Neurological Involvement in a Portuguese Cohort of IgG4-Related Disease
| Title: | Neurological Involvement in a Portuguese Cohort of IgG4-Related Disease |
|---|---|
| Authors: | João Moura, Maria João Malaquias, Firmina Jorge, Eduarda Pinto, Ana Sardoeira, Inês Laranjinha, Vanessa Oliveira, Ana Paula Sousa, Joana Damásio, Luís Maia, Nuno Vila-Chã, Raquel Samões, Ricardo Taipa, Ana Martins da Silva, Ernestina Santos |
| Source: | Acta Médica Portuguesa (2024) |
| Publisher Information: | Ordem dos Médicos, 2024. |
| Publication Year: | 2024 |
| Collection: | LCC:Medicine LCC:Medicine (General) |
| Subject Terms: | Immunoglobulin G, Immunoglobulin G4-Related Disease/diagnosis, Nervous System Diseases, Medicine, Medicine (General), R5-920 |
| More Details: | 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 – 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 – 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. |
| Document Type: | article |
| File Description: | electronic resource |
| Language: | English Portuguese |
| ISSN: | 0870-399X 1646-0758 |
| Relation: | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/20767; https://doaj.org/toc/0870-399X; https://doaj.org/toc/1646-0758 |
| DOI: | 10.20344/amp.20767 |
| Access URL: | https://doaj.org/article/d8f283e9be9b4e39a241e4cd56a4f4af |
| Accession Number: | edsdoj.8f283e9be9b4e39a241e4cd56a4f4af |
| Database: | Directory of Open Access Journals |
| ISSN: | 0870399X 16460758 |
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| DOI: | 10.20344/amp.20767 |
| Published in: | Acta Médica Portuguesa |
| Language: | English Portuguese |