Combined Hypertrophic Pachymeningitis and Cerebral Venous Thrombosis in a Case of Granulomatosis with Polyangiitis

• Published in: Case reports in neurology Vol. 11; no. 2; pp. 252 - 255
• Main Authors: Kuribayashi, Tadahiro, Manabe, Yasuhiro, Fujiwara, Shunya, Omote, Yoshio, Narai, Hisashi, Abe, Koji
• Format: Journal Article
• Language: English
• Published: Basel, Switzerland S. Karger AG 01.05.2019; Karger Publishers
• Subjects: Antineutrophil cytoplasmic antibody; Case Report; Case reports; Central nervous system; Cerebral venous thrombosis; Ear diseases; Edema; Granulomatosis with polyangiitis; Headaches; Hypertrophic pachymeningitis; Inflammation; Inflammatory bowel disease; Inflammatory diseases; Nervous system; Neurology; NMR; Nuclear magnetic resonance; Patients; Proteins; Sinuses; Thrombosis; Vein & artery diseases; United States > US; Japan; Baltimore Maryland; Granulomatosis with polyangiitis; Antineutrophil cytoplasmic antibody; Hypertrophic pachymeningitis; Cerebral venous thrombosis; Central nervous system
• ISSN: 1662-680X; 1662-680X
• DOI: 10.1159/000502284

We report a rare case of hypertrophic pachymeningitis (HP) and cerebral venous thrombosis associated with proteinase-3-antineutrophil cytoplasmic antibody (PR3-ANCA)-positive granulomatosis with polyangiitis (GPA). A 58-year-old male developed left headache after exudative otitis media. The laboratory data were positive for PR3-ANCA. Brain magnetic resonance imaging revealed bilateral paranasal sinusitis, left frontal lobe edema, and a thick dura mater with abnormal enhancement in the frontotemporal lobe. Magnetic resonance venography detected stenosis of the superior sagittal sinus. The patient was successfully treated with glucocorticoid, cyclophosphamide, and apixaban. Contrast neuroimaging should be performed for patients who present with unexplained headache, especially with middle ear and paranasal inflammation. These symptoms should be considered as GPA-related HP and cerebral venous thrombosis.