{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T08:23:53Z","timestamp":1740126233121,"version":"3.37.3"},"reference-count":24,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2017,11,14]],"date-time":"2017-11-14T00:00:00Z","timestamp":1510617600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100003710","name":"Korea Health Industry Development Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003710","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2018,4]]},"abstract":"<jats:p>Granulomatosis with polyangiitis (GPA), previously known as Wegener\u2019s granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti\u2010neutrophil cytoplasmic antibody (ANCA)\u2010negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50\u2010year\u2010old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms. Since multiple neurologic deficits were the first manifestation and there were no pulmonary or renal symptoms, our patient was treated for 1\u2009year for suspected infectious or inflammatory diseases such as non\u2010tuberculous mycobacterial infection or immunoglobulin G4 (IgG4)\u2010related disease with brain involvement; however, his symptoms would wax and wane. Reanalysis of the laboratory findings including biopsies, finally revealed this case to be compatible with GPA. Here, we report a case of GPA, focusing on the diagnostic pitfalls.<\/jats:p>","DOI":"10.1111\/neup.12437","type":"journal-article","created":{"date-parts":[[2017,11,15]],"date-time":"2017-11-15T03:02:38Z","timestamp":1510714958000},"page":"192-197","source":"Crossref","is-referenced-by-count":5,"title":["Seronegative granulomatosis with polyangiitis presenting with multiple cranial nerve palsies"],"prefix":"10.1111","volume":"38","author":[{"given":"Eunhye","family":"Lee","sequence":"first","affiliation":[{"name":"Department of Pathology Seoul National University Hospital, College of Medicine  Seoul Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jynkyun","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine (Rheumatology) Seoul National University Hospital, College of Medicine  Seoul Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seung Hong","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Radiology Seoul National University Hospital, College of Medicine  Seoul Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8681-1597","authenticated-orcid":false,"given":"Sung\u2010Hye","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Pathology Seoul National University Hospital, College of Medicine  Seoul Republic of Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,11,14]]},"reference":[{"key":"e_1_2_6_2_1","first-page":"177","article-title":"Update on the diagnosis and treatment of Wegener\u2019s granulomatosis","volume":"46","author":"Langford CA","year":"2001","journal-title":"Adv Intern Med"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.37715"},{"key":"e_1_2_6_4_1","article-title":"Paediatric anti\u2010neutrophil 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