{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T06:03:30Z","timestamp":1747202610631,"version":"3.40.5"},"reference-count":23,"publisher":"Ordem dos Medicos","issue":"6","license":[{"start":{"date-parts":[[2024,4,26]],"date-time":"2024-04-26T00:00:00Z","timestamp":1714089600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Med Port"],"abstract":"<jats:p>Introduction: Neurological involvement in immunoglobulin G4-related disease (IgG4-RD) is increasingly recognized. Its diagnosis can be challenging due to clinical mimics and difficulty in obtaining nervous system biopsies. The aim of this study was to describe a cohort of neurological IgG4-RD patients.Methods: Patients were recruited from a neuroimmunology tertiary center. Clinical, laboratory, neuroimaging and histological data were reviewed. Results: Fifteen patients (60% women), with a median age of 53 years (48.5 \u2013 65.0) were included: 13 (86.7%) classified as possible IgG4-RD, one (6.7%) as probable and one (6.7%) as definitive. The most common neurological phenotypes were meningoencephalitis (26.7%), orbital pseudotumor (13.3%), cranial neuropathies (13.3%), peripheral neuropathy (13.3%), and longitudinally extensive transverse myelitis (LTEM) (13.3%). Median serum IgG4 concentration was 191.5 (145.0 \u2013 212.0) mg\/dL. Seven in 14 patients had CSF pleocytosis (50.0%) and oligoclonal bands restricted to the intrathecal compartment, while most cases presented elevated CSF proteins (64.3%). Magnetic resonance imaging abnormalities included white matter lesions in four (26.7%), hypertrophic pachymeningitis in two (13.3%), and LETM in two (13.3%). Two patients had biopsy-proven IgG4-RD in extra-neurological sites.Conclusion: This study highlights the phenotypical variability of the neurological IgG4-RD. Biopsy inaccessibility reinforces the importance of new criteria for the diagnosis of this subset of patients.<\/jats:p>","DOI":"10.20344\/amp.20767","type":"journal-article","created":{"date-parts":[[2024,4,26]],"date-time":"2024-04-26T15:21:17Z","timestamp":1714144877000},"page":"429-435","source":"Crossref","is-referenced-by-count":0,"title":["Neurological Involvement in a Portuguese Cohort of IgG4-Related Disease"],"prefix":"10.20344","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4212-906X","authenticated-orcid":false,"given":"Jo\u00e3o","family":"Moura","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Jo\u00e3o","family":"Malaquias","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Firmina","family":"Jorge","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eduarda","family":"Pinto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana","family":"Sardoeira","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"In\u00eas","family":"Laranjinha","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vanessa","family":"Oliveira","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana Paula","family":"Sousa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joana","family":"Dam\u00e1sio","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lu\u00eds","family":"Maia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nuno","family":"Vila-Ch\u00e3","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raquel","family":"Sam\u00f5es","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ricardo","family":"Taipa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana","family":"Martins da Silva","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ernestina","family":"Santos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"8464","published-online":{"date-parts":[[2024,4,26]]},"reference":[{"key":"5857","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(17)30471-4"},{"key":"5858","doi-asserted-by":"publisher","DOI":"10.1016\/j.medcle.2018.05.005"},{"key":"5859","doi-asserted-by":"publisher","DOI":"10.1016\/j.rdc.2017.06.010"},{"key":"5860","doi-asserted-by":"publisher","DOI":"10.1007\/s11940-017-0450-9"},{"key":"5861","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2012.72"},{"key":"5862","doi-asserted-by":"publisher","DOI":"10.1080\/14397595.2020.1859710"},{"key":"5863","doi-asserted-by":"publisher","DOI":"10.1002\/art.41120"},{"key":"5864","doi-asserted-by":"publisher","DOI":"10.1007\/s10072-018-3689-3"},{"key":"5865","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2014.243"},{"key":"5866","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-010-0746-2"},{"key":"5867","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000568"},{"key":"5868","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2019.05.003"},{"key":"5869","doi-asserted-by":"publisher","DOI":"10.1007\/s12026-016-8863-1"},{"key":"5870","doi-asserted-by":"crossref","unstructured":"Mehta SH, Switzer JA, Biddinger P, Rojiani AM. 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