IgG4-related disease presenting with headache and papilloedema

Description

A previously healthy man aged 57 years presented with a 4-week duration of predominant decubitus daily holocranial headache after minor head injury. Apart from unintentional weight loss of 8 pounds, there were no associated constitutional or systemic manifestations. The general and neurological examination was unremarkable. The funduscopy showed bilateral grade 2 papilloedema. Brain magnetic resonance (MRI) findings are shown in figure 1A–E. The brain MRI showed the presence of diffuse dural thickening (figure 1B–E) hypointense on unenhanced T1 imaging (figure 1B–F), with dural enhancement on T1-weighed imaging (figure 1B–F). The opening cerebrospinal fluid (CSF) pressure was 380 mm  Hg. With the exception of serum-elevated IgG4 immunoglobulin (IgG4=302 mg/dL) and elevated CSF protein level (68 mg/dL), the wide and extensive complementary study including body positron emission tomography scan, laboratory blood/CSF work-up was negative, excluding neoplastic, infectious, autoimmune and collagen vascular disorders. Meningeal biopsy showed fibrous...

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