\n\u003cp\u003eConflicts of\u0026nbsp;interest\u003c/p\u003e\n\u003cp\u003eThe authors declare that there is no conflict of interest relevant to this paper.\u003c/p\u003e\n\u003cp\u003eEthical standard\u003c/p\u003e\n\u003cp\u003ePatient\u003csup\u003e\u0026rsquo;\u003c/sup\u003es informed consent and permission to publish his information have been obtained.\u003c/p\u003e\n\u003cp\u003eInformed consent\u003c/p\u003e\n\u003cp\u003eWritten informed consent was collected from the patient for the inclusion of anonymized clinical data in a scientific publication, in agreement with the Declaration of Helsinki.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eNobile-Orazio E, Giannotta C, Briani C. Anti-ganglioside complex IgM antibodies in multifocal motor neuropathy and chronic immune-mediated neuropathies. J Neuroimmunol. 2010;219:119\u0026ndash;22. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.jneuroim.2009.11.012\u003c/span\u003e\u003cspan address=\"10.1016/j.jneuroim.2009.11.012\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eBardwickPA ZNJ, GillGN, et al. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. Med (Baltim). 1980;59:311\u0026ndash;22. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1097/00005792-198007000-00006\u003c/span\u003e\u003cspan address=\"10.1097/00005792-198007000-00006\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eVan den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint Task Force\u0026ndash;Second revision. Eur J Neurol. 2021;28:3556\u0026ndash;83. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1111/ene.15225\u003c/span\u003e\u003cspan address=\"10.1111/ene.15225\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-neurology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"nurl","sideBox":"Learn more about [BMC Neurology](http://bmcneurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/nurl","title":"BMC Neurology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"","lastPublishedDoi":"10.21203/rs.3.rs-4789563/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4789563/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cb\u003eBackground\u003c/b\u003e\u003c/p\u003e \u003cp\u003ePOEMS syndrome is a rare paraneoplastic syndrome caused by abnormal proliferation of plasma cells. It can cause multiple peripheral neuropathy, but the specific mechanism is still unclear. we describe a case of POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy.\u003c/p\u003e\u003cp\u003e\u003cb\u003eCase presentation\u003c/b\u003e\u003c/p\u003e \u003cp\u003eA 66-year-old male patient developed flaccid paralysis of the limbs after diarrhea two months ago. He was diagnosed with POEMS syndrome 4 years ago. The titer of the IgG antibody against contact associated protein1 (Caspr1) in the patient's serum was 1:100, and the IgG and IgM antibodies against GM1 were weakly positive. Brain MRI shows left paraventricular infarction, accompanied by cerebrovascular stenosis, severe heart failure, and pleural effusion. The patient's condition improved after receiving plasma exchange (PE) and methylprednisolone treatment.\u003c/p\u003e\u003cp\u003e\u003cb\u003eConclusion\u003c/b\u003e\u003c/p\u003e \u003cp\u003eThere is report of POEMS syndrome combined with anti-GM1 antibody1, but there has been no report of POEMS syndrome combined with anti-Caspr1 antibody. Abnormal plasma cell proliferation in POEMS syndrome may be the culprit behind the production of antibodies.\u003c/p\u003e","manuscriptTitle":"POEMS syndrome combined with anti-Caspri1 antibody-mediated autoimmune nodo-paranodopathy: a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-09-04 23:22:18","doi":"10.21203/rs.3.rs-4789563/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2024-10-07T11:25:51+00:00","index":"","fulltext":""},{"type":"reviewerAgreed","content":"55060898545937071888597032488478703893","date":"2024-09-16T11:18:32+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-09-16T08:38:46+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"310608037186505415310983339818882273469","date":"2024-09-09T23:48:42+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2024-09-09T06:33:51+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"143640169505748201247666262043632232935","date":"2024-09-03T01:51:40+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-09-02T12:04:02+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2024-08-02T19:58:17+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2024-08-02T02:34:03+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2024-08-02T02:32:54+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Neurology","date":"2024-07-23T14:31:10+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-neurology","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"nurl","sideBox":"Learn more about [BMC Neurology](http://bmcneurol.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/nurl","title":"BMC Neurology","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"49a1e1d3-0e56-4465-a78e-9ade697d2322","owner":[],"postedDate":"September 4th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"under-review","subjectAreas":[],"tags":[],"updatedAt":"2024-10-17T06:08:16+00:00","versionOfRecord":[],"versionCreatedAt":"2024-09-04 23:22:18","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-4789563","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4789563","identity":"rs-4789563","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}