{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T01:41:42Z","timestamp":1790991702411,"version":"4.1.0"},"reference-count":75,"publisher":"Georg Thieme Verlag KG","issue":"06","license":[{"start":{"date-parts":[[2024,2,7]],"date-time":"2024-02-07T00:00:00Z","timestamp":1707264000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arq Neuropsiquiatr"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Background\u2003Patients with anti-MAG neuropathy present with distal demyelinating polyneuropathy, IgM monoclonal gammopathy, and elevated titers of anti-MAG antibodies.<\/jats:p>\n                  <jats:p>Objective\u2003This paper reviews what is known about the clinical presentation, course, pathophysiology, and treatment of anti-MAG neuropathy, with considerations for the design of therapeutic trials.<\/jats:p>\n                  <jats:p>Methods\u2003A literature review of the medical and scientific literature related to anti-MAG neuropathy, and the design of therapeutic clinical trials in peripheral neuropathy.<\/jats:p>\n                  <jats:p>Results\u2003Anti-MAG neuropathy can remain indolent for many years but then enter a progressive phase. Highly elevated antibody titers are diagnostic, but intermediate titers can also occur in chronic inflammatory demyelinating polyneuropathy (CIDP). The peripheral nerves can become inexcitable, thereby masking the demyelinating abnormalities. There is good evidence that the anti-MAG antibodies cause neuropathy. Reduction of the autoantibody concentration by agents that target B-cells was reported to result in clinical improvement in case series and uncontrolled trials, but not in controlled clinical trials, probably due to inadequate trial design.<\/jats:p>\n                  <jats:p>Conclusion\u2003We propose that therapeutic trials for anti-MAG neuropathy include patients with the typical presentation, some degree of weakness, highly elevated anti-MAG antibody titers, and at least one nerve exhibiting demyelinating range abnormalities. Treatment with one or a combination of anti-B-cell agents would aim at reducing the autoantibody concentration by at least 60%. A trial duration of 2 years may be required to show efficacy. The neuropathy impairment score of the lower extremities (NIS-LL) plus the Lower Limb Function (LLF) score would be a suitable primary outcome measure.<\/jats:p>","DOI":"10.1055\/s-0043-1777728","type":"journal-article","created":{"date-parts":[[2024,2,7]],"date-time":"2024-02-07T18:35:22Z","timestamp":1707330922000},"page":"001-007","source":"Crossref","is-referenced-by-count":11,"title":["Anti-MAG neuropathy: historical aspects, clinical-pathological correlations, and considerations for future therapeutical trials"],"prefix":"10.1055","volume":"82","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5330-0371","authenticated-orcid":false,"given":"Norman","family":"Latov","sequence":"additional","affiliation":[{"name":"Weil Medical College of Cornell University, Peripheral Neuropathy Center, New York, New York, United States."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1812-8331","authenticated-orcid":false,"suffix":"III","given":"Thomas H.","family":"Brannagan","sequence":"additional","affiliation":[{"name":"Columbia University, Vagelos College of Physicians and Surgeons, Peripheral Neuropathy Center, Department of Neurology, New York, New York, United States."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9654-7849","authenticated-orcid":false,"given":"Howard W.","family":"Sander","sequence":"additional","affiliation":[{"name":"New York University, Department of Neurology, New York, New York, United States."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8957-5796","authenticated-orcid":false,"given":"Francisco de Assis Aquino","family":"Gondim","sequence":"additional","affiliation":[{"name":"Universidade Federal do Cear\u00e1, Departamento de Medicina Cl\u00ednica, Servi\u00e7o de Neurologia, Fortaleza CE, Brazil."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2024,2,7]]},"reference":[{"issue":"11","key":"ref1","doi-asserted-by":"publisher","first-page":"618","DOI":"10.1056\/NEJM198009113031105","article-title":"Plasma-cell dyscrasia and peripheral neuropathy with a monoclonal antibody to peripheral-nerve myelin","volume":"303","author":"N Latov","year":"1980","journal-title":"N Engl J Med"},{"issue":"11","key":"ref2","doi-asserted-by":"publisher","first-page":"7139","DOI":"10.1073\/pnas.78.11.7139","article-title":"Plasma cell dyscrasia and peripheral neuropathy: identification of the myelin antigens that react with human paraproteins","volume":"78","author":"N Latov","year":"1981","journal-title":"Proc Natl Acad Sci U S 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