{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:32:19Z","timestamp":1760189539556},"reference-count":32,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,3,18]],"date-time":"2017-03-18T00:00:00Z","timestamp":1489795200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2018,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p><jats:italic>Introduction<\/jats:italic>: Autosomal dominant haploinsufficiency of <jats:italic>GATA2<\/jats:italic> causes monocytopenia and natural killer cell lymphopenia, resulting in predisposition to mycobacterial, fungal, and viral infections. <jats:italic>Methods<\/jats:italic>: Herein we report on the clinical, serologic, electrophysiologic, and pathologic evaluations of a 29\u2010year\u2010old woman with <jats:italic>GATA2<\/jats:italic> haploinsufficiency and active Epstein\u2013Barr virus (EBV) infection complicated by subacute painful neuropathy. <jats:italic>Results<\/jats:italic>: Nerve conduction and electromyography studies showed predominantly demyelinating sensorimotor polyradiculoneuropathy. Lumbar spine MRI showed thickening and enhancement of the cauda equina nerve roots. Serum and cerebrospinal fluid anti\u2010IgG and IgM EBV capsid and nucleic acid antibodies were positive. Sural nerve biopsy showed microvasculitis and an increased frequency of fibers with segmental demyelination. Intravenous immunoglobulin and steroids improved the patient's neuropathy. <jats:italic>Conclusion: GATA2<\/jats:italic> mutation\u2013related immunodeficiency may predispose to EBV\u2010associated subacute demyelinating polyradiculoneuropathy by both viral susceptibility and immune dysregulation. In patients who present in this manner, immunodeficiency syndromes should be considered when lymphomatous infiltration is excluded. Immunotherapy can be helpful. <jats:italic>Muscle Nerve<\/jats:italic> <jats:bold>57<\/jats:bold>: 150\u2013156, 2018<\/jats:p>","DOI":"10.1002\/mus.25581","type":"journal-article","created":{"date-parts":[[2017,1,17]],"date-time":"2017-01-17T10:40:38Z","timestamp":1484649638000},"page":"150-156","source":"Crossref","is-referenced-by-count":7,"title":["Subacute demyelinating polyradiculoneuropathy complicating Epstein\u2013Barr virus infection in <i>GATA2<\/i> haploinsufficiency"],"prefix":"10.1002","volume":"57","author":[{"given":"Mohamed","family":"Kazamel","sequence":"first","affiliation":[{"name":"Department of Neurology Mayo Clinic 200 First Street SW, Rochester Minnesota 55905 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christopher J.","family":"Klein","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic 200 First Street SW, Rochester Minnesota 55905 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eduardo E.","family":"Benarroch","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic 200 First Street SW, Rochester Minnesota 55905 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mrinal M.","family":"Patnaik","sequence":"additional","affiliation":[{"name":"Department of Hematology Mayo Clinic Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer A.","family":"Tracy","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic 200 First Street SW, Rochester Minnesota 55905 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,3,18]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp312"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/00019052-200006000-00012"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.51.4.1110"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.56.6.758"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0000344"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1750-3639.2001.tb00413.x"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2009.11.003"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.3109\/13550289709031193"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-199511000-00011"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01607163"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2009-03-208629"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-05-356352"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20101459"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-06-360313"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.913"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.923"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/cam4.384"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-07-515528"},{"key":"e_1_2_5_20_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-09-453969"},{"key":"e_1_2_5_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4598(199911)22:11<1607::AID-MUS21>3.0.CO;2-J"},{"key":"e_1_2_5_22_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000150582.80346.2C"},{"key":"e_1_2_5_23_1","first-page":"357","article-title":"Brachial plexopathy complicating Epstein\u2010Barr virus infection in an adult","volume":"29","author":"Dodig D","year":"2010","journal-title":"Acta Myol"},{"key":"e_1_2_5_24_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2377-13-96"},{"key":"e_1_2_5_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410400316"},{"key":"e_1_2_5_26_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-004-0897-0"},{"key":"e_1_2_5_27_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2559.1998.00377.x"},{"key":"e_1_2_5_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0889-8588(05)70521-9"},{"key":"e_1_2_5_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2004.11.009"},{"key":"e_1_2_5_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00993264"},{"key":"e_1_2_5_31_1","doi-asserted-by":"crossref","first-page":"1310","DOI":"10.4049\/jimmunol.122.4.1310","article-title":"Polyclonal immunoglobulin secretion by human B lymphocytes exposed to Epstein\u2010Barr virus in vitro","volume":"122","author":"Kirchner H","year":"1979","journal-title":"J Immunol"},{"key":"e_1_2_5_32_1","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/59.6.525"},{"key":"e_1_2_5_33_1","first-page":"297","volume-title":"Handbook of neurovirology","author":"Junker AK","year":"1994"}],"container-title":["Muscle &amp; 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