Oommen Abel T, Gujjar Arunodaya R, Al-Jahdhami Suad, Al-Asmi Abdullah R, Sajjad Ali
Department of Clinical Physiology, Sultan Qaboos University Hospital, University Medical City, Muscat, Oman.
Department of Medicine, Sultan Qaboos University Hospital, University Medical City, Muscat, Oman.
Sultan Qaboos Univ Med J. 2025 May 2;25(1):282-287. doi: 10.18295/2075-0528.2820.
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) usually manifests as systemic vasculitis. AAV with central nervous system involvement in the form of mesenrhombencephalitis and peripheral neuropathy is extremely rare. We report a 59-year-old male patient who presented to a tertiary care hospital in Muscat, Oman, in 2024. He had a subacute presentation of altered sensorium, multiple cranial nerve palsy and ataxia with 3 months history of bilateral foot drop. Brain imaging was suggestive of mesenrhombencephalitis with subarachnoid and intraparenchymal haemorrhage. Nerve conduction studies revealed sensorimotor axonal neuropathy. Vasculitis profile showed perinuclear-ANCA positivity and the nerve biopsy was suggestive of vasculitic neuropathy. He was treated with intravenous steroids and maintained on rituximab. His neurological deficits improved with residual bilateral foot drop.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）通常表现为系统性血管炎。以中脑菱形窝脑炎和周围神经病变形式累及中枢神经系统的AAV极为罕见。我们报告一例2024年就诊于阿曼马斯喀特一家三级护理医院的59岁男性患者。他呈亚急性起病，有意识改变、多组颅神经麻痹和共济失调，伴有3个月双侧足下垂病史。脑部影像学检查提示中脑菱形窝脑炎伴蛛网膜下腔和脑实质内出血。神经传导研究显示感觉运动性轴索性神经病变。血管炎相关检查显示核周型ANCA阳性，神经活检提示血管炎性神经病变。他接受了静脉注射类固醇治疗，并持续使用利妥昔单抗。其神经功能缺损有所改善，但仍遗留双侧足下垂。
