{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T23:52:17Z","timestamp":1783122737693,"version":"3.54.6"},"reference-count":25,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,1,13]],"date-time":"2014-01-13T00:00:00Z","timestamp":1389571200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Pathology"],"published-print":{"date-parts":[[2014,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Central nervous system Whipple's disease (<jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content>) with clinically isolated neurological involvement is a rare condition fatal without an early diagnosis. We aimed to present clinical and neuropathological features of three cases of pre\u2010 or post\u2010mortem polymerase chain reaction confirmed <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content> with distinct clinical presentation, outcome and pathological findings. One patient had an acute onset with spinal and brainstem involvement and died without <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content> diagnosis after 14 weeks. Neuropathology showed extensive inflammatory and necrotizing lesions with abundant foamy periodic\u2010acid\u2010<jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>chiff (<jats:styled-content style=\"fixed-case\">PAS<\/jats:styled-content>)+ macrophages. The second patient had a subacute evolution with late <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content> diagnosis and death occurring 18 months after onset despite antibiotic treatment. Brain examination showed inflammatory lesions in the brainstem, thalamus and cerebellum, and abundant foamy <jats:styled-content style=\"fixed-case\">PAS<\/jats:styled-content>+ macrophages. The third case was diagnosed within 4 weeks of onset and treated with an excellent response. He died after a disease\u2010free period of 24 months of unrelated causes. Neuropathology showed cystic residual lesions devoid of microorganisms without inflammatory reaction. <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">WD<\/jats:styled-content> may have an acute or subacute course with variable response to treatment. Accordingly, subjacent lesions may be those of a severe acute necrotizing encephalitic process or subacute inflammatory lesions involving diencephalic, brainstem, cerebellar and spinal regions. Chronic, cavitary brain lesions may be sequelae of a successful treatment. Early diagnosis should allow appropriate treatment and improve prognosis.<\/jats:p>","DOI":"10.1111\/bpa.12113","type":"journal-article","created":{"date-parts":[[2013,12,10]],"date-time":"2013-12-10T08:42:43Z","timestamp":1386664963000},"page":"230-238","source":"Crossref","is-referenced-by-count":16,"title":["Clinical and Neuropathological Variability in Clinically Isolated Central Nervous System <scp>W<\/scp>hipple's Disease"],"prefix":"10.1111","volume":"24","author":[{"given":"Mircea","family":"Balasa","sequence":"first","affiliation":[{"name":"Neurology Department Hospital Clinic and Institut d'Investigaci\u00f3 Biom\u00e8dica August Pi I Sunyer (IDIBAPS)  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen","family":"Gelpi","sequence":"additional","affiliation":[{"name":"Neurological Tissue Bank Biobanc\u2010Hospital Clinic\u2010Institut d'Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS)  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MJ","family":"Rey","sequence":"additional","affiliation":[{"name":"Neurological Tissue Bank Biobanc\u2010Hospital Clinic\u2010Institut d'Investigacions Biom\u00e8diques August Pi i Sunyer (IDIBAPS)  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordi","family":"Vila","sequence":"additional","affiliation":[{"name":"Microbiology Department Hospital Clinic CRESIB  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Llu\u00eds","family":"Rami\u00f3\u2010Torrent\u00e0","sequence":"additional","affiliation":[{"name":"Neuroinmunology and Multiple Sclerosis Unit Neurology Department IDIBGI Hospital Universitari Dr. Josep Trueta  Girona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Ma","family":"Quiles Granado","sequence":"additional","affiliation":[{"name":"Radiology Department Hospital Universitari Dr. Josep Trueta  Girona 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