{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,11]],"date-time":"2025-11-11T15:37:57Z","timestamp":1762875477050},"reference-count":44,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2012,10,1]],"date-time":"2012-10-01T00:00:00Z","timestamp":1349049600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain and Behavior"],"published-print":{"date-parts":[[2012,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Whipple disease (<jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content>) is usually a systemic infectious disease that can have central nervous system (<jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>) involvement. <jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content> confined to the <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content> is extremely rare and difficult to diagnose, but can be fatal if not treated in a timely fashion. We present the case of a 42\u2010year\u2010old man with a subacute dementia accompanied by a movement disorder consisting of progressive supranuclear gaze palsy, myoclonus, and ataxia. Our patient lacked the typical magnetic resonance imaging (<jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>) findings reported with isolated <jats:styled-content style=\"fixed-case\">CNS WD<\/jats:styled-content> and had a false\u2010positive cerebrospinal fluid (<jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content>) 14\u20103\u20103 protein. The patient expired, and definitive diagnosis of isolated <jats:styled-content style=\"fixed-case\">CNS WD<\/jats:styled-content> was made by autopsy with characteristic macrophage accumulations found in the brain but not in the gastrointestinal tract. We examine the literature on isolated <jats:styled-content style=\"fixed-case\">CNS WD<\/jats:styled-content> and discuss how these previously unreported findings make a rare diagnosis even more challenging. The reported patient is the first in the literature with tissue diagnosis of isolated <jats:styled-content style=\"fixed-case\">CNS WD<\/jats:styled-content> in the setting of normal brain <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> and positive <jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content> 14\u20103\u20103 protein. Isolated <jats:styled-content style=\"fixed-case\">CNS WD<\/jats:styled-content> should be added to the list of considerations for a false\u2010positive <jats:styled-content style=\"fixed-case\">CSF<\/jats:styled-content> 14\u20103\u20103 protein. Even in the absence of typical <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> lesions, a patient with subacute progressive dementia, supranuclear gaze palsy, and other various neurologic abnormalities should have the diagnosis of isolated <jats:styled-content style=\"fixed-case\">CNS WD<\/jats:styled-content> considered.<\/jats:p>","DOI":"10.1002\/brb3.97","type":"journal-article","created":{"date-parts":[[2012,10,2]],"date-time":"2012-10-02T04:07:05Z","timestamp":1349150825000},"page":"838-843","source":"Crossref","is-referenced-by-count":10,"title":["Isolated <scp>CNS W<\/scp>hipple disease with normal brain <scp>MRI<\/scp> and false\u2010positive <scp>CSF<\/scp> 14\u20103\u20103 protein: a case report and review of the literature"],"prefix":"10.1002","volume":"2","author":[{"given":"Victor W.","family":"Sung","sequence":"first","affiliation":[{"name":"Department of Neurology University of Alabama at Birmingham Birmingham Alabama"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael J.","family":"Lyerly","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Alabama at Birmingham Birmingham Alabama"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kenneth B.","family":"Fallon","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Alabama at Birmingham Birmingham Alabama"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Khurram","family":"Bashir","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Alabama at Birmingham Birmingham Alabama"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2012,10]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.3171\/jns.2002.97.4.0988"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.68.1.2"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000277047.22403.8d"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1080\/00365540601115953"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2008.04.014"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A2089"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.56.11.1528"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00596579"},{"key":"e_1_2_5_10_1","first-page":"225","article-title":"A light\u2010 and electron\u2010microscopic study of bacterial invasion in Whipple's disease","volume":"51","author":"Dobbins W. 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