{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,11,22]],"date-time":"2023-11-22T05:05:57Z","timestamp":1700629557719},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2013,6,19]],"date-time":"2013-06-19T00:00:00Z","timestamp":1371600000000},"content-version":"vor","delay-in-days":18,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Peripheral Nervous Sys"],"published-print":{"date-parts":[[2013,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>At age 35, a man with a genetic diagnosis of Charcot\u2010Marie\u2010Tooth disease type 1A (<jats:styled-content style=\"fixed-case\">CMT1A<\/jats:styled-content>) but no family history of neuropathy and no clinical symptoms developed rapidly progressive loss of balance, distal limb numbness, loss of manual dexterity, and hand tremor. Five years later, he walked with support and had mild pes cavus, marked sensory ataxia, severe leg and hand weakness, absent deep tendon reflexes (DTRs), severe sensory loss, and hand tremor. He had dramatically reduced motor nerve conduction velocity (MNCV), strikingly prolonged motor distal latencies, absent sensory action potentials and lower limb compound muscle action potentials. <jats:styled-content style=\"fixed-case\">CMT1A<\/jats:styled-content> duplication was reconfirmed but the dramatic change in his clinical course suggested a superimposed acquired neuropathy. An <jats:styled-content style=\"fixed-case\">IgM<\/jats:styled-content>\u2010kappa monoclonal gammopathy of uncertain significance (MGUS) with high titer anti\u2010myelin associated glycoprotein (anti\u2010MAG) activity was found. Nerve biopsy showed severe loss of myelinated fibers with onion bulbs, no evidence of uncompacted myelin, and few <jats:styled-content style=\"fixed-case\">IgM<\/jats:styled-content> deposits. Rituximab was given and he improved. It is very likely that this is a chance association of two rare and slowly progressive neuropathies; rapidly worsening course may have been due to a \u201cdouble hit\u201d. Interestingly, there are reports of possible superimposition of dysimmune neuropathies on hereditary ones, and the influence of the immune system on inherited neuropathies is matter for debate.<\/jats:p>","DOI":"10.1111\/jns5.12029","type":"journal-article","created":{"date-parts":[[2013,5,24]],"date-time":"2013-05-24T17:47:51Z","timestamp":1369417671000},"page":"185-188","source":"Crossref","is-referenced-by-count":1,"title":["Coexistence of Charcot\u2010Marie\u2010Tooth disease type 1A and anti\u2010MAG neuropathy"],"prefix":"10.1111","volume":"18","author":[{"given":"Giuseppe","family":"Piscosquito","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ettore","family":"Salsano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Ciano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luisa","family":"Palamara","sequence":"additional","affiliation":[{"name":"Division of Neurology and Neuropathology, Department of Diagnostic and Applied Technology IRCCS Foundation, \u201cC. Besta\u201d Neurological Institute Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michela","family":"Morbin","sequence":"additional","affiliation":[{"name":"Division of Neurology and Neuropathology, Department of Diagnostic and Applied Technology IRCCS Foundation, \u201cC. Besta\u201d Neurological Institute Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Davide","family":"Pareyson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,6,19]]},"reference":[{"key":"e_1_2_3_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000335268.70325.33"},{"key":"e_1_2_3_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.21577"},{"key":"e_1_2_3_4_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0029-1237720"},{"key":"e_1_2_3_5_1","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1007\/BF00878529","article-title":"Neuropathy associated with \u201cbenign\u201d anti\u2010myelin\u2010associated glycoprotein IgM gammopathy: clinical, immunological, neurophysiological, pathological findings and response to treatment in 33 cases","volume":"243","author":"Ellie E","year":"1996","journal-title":"J Neurol"},{"key":"e_1_2_3_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh017"},{"key":"e_1_2_3_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410330615"},{"key":"e_1_2_3_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awr283"},{"key":"e_1_2_3_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2009.01.006"},{"key":"e_1_2_3_10_1","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/j.nbd.2012.08.009","article-title":"An essential role of MAG in mediating axon\u2010myelin attachment in Charcot\u2010Marie\u2010Tooth 1A disease","volume":"49","author":"Kinter J","year":"2012","journal-title":"Neurobiol Dis"},{"key":"e_1_2_3_11_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa054494"},{"key":"e_1_2_3_12_1","first-page":"301","article-title":"Peripheral neuropathy and anti\u2010MAG antibodies","volume":"3","author":"Latov N","year":"1988","journal-title":"Crit Rev Neurobiol"},{"issue":"3","key":"e_1_2_3_13_1","first-page":"S73","article-title":"A randomized placebo\u2010controlled TRIAL of Rituximab in IgM anti\u2010Myelin\u2010associated glycoprotein antibody demyelinating neuropathy (RIMAG study)","volume":"16","author":"Leger JM","year":"2011","journal-title":"J Peripher Nerv Syst"},{"key":"e_1_2_3_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(04)00822-1"},{"key":"e_1_2_3_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1529-8027.2011.00350.x"},{"key":"e_1_2_3_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(09)70110-3"},{"key":"e_1_2_3_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199756070-00008"},{"key":"e_1_2_3_18_1","doi-asserted-by":"publisher","DOI":"10.1385\/NMM:8:1-2:175"}],"container-title":["Journal of the Peripheral Nervous System"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjns5.12029","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fjns5.12029","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/jns5.12029","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,22]],"date-time":"2023-11-22T03:44:28Z","timestamp":1700624668000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/jns5.12029"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,6]]},"references-count":17,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2013,6]]}},"alternative-id":["10.1111\/jns5.12029"],"URL":"https:\/\/doi.org\/10.1111\/jns5.12029","archive":["Portico"],"relation":{},"ISSN":["1085-9489","1529-8027"],"issn-type":[{"value":"1085-9489","type":"print"},{"value":"1529-8027","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,6]]}}}