Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Granulomatosis with Polyangiitis Complicated by Hypertrophic Pachymeningitis Presenting with Simultaneous Multiple Intracerebral Hemorrhages
Eri KatoKoichiro TaharaHaeru HayashiAki ShojiHiroaki MoriTetsuji Sawada
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JOURNAL OPEN ACCESS

2018 Volume 57 Issue 8 Pages 1167-1172

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Abstract

Central nervous system (CNS) involvement in granulomatosis with polyangiitis (GPA), including pachymeningitis and CNS vasculitis, is uncommon. Although intracerebral hemorrhage (ICH) has been reported in GPA, simultaneous multiple ICH (SMICH) is rare. We describe the case of a 50-year-old woman with a history of a limited form of GPA with chronic pachymeningitis who presented with acute-onset headache accompanied by nausea and vomiting, and who developed consciousness impairment. Computed tomography revealed bilateral subcortical ICH. Sinus thrombosis was not apparent on angiography. The patient was treated with high-dose corticosteroid therapy. The cause of the steroid-responsive SMICH in this case was unknown, but it might have been CNS vasculitis. Patients with GPA may present with SMICH, which is considered an indication for immunosuppressive therapy.

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© 2018 by The Japanese Society of Internal Medicine
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