CLIPPERS 综合征的广泛炎症可通过尸检和超高场 7T MRI 显示。
Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI.
机构信息
Departments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
出版信息
Neurol Neuroimmunol Neuroinflamm. 2016 Apr 20;3(3):e226. doi: 10.1212/NXI.0000000000000226. eCollection 2016 Jun.
OBJECTIVE
To examine if there is widespread inflammation in the brain of patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome by using histology and ultra-high-field MRI at 7.0T.
METHODS
We performed a detailed neuropathologic examination in 4 cases, including 1 autopsy case, and studied 2 additional patients by MRI at 7.0T to examine (1) extension of inflammation to areas appearing normal on 3.0T MRI, (2) potential advantages of 7.0T MRI compared to 3.0T MRI in reflecting widespread inflammation, perivascular pathology, and axonal damage, and (3) the possibility of lymphoma.
RESULTS
In the autopsy case, perivascular inflammation dominated by CD4+ T cells was not only detected in the brainstem and cerebellum but also in brain areas with normal appearance on 3.0T MRI, including supratentorial regions and cranial nerve roots. There was no evidence of lymphoma in any of the 4 patients. The 7.0T MRI in clinical remission also revealed supratentorial lesions and perivascular pathology in vivo with contrast-enhancing lesions centered around a small venous vessel. Ultra-high-field MRI at 7.0T disclosed prominent T1 hypointensities in the brainstem, which were not seen on 3.0T MRI. This corresponded to neuropathologic detection of axonal injury in the autopsy case.
CONCLUSION
Our findings suggest more widespread perivascular inflammation and postinflammatory axonal injury in patients with CLIPPERS.
目的
通过组织病理学和 7.0T 超高场 MRI 检查，研究慢性淋巴细胞性炎症伴脑桥血管周围强化反应性类固醇（CLIPPERS）综合征患者的大脑是否存在广泛炎症。
方法
我们对 4 例患者（包括 1 例尸检病例）进行了详细的神经病理学检查，并通过 7.0T MRI 对另外 2 例患者进行了研究，以检查（1）炎症向 3.0T MRI 正常区域的扩展，（2）7.0T MRI 与 3.0T MRI 相比在反映广泛炎症、血管周围病理和轴突损伤方面的潜在优势，以及（3）淋巴瘤的可能性。
结果
在尸检病例中，以 CD4+T 细胞为主的血管周围炎症不仅在脑干和小脑中被检测到，而且在 3.0T MRI 上外观正常的脑区也被检测到，包括大脑半球和颅神经根。在这 4 例患者中均未发现淋巴瘤的证据。在临床缓解期的 7.0T MRI 也显示了大脑半球的病变和血管周围病理，伴有增强病变集中在小静脉周围。7.0T 的超高场 MRI 显示了脑干的明显 T1 低信号，而在 3.0T MRI 上则没有。这与尸检病例中轴突损伤的神经病理学检测结果相对应。
结论
我们的发现提示 CLIPPERS 患者存在更广泛的血管周围炎症和炎症后轴突损伤。
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