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Post a Comment The role of magnetic resonance imaging in the early diagnosis of chronic lymphocytic inflammation (CLIPPERS syndrome)
- Authors: Fomina L.E.1, Kieva I.N.2, Anikin A.V.1, Nudnov N.V.3, Abdullaeva L.M.1
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Affiliations:
- National Medical Research Center for Children's Health
- G.N. Speransky Children's City Clinical Hospital № 9
- Russian Scientific Center of Roentgenoradiology
- Issue: Vol 13, No 4 (2022)
- Pages: 109-119
- Section: Case reports
- URL: https://journals.rcsi.science/clinpractice/article/view/144176
- DOI: https://doi.org/10.17816/clinpract114814
- ID: 144176
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Abstract
Background: Chronic lymphocytic inflammation with perivascular contrast enhancement in the pons responsive to steroids (CLIPPERS) is a rare and relatively new pathology and magnetic resonance imaging (MRI) is highly important in its diagnosis.
Clinical case description: The analysis of the MRI results revealed typical changes characteristic of CLIPPERS, such as contrast-positive small-point and curvilinear lesions of the pons, medulla oblongata and midbrain, as well as of the cerebral peduncles and cerebellum. In addition to the changes we described, according to the literature, lesions of the white matter of the large hemispheres and thalami may also be observed in the syndrome of chronic lymphocytic inflammation.
Conclusion: MRI not only enables one to achieve high-quality neuroimaging of CLIPPERS, but also allows avoiding invasive, traumatic diagnostic methods.
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##article.viewOnOriginalSite##About the authors
Lyubov E. Fomina
National Medical Research Center for Children's Health
Author for correspondence.
Email: love.fomina@mail.ru
ORCID iD: 0000-0002-3838-3284
SPIN-code: 1298-8350
researcher, laboratory of radiation and instrumental diagnostics
Russian Federation, 2 b. 1, Lomonosovsky prospect, Moscow, 119991Irina N. Kieva
G.N. Speransky Children's City Clinical Hospital № 9
Email: miamodiaz@gmail.com
ORCID iD: 0000-0002-4060-5966
SPIN-code: 2279-9141
radiologist
Russian Federation, MoscowAnatoly V. Anikin
National Medical Research Center for Children's Health
Email: anikacor@gmail.com
ORCID iD: 0000-0003-0362-6511
SPIN-code: 7592-1352
MD, PhD
Russian Federation, MoscowNikolay V. Nudnov
Russian Scientific Center of Roentgenoradiology
Email: nudnov@rncrr.ru
ORCID iD: 0000-0001-5994-0468
SPIN-code: 3018-2527
MD, PhD
Russian Federation, MoscowLuizat M. Abdullaeva
National Medical Research Center for Children's Health
Email: instorm@inbox.ru
ORCID iD: 0000-0003-1574-2050
neurologist
Russian Federation, MoscowReferences
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Fig. 1. Primary MRI of the brain in the axial plane of March 12, 2020 without intravenous contrast enhancement (a, в — T2-weighted image; б, г — FLAIR): there are multiple bilateral punctate and curvilinear small foci of the altered MR signal, including confluent foci, located in the pons, cerebellar peduncles and the white matter of the brain hemispheres and cerebellum (arrows).
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Fig. 2. Follow-up (after 5 days of pulse therapy) MRI of the brain in the axial plane of August 16, 2021 with intravenous contrast enhancement (a, в — T2-weighted image; б, г — FLAIR; д, е — T1-weighted image before and after contrast enhancement respectively): compared with the MRI data of March 12, 2020, small foci of the altered MR signal with the same localization, structure and size remain (arrows). A short-term pulse therapy course doesn’t result in strong contrast enhancement (arrows).
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Fig. 3. MRI of the brain in the axial plane of January 10, 2022 with intravenous contrast enhancement (a, в — T2-weighted image; б, г — T1-weighted image after contrast enhancement): compared with the MRI data of August 16, 2021, small foci of the altered MR signal with the same localization, structure and size remain (arrows). Strong positive contrast enhancement is visualized (arrows).
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Fig. 4. MRI of the brain in the axial plane of March 25, 2022 with intravenous contrast enhancement in the acute stage of CLIPPERS (a, в — T2-weighted image; б, г — T1-weighted image after contrast enhancement): compared with the MRI data of August 16, 2021, a negative trend is observed namely an increase in the size and number of foci with strong contrast enhancement (arrows).
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Fig. 5. MRI of the brain in the axial plane of June 15, 2022 with intravenous contrast enhancement after a course of pulse therapy and maintenance therapy with corticosteroids for three months (a, в, д — T2-weighted image; б, г, е — T1-weighted image after contrast enhancement): compared with the MRI data of March 25, 2022 there is a regression of focal changes in the subtentorial structures (arrows). Absence of pathological contrast enhancement.
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Fig. 6. MRI of the brain in the axial plane of October 27, 2022 with intravenous contrast enhancement after a maintenance therapy with corticosteroids for seven months (a, б — T1-weighted image before and after contrast enhancement respectively; в, д — T2-weighted image; г, е — FLAIR): compared with the MRI data of June 15, 2022 no new focal changes have appeared. Absence of pathological contrast enhancement. However there are changes of the MR signal in the pons (white arrows) and changes of the white matter of the brain hemispheres with secondary dilatation of liquor spaces (red arrows) specific to pseudoatrophy during the treatment with steroids.
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Fig. 7. Fragments of MRI studies obtained in the order they were performed that allow one to clearly assess the dynamics of changes.
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