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We report a case of localized convexity hypertrophic pachymeningitis (HP)associated with aortitis. HP is considered to be one type of IgG4-related systemic disease as well as aortitis. A 38-year-old man with aortitis was admitted to our department due to generalized convulsive seizures. MRI revealed a localized mass like thickening of the convexity dura mater resembling meningioma. Because MRI showed rapid growth of the mass, diagnostic removal of the mass was performed to rule out the malignant tumor. Pathological examination showed hyalinization and fibrillation with infiltration of lymphocytes and IgG4-positive plasma cells. These findings suggest that our case is an IgG4-related systemic disease. However several combinations of IgG4-related diseases have been reported, reports of HP associated with aortitis are rare. In addition, this is the first case of localized convexity HP. We propose that resection of a symptomatic localized lesion is considered to be a therapeutic alternation for HP. (Received September 5, 2014;accepted June 12, 2015)

A Case of Convexity Hypertrophic Pachymeningitis associated with Aortitis