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Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI

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Item Type:Article
Title:Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI
Creators Name:Blaabjerg, M. and Ruprecht, K. and Sinnecker, T. and Kondziella, D. and Niendorf, T. and Kerrn-Jespersen, B.M. and Lindelof, M. and Lassmann, H. and Kristensen, B.W. and Paul, F. and Illes, Z.
Abstract: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.
Keywords:Chronic Lymphocytic Inflammation, Multiple-Sclerosis Lesions, B-Cell Lymphoma, Steroids Clippers, Susac Syndrome, MS Lesions, Patient
Source:Neurology Neuroimmunology & Neuroinflammation
Publisher:American Academy of Neurology
Page Range:e226
Date:June 2016
Official Publication:https://doi.org/10.1212/NXI.0000000000000226
PubMed:View item in PubMed

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