IgG4-related disease that presented cranial, cervical, lumbar and sacral hypertrophic pachymeningitis associated with infundibulo-hypophysitis

Abstract

We report a patient of 32-year-old female with central IgG4-related disease. She developed headache and visual disturbance. On examination, she revealed diabetes insipidus, retrobulbar neuritis, hyperreflexia and limb weakness. Her laboratory findings showed serum IgG4 elevation, pleocytosis and protein elevation in cerebrospinal fluid. Chest CT showed a nodular lesion in the S8 of the left lung. Cranial and spinal magnetic resonance images with gadolinium contrast material showed cranial, cervical and lumbosacral hypertrophic pachymeningitis associated with infundibulo-hypophysitis. Pathological findings of the left frontal dura mater revealed lymphoplasmacytic inflammatory cell infiltrate with dense fibrosis. IgG4 immunohistochemistry showed no IgG4 + plasma cells within the inflammatory infiltrate. During treatment with intravenous pulse methylprednisolone followed by oral prednisolone, she revealed recovery of visual acuity with improvement of hypertrophic pachymeningitis and normalization of serum IgG4. This is a first report of IgG4-related hypertrophic pachymeningitis which involved cranial, cervical and lumbosacral regions as well as infundibulo-hypophysitis in a young female.