{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T15:02:13Z","timestamp":1777561333964,"version":"3.51.4"},"reference-count":5,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,3,10]],"date-time":"2023-03-10T00:00:00Z","timestamp":1678406400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Peripheral Nervous Sys"],"published-print":{"date-parts":[[2023,6]]},"abstract":"Abstract<\/jats:title>Aim<\/jats:title>Neuropathy is a frequent complication of Waldenstr\u00f6m's macroglobulinemia (WM), the most common being a demyelinating polyneuropathy with anti\u2010myelin associated glycoprotein (MAG) antibodies, but also cryoglobulins, vasculitis, neurolymphomatosis, and amyloidosis. We describe a patient with IgM\/kappa WM who presented with a severe, not length\u2010dependent, peripheral neuropathy as clinical onset of IgM\/kappa\u2010related amyloidosis.<\/jats:p><\/jats:sec>Methods<\/jats:title>A 69\u2010year\u2010old woman came to our attention for weight loss, gait imbalance and sensory loss at upper limbs. In her medical history, she was in hematological follow\u2010up for WM, and had undergone left carpal tunnel release. At neurological evaluation she had weakness and loss of sensation at upper limbs up to the elbows, more at the left side, gait was unsteady with right foot drop. Hypotrophy and areflexia were present at four limbs. Sensory loss and vibration sense were dramatically reduced. She underwent extensive diagnostic workup.<\/jats:p><\/jats:sec>Results<\/jats:title>Laboratory workup revealed an IgM\/kappa monoclonal paraprotein of 16\u2009g\/L and increased NT\u2010proBNP; anti\u2010MAG antibodies were absent. Bone marrow biopsy demonstrated a population of neoplastic B\u2010lymphocytes. Total\u2010body CT scan and echocardiogram were negative. Neurophysiology revealed a symmetric, no length dependent sensory\u2010motor polyneuropathy Periumbilical fat biopsy was positive for amyloid. Sural nerve biopsy detected amyloid in the wall of an epineurial vein.<\/jats:p><\/jats:sec>Conclusions<\/jats:title>This case report describes a rare and unusual manifestation of IgM\u2010related AL amyloidosis in WM. The patient presented with a subacute clinically asymmetric neuropathy with no pain or dysautonomic features as clinical onset of IgM\/kappa\u2010related amyloidosis. Sural nerve biopsy was crucial for the diagnosis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jns.12540","type":"journal-article","created":{"date-parts":[[2023,3,2]],"date-time":"2023-03-02T04:54:21Z","timestamp":1677732861000},"page":"262-265","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Peripheral neuropathy as clinical onset of monoclonal IgM\/k\u2010related amyloidosis"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8035-0200","authenticated-orcid":false,"given":"Chiara","family":"Briani","sequence":"first","affiliation":[{"name":"Department of Neurosciences University of Padova Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3855-5135","authenticated-orcid":false,"given":"Sergio","family":"Ferrari","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences University of Verona Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tamara","family":"Berno","sequence":"additional","affiliation":[{"name":"Hematology and Clinical Immunology Unit, Department of Medicine University of Padova Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Visentin","sequence":"additional","affiliation":[{"name":"Hematology and Clinical Immunology Unit, Department of Medicine University of Padova Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mario","family":"Cacciavillani","sequence":"additional","affiliation":[{"name":"Datamedica Group CEMES SynLab Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tiziana","family":"Cavallaro","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Neuroscience, Biomedicine and Movement Sciences University of Verona Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marny","family":"Fedrigo","sequence":"additional","affiliation":[{"name":"Cardiovascular Pathology, Department of Cardiac, Thoracic, Vascular Sciences and Public Health University of Padova Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefania","family":"Rizzo","sequence":"additional","affiliation":[{"name":"Cardiovascular Pathology, Department of Cardiac, Thoracic, Vascular Sciences and Public Health University of Padova Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandro","family":"Salvalaggio","sequence":"additional","affiliation":[{"name":"Department of Neurosciences University of Padova Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Livio","family":"Trentin","sequence":"additional","affiliation":[{"name":"Hematology and Clinical Immunology Unit, Department of Medicine University of Padova Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Piazza","sequence":"additional","affiliation":[{"name":"Hematology and Clinical Immunology Unit, Department of Medicine University of Padova Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,3,10]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/jns.12295"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.3816\/CLM.2009.n.021"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2015.63.3123"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41375-019-0667-6"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-020-10127-8"}],"container-title":["Journal of the Peripheral Nervous 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