Norman James, Pande Ira, Taylor Timothy, Gran Bruno
Department of Neurology, Nottingham University Hospitals NHS Trust, Queen's Medical Centre Campus, Nottingham NG7 2UH, UK.
Department of Rheumatology, Nottingham University Hospitals NHS Trust, Queen's Medical Centre Campus, Nottingham NG7 2UH, UK.
Case Rep Neurol Med. 2014;2014:914530. doi: 10.1155/2014/914530. Epub 2014 May 13.
Neurological manifestations of GPA are common, most frequently as a peripheral neuropathy. Cerebritis as a principal presentation is extremely rare. We report a patient who presented with subacute progression of ataxia, confusion, and vacant episodes. An MRI of her brain showed bilateral signal abnormalities in the cingulate and superior sagittal gyrus while a staging CT revealed a mass in the right upper lobe of the patient's lung with a satellite nodule. C-ANCA antibodies specific for PR3 at high titres were positive and a diagnosis of GPA was made. The patient was commenced on intravenous methylprednisolone followed by cyclophosphamide and responded well to treatment. GPA is a rare and treatable differential diagnosis for confused patients with acute or subacute neurological features and unusual MRI findings.
显微镜下多血管炎（GPA）的神经表现很常见，最常见的是周围神经病变。以脑脊髓炎作为主要表现极为罕见。我们报告了一名患者，其出现共济失调、意识模糊和失神发作的亚急性进展。她的脑部MRI显示扣带回和上矢状窦回出现双侧信号异常，而分期CT显示患者右肺上叶有一个肿块及一个卫星结节。针对蛋白酶3（PR3）的高滴度C-ANCA抗体呈阳性，从而确诊为GPA。患者开始接受静脉注射甲泼尼龙，随后使用环磷酰胺，治疗反应良好。对于具有急性或亚急性神经特征且MRI表现异常的意识模糊患者，GPA是一种罕见但可治疗的鉴别诊断。
