Yoo Il-Han, Choi Sang Tae, Choi Seong-Ho, Kim Jeong-Min, Ahn Suk-Won
Department of Neurology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul 06973, Korea.
Division of Rheumatology, Department of Internal Medicine, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul 06973, Korea.
Exp Neurobiol. 2017 Jun;26(3):168-171. doi: 10.5607/en.2017.26.3.168. Epub 2017 Jun 14.
Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.
嗜酸性肉芽肿性多血管炎(EGPA)是一种免疫相关的全身性疾病,由影响多个器官系统的血管炎引起。其特征为哮喘、发热、嗜酸性粒细胞增多、心脏问题、肾损伤和周围神经病变。在本报告中,我们描述了一名患有EGPA并并发脑梗死和类似吉兰 - 巴雷综合征(GBS)的急性多发性神经病的患者。一名46岁男性出现快速进展的步态障碍、肌肉无力以及四肢刺痛感。神经传导研究显示四肢均存在感觉运动性多发性神经病,脑脊液检查显示蛋白细胞分离。此外,使用液体衰减反转恢复序列和扩散加权磁共振成像(MRI)的脑部MRI显示,右侧尾状核在T1加权MRI上有钆增强的高信号强度病变。在进行实验室检查、鼻窦计算机断层扫描和鼻涂片检查后,该患者被诊断为EGPA,并接受了高剂量糖皮质激素和口服环磷酰胺治疗。总之,我们的研究结果表明,当患者出现类似GBS的快速进展性多发性神经病并伴有不寻常的全身症状或脑部病变时,应考虑EGPA的诊断。