Document resource
A 37-year-old woman had a 3-week history of headache with brief intermittent episodes of confusion and collapse. There were no neurological signs. Serum IgG4 concentration was normal (0.24 g/L, normal 0.04–0.86) as were an electroencephalogram, cerebrospinal fluid analysis and whole body fluorodeoxyglucose positron emission tomography. MR scan of brain with venography showed parafalcine dural thickening with occlusion of the superior sagittal sinus (figure 1). Dural biopsy confirmed the diagnosis of IgG4-related pachymeningitis (figure 2) with secondary cerebral venous sinus thrombosis. Her symptoms resolved with levetiracetam, anticoagulation (warfarin) and prednisolone (40 mg orally one time per day, weaning to the maintenance of 5 mg one time per day). The patient did not wish to take a steroid-sparing agent. There has been no subsequent clinical or radiological evolution over 3 years.