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

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 neuropatholo...

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Autores principales: Blaabjerg, Morten, Ruprecht, Klemens, Sinnecker, Tim, Kondziella, Daniel, Niendorf, Thoralf, Kerrn-Jespersen, Bjørg Morell, Lindelof, Mette, Lassmann, Hans, Kristensen, Bjarne Winther, Paul, Friedemann, Illes, Zsolt
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4841643/
https://www.ncbi.nlm.nih.gov/pubmed/27144217
http://dx.doi.org/10.1212/NXI.0000000000000226
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author Blaabjerg, Morten
Ruprecht, Klemens
Sinnecker, Tim
Kondziella, Daniel
Niendorf, Thoralf
Kerrn-Jespersen, Bjørg Morell
Lindelof, Mette
Lassmann, Hans
Kristensen, Bjarne Winther
Paul, Friedemann
Illes, Zsolt
author_facet Blaabjerg, Morten
Ruprecht, Klemens
Sinnecker, Tim
Kondziella, Daniel
Niendorf, Thoralf
Kerrn-Jespersen, Bjørg Morell
Lindelof, Mette
Lassmann, Hans
Kristensen, Bjarne Winther
Paul, Friedemann
Illes, Zsolt
author_sort Blaabjerg, Morten
collection PubMed
description 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.
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spelling pubmed-48416432016-05-03 Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI Blaabjerg, Morten Ruprecht, Klemens Sinnecker, Tim Kondziella, Daniel Niendorf, Thoralf Kerrn-Jespersen, Bjørg Morell Lindelof, Mette Lassmann, Hans Kristensen, Bjarne Winther Paul, Friedemann Illes, Zsolt Neurol Neuroimmunol Neuroinflamm Article 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. Lippincott Williams & Wilkins 2016-04-20 /pmc/articles/PMC4841643/ /pubmed/27144217 http://dx.doi.org/10.1212/NXI.0000000000000226 Text en © 2016 American Academy of Neurology This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND) (http://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially.
spellingShingle Article
Blaabjerg, Morten
Ruprecht, Klemens
Sinnecker, Tim
Kondziella, Daniel
Niendorf, Thoralf
Kerrn-Jespersen, Bjørg Morell
Lindelof, Mette
Lassmann, Hans
Kristensen, Bjarne Winther
Paul, Friedemann
Illes, Zsolt
Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI
title Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI
title_full Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI
title_fullStr Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI
title_full_unstemmed Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI
title_short Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI
title_sort widespread inflammation in clippers syndrome indicated by autopsy and ultra-high-field 7t mri
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4841643/
https://www.ncbi.nlm.nih.gov/pubmed/27144217
http://dx.doi.org/10.1212/NXI.0000000000000226
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