Wegener granulomatosis manifesting as meningitis

Case report

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✓ The authors describe a unique presentation of Wegener granulomatosis (WG) manifesting predominantly as meningitis. Magnetic resonance imaging demonstrated diffuse meningeal enhancement, including the pia mater, in a 28-yearold man with meningitis. A diagnosis of atypical WG was based on the findings of a dural biopsy sample and an elevated cytoplasmic antineutrophil cytoplasmic antibody (cANCA) titer, although the patient did not have any of the lesions common to WG. Immunosuppressive therapy was quite effective. With treatment, the meningeal enhancement resolved and the cANCA titer normalized. Meningeal granulomatosis as the sole lesion in WG has never been reported in the literature. This atypical course of WG should be noted.

Article Information

Address reprint requests to: Takao Yasuhara, M.D., Department of Neurological Surgery, Okayama University Graduate School of Medicine and Dentistry, 2–5–1 Shikata-cho, Okayama, 700–8558, Japan. email: tyasu37@cc.okayama-u.ac.jp.

© AANS, except where prohibited by US copyright law.

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Figures

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    Axial T1-weighted MR imaging studies obtained at admission without (left) and with (right) Gd enhancement, revealing diffuse dural thickening extending to the sulcus, which is consistent with pial involvement.

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    Photomicrograph of a biopsy sample showing a granuloma involving necrosis (arrow) beneath the dura mater (arrowheads). H & E, original magnification × 40.

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    Axial Gd-enhanced T1-weighted MR imaging studies obtained 1 month (left) and 3 months (right) after the initiation of immunosuppressive therapy. The images show a gradual resolution of the meningeal enhancement, especially in the pia mater.

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