{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,21]],"date-time":"2025-09-21T18:09:23Z","timestamp":1758478163516,"version":"3.40.5"},"reference-count":13,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2019,12,19]],"date-time":"2019-12-19T00:00:00Z","timestamp":1576713600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Peripheral Nervous Sys"],"published-print":{"date-parts":[[2020,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>To describe clinical features, disease course, treatment response, and sural nerve biopsy findings in a patient with chronic sensory ataxic neuropathy, Binet stage A chronic lymphocytic leukemia, and monoclonal IgM\u03bb paraprotein against ganglioside GD1b. During 9 months of hospitalization at two neurologic centers, the patient underwent serial neurologic examinations, neurophysiologic studies, imaging investigations, extensive laboratory work\u2010up, bone marrow, and sural nerve biopsies. The patient had a severe progressive sensory neuropathy accompanied by motor involvement, dysautonomia, and marked bulbar weakness with preserved ocular movements. Conduction studies were characterized by prolonged F\u2010wave minimal latencies, prolonged distal latencies, reduction of compound motor action potentials, and absence of sensory nerve action potentials. Sural nerve biopsy showed endoneurial edema, axonal degeneration, and regeneration, in the absence of cellular inflammation, macrophagic activation, and B\u2010lymphocyte infiltration; no IgM or complement deposition was detected. Myelinated fibers showed redundant\/abnormally thickened myelin, myelin vacuolation, and frank intramyelinic edema with condensed axoplasm. Ultrastructural features included axo\u2010glial detachment, disruption of membrane integrity, and myelin uncompaction. This study shows that monospecific anti\u2010GD1b IgM paraprotein is associated with non\u2010inflammatory nerve damage. We suggest that the loss of myelin and axonal integrity reflects antibody\u2010induced disruption of membrane lipid rafts.<\/jats:p>","DOI":"10.1111\/jns.12359","type":"journal-article","created":{"date-parts":[[2019,12,14]],"date-time":"2019-12-14T11:14:40Z","timestamp":1576322080000},"page":"54-59","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Myelin uncompaction and axo\u2010glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b"],"prefix":"10.1111","volume":"25","author":[{"given":"Matteo","family":"Tagliapietra","sequence":"first","affiliation":[{"name":"Department of Neurosciences, Biomedicine and Movement Sciences University of Verona  Italy"},{"name":"Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gianluigi","family":"Zanusso","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Biomedicine and Movement Sciences University of Verona  Italy"},{"name":"Azienda Ospedaliera Universitaria Integrata  Verona 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":"Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Riccardo","family":"Orlandi","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Biomedicine and Movement Sciences University of Verona  Italy"},{"name":"Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laura","family":"Bertolasi","sequence":"additional","affiliation":[{"name":"Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tiziana","family":"Cavallaro","sequence":"additional","affiliation":[{"name":"Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Salvatore","family":"Monaco","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Biomedicine and Movement Sciences University of Verona  Italy"},{"name":"Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,12,19]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.it.2013.05.001"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.24192"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.expneurol.2011.11.039"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/124.10.1968"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.42.3.686"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.22167"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4598(199910)22:10<1426::AID-MUS13>3.0.CO;2-H"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-018-8853-4"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2007.120444"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awx012"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.2095-18.2018"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23256"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-4-431-55594-0_10"}],"container-title":["Journal of the Peripheral Nervous System"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjns.12359","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jns.12359","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/jns.12359","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jns.12359","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,6]],"date-time":"2023-09-06T20:53:22Z","timestamp":1694033602000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jns.12359"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,12,19]]},"references-count":13,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,3]]}},"alternative-id":["10.1111\/jns.12359"],"URL":"https:\/\/doi.org\/10.1111\/jns.12359","archive":["Portico"],"relation":{},"ISSN":["1085-9489","1529-8027"],"issn-type":[{"type":"print","value":"1085-9489"},{"type":"electronic","value":"1529-8027"}],"subject":[],"published":{"date-parts":[[2019,12,19]]},"assertion":[{"value":"2019-10-11","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-12-04","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-12-19","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}