CNS involvement in IgG4-RD presenting as DI associated with CVT
Year: 2025
Type: article
Abstract: Immunoglobulin G4-related disease (IgG4-RD) is a multisystem disease. Central nervous system (CNS) involvement in IgG4-RD is rare. We report a case of IgG4-RD with a rare constellation of spectrum of neurological manifestations. A young male presented with right hemicranial headache, bilateral blurred vision, polydipsia, and polyuria. MRI brain revealed hypertrophic cranial pachymeningitis, hypophysitis, and left transverse and sigmoid sinus thrombosis. Serum IgG4 level was elevated suggesting possibility ofIgG4-RD. Patient was managed with steroid, anticoagulant and desmopressin leading to improvement in headache, polydipsia, polyuria, and visual impairment. One must search for associated venous sinus thrombosis and pituitary dysfunction in a case of IgG4-RD with hypertrophic pachymeningitis.
Source: Medical Reports
Authors: Sanchit Shailendra Chouksey, Akansha Jain, Varun Kumar Singh, Ritesh Kumar, Rameshwar Nath Chaurasia, Deepika Joshi, Pratishtha Sengar
Institutions: Institute of Medical Sciences, Banaras Hindu University
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $1,900
OpenAlex ID: W6940563710