CLIPPERS and its mimics: evaluation of new criteria for the diagnosis of CLIPPERS
CLIPPERS and its mimics: evaluation of new criteria for the diagnosis of CLIPPERS
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DOI：
10.1136/jnnp-2018-318957
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发表时间：
2019-09-01
影响因子：
11
通讯作者：
Bejot, Yannick
中科院分区：
文献类型：
--
作者：
Taieb, Guillaume;Mulero, Patricia;Bejot, Yannick
目的评价新提出的激素敏感性桥脑血管周围强化的慢性淋巴细胞性炎症诊断标准的准确性方法我们纳入了42例患者的后脑点状和/或线性增强（直径= 3 mm），在CLIPPERS标准中被视为危险信号，在13例CLIPPERS模拟者中有4例存在，但在29例CLIPPERS患者中也有2例存在，这解释了灵敏度的缺乏。最初符合CLIPPERS标准的13名CLIPPERS模仿者中有4名在第二次发作时显示红旗，中位时间为5.5个月（最小3个月，最大18个月），这解释了缺乏特异性。4例患者中有1例有抗髓鞘少突胶质细胞糖蛋白抗体，其余3例尽管每日剂量>= 30 mg泼尼松/泼尼松龙仍复发，且针对非典型增强病变的活检显示为淋巴瘤。结论我们的研究强调：（1）结节性增强应更多地被视为一种不寻常的发现，而不是排除CLIPPERS诊断的红旗;（2）在疾病发作后18个月内可能出现危险信号;（3）与CLIPPERS模拟物相反，当泼尼松/泼尼松龙的日剂量>= 30 mg时，不会发生复发;（4）当非侵入性检查仍不确定时，脑活检应针对非典型增强病变。
Objective To evaluate the accuracy of the recently proposed diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).Methods We enrolled 42 patients with hindbrain punctate and/or linear enhancements (= 3 mm in diameter), considered as a red flag in CLIPPERS criteria, was present in 4 out of 13 CLIPPERS-mimics but also in 2 out of 29 patients with CLIPPERS, explaining the lack of sensitivity. Four out of 13 CLIPPERS-mimics who initially met the CLIPPERS criteria displayed red flags at the second attack with a median time of 5.5 months (min 3, max 18), explaining the lack of specificity. One of these four patients had antimyelin oligodendrocyte glycoprotein antibodies, and the three remaining patients relapsed despite a daily dose of prednisone/prednisolone >= 30 mg and a biopsy targeting atypical enhancing lesions revealed a lymphoma.Conclusions Our study highlights that (1) nodular enhancement should be considered more as an unusual finding than a red flag excluding the diagnosis of CLIPPERS; (2) red flags may occur up to 18 months after disease onset; (3) as opposed to CLIPPERS-mimics, no relapse occurs when the daily dose of prednisone/prednisolone is >= 30 mg; and (4) brain biopsy should target an atypical enhancing lesion when non-invasive investigations remain inconclusive.