Emanuele Cassano, Rosa Iodice, Isabella Di Sarno, Roberta Bencivenga, Fiore Manganelli, Stefano Tozza
{"title":"Acute onset anti-MAG neuropathy and paradoxical worsening to rituximab: a challenging case.","authors":"Emanuele Cassano, Rosa Iodice, Isabella Di Sarno, Roberta Bencivenga, Fiore Manganelli, Stefano Tozza","doi":"10.1007/s10072-025-08087-w","DOIUrl":null,"url":null,"abstract":"<p><strong>Background and aim: </strong>Anti-myelin-associated glycoprotein (anti-MAG) neuropathy is typically a chronic, progressive, predominantly sensory distal and demyelinating neuropathy, with ataxia and postural tremor METHODS AND RESULTS: Herein we describe an atypical case of anti-MAG neuropathy, characterized by acute lower limb weakness and severe ataxia with difficulty in stance and walking, resembling a Guillain-Barrè Syndrome. The presence of disproportionate distal nerve conduction slowing, and an IgM k monoclonal component have arisen the suspect of anti-MAG neuropathy, confirmed by high titer anti-MAG antibody. Rituximab treatment was started, and patient experienced a dramatic clinical worsening which was rescued by Plasma Exchange.</p><p><strong>Interpretation: </strong>We described an atypical case of anti-MAG neuropathy that was challenging in diagnosis and therapeutic management.</p>","PeriodicalId":19191,"journal":{"name":"Neurological Sciences","volume":" ","pages":""},"PeriodicalIF":2.7000,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Neurological Sciences","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s10072-025-08087-w","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Background and aim: Anti-myelin-associated glycoprotein (anti-MAG) neuropathy is typically a chronic, progressive, predominantly sensory distal and demyelinating neuropathy, with ataxia and postural tremor METHODS AND RESULTS: Herein we describe an atypical case of anti-MAG neuropathy, characterized by acute lower limb weakness and severe ataxia with difficulty in stance and walking, resembling a Guillain-Barrè Syndrome. The presence of disproportionate distal nerve conduction slowing, and an IgM k monoclonal component have arisen the suspect of anti-MAG neuropathy, confirmed by high titer anti-MAG antibody. Rituximab treatment was started, and patient experienced a dramatic clinical worsening which was rescued by Plasma Exchange.
Interpretation: We described an atypical case of anti-MAG neuropathy that was challenging in diagnosis and therapeutic management.
期刊介绍:
Neurological Sciences is intended to provide a medium for the communication of results and ideas in the field of neuroscience. The journal welcomes contributions in both the basic and clinical aspects of the neurosciences. The official language of the journal is English. Reports are published in the form of original articles, short communications, editorials, reviews and letters to the editor. Original articles present the results of experimental or clinical studies in the neurosciences, while short communications are succinct reports permitting the rapid publication of novel results. Original contributions may be submitted for the special sections History of Neurology, Health Care and Neurological Digressions - a forum for cultural topics related to the neurosciences. The journal also publishes correspondence book reviews, meeting reports and announcements.