A 41-year-old woman was seen at the National Institutes of Health (NIH) Neuroimmunology Clinic in 2017 for recurrent episodes of fever, neck stiffness, and back and leg pain. In 2002, at age 26 years, she had several episodes of back and neck pain, malaise, and fever, each lasting 1 to 3 days (Fig 1). A chest x-ray and complete blood count were normal. Cerebrospinal fluid (CSF) during one of these episodes showed pleocytosis (60 white blood cells [WBC]/ μl; 60% monocytes, 25% lymphocytes, 15% neutrophils), with elevated protein (96 mg/dl) and low glucose (26 mg/dl, Supplementary Table 1). Magnetic resonance imaging (MRI) of the brain showed subtle fluid-attenuated inversion recovery (FLAIR) hyperintensity in the sulcal CSF. MRI of the spine was normal. Extensive investigations including CSF Mycobacterium tuberculosis (TB) complex polymerase chain reaction (PCR) and culture, Coccidioides antibodies, histoplasma antigen, cryptococcal antigen, and herpes simplex virus and varicella zoster virus PCRs were negative (see Supplementary Table 1). Nonetheless, she was treated empirically with valacyclovir for 2 weeks. She had had a recent exposure to TB and had converted from a negative purified protein derivative (PPD) skin test in 2001 to a positive result at the time of her presentation in 2002. Thus, she was also treated empirically for TB meningitis (TBM) with rifampin, pyrazinamide, and ethambutol for 1 year. Isoniazid (INH) was started but was discontinued after several weeks due to transaminitis and nausea. She did not receive adjunctive steroids. Her symptoms resolved until 2006 when, immediately following spinal epidural anesthesia during childbirth, she developed a fever with headache, neck stiffness, back pain, and night sweats. She was treated for endometritis but continued to have similar but less severe symptoms for several months. In early 2007, she acutely developed bilateral gluteal pain and left leg dysesthesias. CSF again showed pleocytosis (130 WBC/μl; 83% lymphocytes, 13% monocytes, 2% neutrophils, 2% other) with elevated protein (132 mg/dl) and low glucose (10 mg/dl). CSF TB PCR and culture, cryptococcal antigen, bacterial and fungal cultures, and viral PCRs, as well as CSF cytology and flow cytometry for malignant cells,

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临床病理学会议：41 岁慢性复发性脑膜炎女性

2017 年，一名 41 岁女性因反复发烧、颈部僵硬、背部和腿部疼痛在美国国立卫生研究院 (NIH) 神经免疫学诊所就诊。2002 年，26 岁时，她出现了几次背部和颈部疼痛、不适和发烧，每次持续 1 至 3 天（图 1）。胸部 X 光检查和全血细胞计数正常。其中一次发作期间的脑脊液 (CSF) 显示细胞增多症（60 个白细胞 [WBC]/μl；60% 单核细胞、25% 淋巴细胞、15% 中性粒细胞）、蛋白质升高 (96 mg/dl) 和低葡萄糖 (26 mg/dl，补充表 1）。大脑的磁共振成像 (MRI) 显示脑沟中存在细微的液体衰减反转恢复 (FLAIR) 高信号。脊柱 MRI 正常。包括 CSF 结核分枝杆菌 (TB) 复合聚合酶链反应 (PCR) 和培养、球孢子菌抗体、组织胞浆菌抗原、隐球菌抗原以及单纯疱疹病毒和水痘带状疱疹病毒 PCR 在内的广泛研究均为阴性（见补充表 1）。尽管如此，她还是接受了为期 2 周的伐昔洛韦经验性治疗。她最近接触过结核病，并从 2001 年的纯化蛋白衍生物 (PPD) 皮肤试验阴性转为 2002 年就诊时的阳性结果。因此，她还接受了结核病脑膜炎 (TBM) 的经验性治疗与利福平、吡嗪酰胺和乙胺丁醇合用 1 年。异烟肼（INH）开始使用，但在几周后因转氨酶和恶心而停药。她没有接受辅助类固醇。她的症状直到 2006 年才消退，当时，在分娩期间进行硬脊膜外麻醉后，她立即发烧并伴有头痛、颈部僵硬、背痛和盗汗。她接受了子宫内膜炎的治疗，但几个月来仍然有类似但不太严重的症状。2007 年初，她突然出现双侧臀痛和左腿感觉迟钝。CSF 再次显示细胞增多（130 WBC/μl；83% 淋巴细胞，13% 单核细胞，2% 中性粒细胞，2% 其他），蛋白质升高（132 mg/dl）和低葡萄糖（10 mg/dl）。CSF TB PCR 和培养、隐球菌抗原、细菌和真菌培养、病毒 PCR，以及恶性细胞的 CSF 细胞学和流式细胞术，她接受了子宫内膜炎的治疗，但几个月来仍然有类似但不太严重的症状。2007 年初，她突然出现双侧臀痛和左腿感觉迟钝。CSF 再次显示细胞增多（130 WBC/μl；83% 淋巴细胞，13% 单核细胞，2% 中性粒细胞，2% 其他），蛋白质升高（132 mg/dl）和低葡萄糖（10 mg/dl）。CSF TB PCR 和培养、隐球菌抗原、细菌和真菌培养、病毒 PCR，以及恶性细胞的 CSF 细胞学和流式细胞术，她接受了子宫内膜炎的治疗，但几个月来仍然有类似但不太严重的症状。2007 年初，她突然出现双侧臀痛和左腿感觉迟钝。CSF 再次显示细胞增多（130 WBC/μl；83% 淋巴细胞，13% 单核细胞，2% 中性粒细胞，2% 其他），蛋白质升高（132 mg/dl）和低葡萄糖（10 mg/dl）。CSF TB PCR 和培养、隐球菌抗原、细菌和真菌培养、病毒 PCR，以及恶性细胞的 CSF 细胞学和流式细胞术，