Garcin Syndrome in a Patient with Hypertrophic Pachymeningitis Following Otitis Media with Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (OMAAV).
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引用次数: 0
Abstract
A 72-year-old Japanese woman presented to our hospital with progressive hearing loss and dysphagia. Blood tests revealed elevated C-reactive protein and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA). Contrast-enhanced magnetic resonance imaging of the head showed hypertrophic pachymeningitis of the left middle cranial fossa with compression of the cranial nerves, including the trigeminal (V), facial (VII), glossopharyngeal (IX), and vagal (X) nerves, resulting in cranial nerve palsy. She was diagnosed with Garcin syndrome associated with otitis media with ANCA-associated vasculitis (OMAAV) and treated with high-dose glucocorticoid therapy followed by intravenous cyclophosphamide and rituximab. Therefore, OMAAV should be considered in the differential diagnosis of refractory otitis media with unilateral cranial nerve involvement.
一名 72 岁的日本妇女因进行性听力下降和吞咽困难来我院就诊。血液检查显示 C 反应蛋白和髓过氧化物酶-抗中性粒细胞胞浆抗体（MPO-ANCA）升高。头部对比增强磁共振成像显示，左侧中颅窝肥厚性咽膜炎压迫颅神经，包括三叉神经（V）、面神经（VII）、舌咽神经（IX）和迷走神经（X），导致颅神经麻痹。她被诊断为中耳炎伴 ANCA 相关血管炎（OMAAV）的加钦综合征，并接受了大剂量糖皮质激素治疗，随后静脉注射环磷酰胺和利妥昔单抗。因此，在鉴别诊断单侧颅神经受累的难治性中耳炎时，应考虑 OMAAV。
期刊介绍：
Internal Medicine is an open-access online only journal published monthly by the Japanese Society of Internal Medicine.
Articles must be prepared in accordance with "The Uniform Requirements for Manuscripts Submitted to Biomedical Journals (see Annals of Internal Medicine 108: 258-265, 1988), must be contributed solely to the Internal Medicine, and become the property of the Japanese Society of Internal Medicine. Statements contained therein are the responsibility of the author(s). The Society reserves copyright and renewal on all published material and such material may not be reproduced in any form without the written permission of the Society.
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