From the Departments of Neurology (M.A.S., B.M.C., A.L.G.Q., K.D.F.L., H.S.T., M.F.S., A.M.B.) and Department of Neuroradiology (C.M.S.C.), Beneficência Portuguesa de São Paulo, Brazil.
Neurol Neuroimmunol Neuroinflamm. 2023 Apr 25;10(4). doi: 10.1212/NXI.0000000000200113. Print 2023 Jul.
We report a case of biopsy-proven giant cell arteritis after an initial presentation of area postrema syndrome.
A 65-year-old man was evaluated using MRI, temporal artery biopsy, and ultrasound.
The patient presented with refractory nausea, vomiting, and hiccups that caused weight loss without any other neurologic or clinical symptoms. His MRI scan 15 days later revealed a hyperintense sign on the area postrema with no abnormal diffusion or contrast enhancement, compatible with isolated area postrema syndrome. An extensive workup for inflammation and other etiologies including neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody disorder, and multiple sclerosis (MS) showed negative results. The patient responded to treatment with methylprednisolone. Two months after the initial clinical manifestation, the patient developed fatigue, headache, and scalp tenderness. He was diagnosed with giant cell arteritis after ultrasonography and biopsy were performed. He responded well to oral glucocorticoids and had only 1 relapse during tapering. He has not had arteritic ischemic optic neuropathy or any new episodes of area postrema syndrome.
This case demonstrates the importance of expanding the differential diagnosis in patients with area postrema syndrome and no other signs of NMOSD.
我们报告了一例经活检证实的巨细胞动脉炎，其最初表现为后区综合征。
对一名 65 岁男性进行了 MRI、颞动脉活检和超声检查。
患者表现为难治性恶心、呕吐和呃逆，导致体重减轻，无其他神经或临床症状。他 15 天后的 MRI 扫描显示后区有高信号征，无弥散异常或对比增强，符合孤立后区综合征。广泛的炎症和其他病因检查，包括视神经脊髓炎谱系疾病（NMOSD）、髓鞘少突胶质细胞糖蛋白抗体病和多发性硬化（MS），结果均为阴性。患者对甲基强的松龙治疗有反应。最初临床表现两个月后，患者出现疲劳、头痛和头皮压痛。在进行超声检查和活检后，他被诊断为巨细胞动脉炎。他对口服糖皮质激素反应良好，在减量过程中仅复发 1 次。他没有发生动脉炎性缺血性视神经病变或后区综合征的新发作。
本病例表明，在后区综合征且无 NMOSD 其他迹象的患者中，扩大鉴别诊断的重要性。
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