CNS involvement in IgG4-RD presenting as DI associated with CVT

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.