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翻译：韩韩；审校：赵鹏 文献提供：刘红 李神经群 文献翻译部 出品 Figure Initial T1-weighted postgadolinium brain MRI T1加权像钆喷酸葡胺脑MRI （A）双侧小脑桥脑角和桥脑对称性强化，高度提示围血管分布的特征。（B）应用皮质类固醇后几乎完全消失。（C）未应用皮质类固醇同部位反复发作。 A 48-year-old man developed progressive vertigo, ataxia, and dysarthria. MRI demonstrated gadolinium-enhancing abnormalities in the pons and cerebellar peduncles (figure, A). Further investigations excluded neurosarcoidosis, CNS lymphoma, granulomatosis, and paraneoplastic orchronic infectious processes. In particular, 2 CSF examinations revealed mildly elevated protein levels (0.73 g/L, N 0.40 g/L) without pleocytosis, tumoral cells, or oligoclonal bands. 男性患者，48岁，表现为进行性加重的眩晕，共济失调，构音障碍。MRI增强显示桥脑小脑角异常（Fig.A）。进一步的检查排除了神经类肉瘤病，中枢神经系统淋巴瘤，肉芽肿，副肿瘤或慢性感染性疾病。特别是2例脑脊液检查发现蛋白轻度增高（0.73 g/L, N 0.40 g/L），无淋巴细胞增生、肿瘤细胞或寡克隆带。 The patient was treated with IV methylprednisolone (1 g daily for 3 days) followed by oral prednisone (1 mg/kg daily). Symptoms and MRI lesions improved at 3 months, while the patient was receiving 30 mg prednisone daily (figure, B). One month after corticosteroid discontinuation, he experienced a recurrence of the same symptoms. Repeat brain MRI revealed similar lesions (figure, C).Reintroduction of IV methylprednisolone was successful. Chronic therapy with oral prednisone was initiated and he experienced steady improvement. At last follow-up, 4 months after the recurrence, clinical examination only found a right nystagmus while the patient was receiving 25 mg prednisone daily. 患者应用静脉输注甲基泼尼松龙（1g/d应用3d）后续口服泼尼松龙（1mg/kg/d）。患者口服泼尼松30mg /d，症状和MRI病灶在3个月时得到改善(figure, B)。停用皮质类固醇后1月病人再次出现类似症状，复查脑MRI发现相似病灶(figure, C)。再次给予静滴甲基泼尼松龙，依然有效。启动长期口服波尼松后病人得到了持续的改善，再发后4个月末次随访时，患者一直每天服用25mg泼尼松，临床仅发现右侧眼震。 The clinical presentation and neuroimaging, and the response to steroids, were highly suggestive of the recently described chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome. 临床表现、神经影像及对类固醇治疗敏感，高度提示最近描述的类固醇激素反应性慢性淋巴细胞性炎症伴脑桥血管周围强化症(CLIPPERS综合征) MYSTERY CASE RESPONSES The Mystery Case series was initiated by the Neurology? Resident & Fellow Section to develop the clinical reasoning skills of trainees. Residency programs, medical student preceptors, and individuals were invited to use this Mystery Case as an educational tool. Responses were solicited through a group e-mail sent to the AAN Consortium of Neurology Residents and Fellows and through social media. 罕见病例系列由神经科住院医师和相关科室发起，为了使培训者掌握临床思维技巧。 住院医师培训项目，预科医学生和个人被邀请应用稀有病例系统作为教学工具。要求大家通过e-mial 组发送给由神经科住院医师和研究员组成的AAN协会，也可通过社会媒体发送。 There were 60 responses to this Mystery Case, 2 of which were submitted via social media. All responses came from individual residents rather than groups and they were wellreasoned and thoughtful, some of them very detailed with extensive discussion of the differential diagnosis. The majority of respondents (35) identified the particular MRI pattern of enhancement, the clinical signs, and the response to therapy seen in CLIPPERS syndrome. Additional considerations included neurosarcoidosis (8), demyelination (5), Bickerstaff encephalitis (4), Behcet disease (4), Erdheim-Chester disease (1), and intravascular angiocentric lymphoma (1). Two respondents discussed the differential without favoring a particular diagnosis. CLIPPERS appears to be a distinct clinic and radiologic brainstem inflammatory syndrome, though some have raised concerns that radiologically compatible CLIPPERS may conceal a number of different pathologies. 本次罕见病例有60个反馈意见，其中2例通过社会媒体投送，所有的反馈均来自于各个住院医师，而不是团队，他们逻辑思维很强，而且想的很周到，他们中的一些还对鉴别诊断进行详细而又广泛的讨论。反馈的大多数（35）强调了CLIPPERS综合征的这些特征，MRI强化形式，临床症状，对治疗的敏感性。其他的考虑包括神经类肉瘤（8），脱髓鞘（5），Bickerstaff 脑炎（4），白塞氏病（4），Erdheim-Chester 病（1），和血管内中心性淋巴瘤（1）。两例反馈只是讨论了鉴别诊断，并没有确切的诊断。CLIPPERS在临床和放射学上表现独特的脑干炎症综合征，尽管有人提出质疑，CLIPPERS的影像学一致性也许掩盖了一些不同的病变。 翻译：韩韩；审校：赵鹏；文献提供：刘红； 李神经群 文献翻译部 出品； |
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