TY - JOUR

T1 - CLIPPERS: Multiparametric and quantitative MRI features

AU - Korostyshevskaya, Alexandra M.

AU - Stankevich, Julia A.

AU - Vasilkiv, Liubov M.

AU - Bogomyakova, Olga B.

AU - Korobko, Denis S.

AU - Gornostaeva, Alyona M.

AU - Tulupov, Andrey

N1 - Funding Information: We thank the Ministry of Science and Higher Education of the Russian Federation for access to MRI equipment. O.B. Bogomyakova and А.А. Tulupov thank the Russian Science Foundation (project # 22-11-00264) for support of the cerebral circulation investigation by MRI techniques. The English language was corrected and certified by shevchuk-editing.com. Publisher Copyright: © 2022

PY - 2023/1

Y1 - 2023/1

N2 - Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare chronic central-nervous-system inflammatory disorder that became known only recently, and the pathogenesis of CLIPPERS remains poorly understood. This report presents clinical and radiological features of a rare case: a young female patient who rapidly died of suspected CLIPPERS. Helpful multiparametric MRI diagnostic criteria are proposed that can help discriminate CLIPPERS from non-CLIPPERS pathologies. We reviewed clinical history, symptoms, quantitative data from brain multiparametric MRI before and after treatment, and histopathological data. Perfusion-weighted imaging revealed a decrease in regional cerebral blood flow by 31% and in cerebral blood volume by 64%, with a moderate increase in transit time and in time to peak by up to 23% in affected pontine and cerebral white matter. As estimated by diffusion tensor imaging, there was elevated density of tracts (n/mm2) and a decrease of fraction anisotropy (×10−3 mm/s2) in the patient's pons as compared to a healthy control: density of tracts = 13.5 vs 12.4 and fraction anisotropy = 0.32 vs 0.45, respectively. Macromolecular proton fraction values proved to be reduced (15.8% and 14.5% in the control, respectively) in the patient's cerebral peduncles by 3% and in the pons by 4.1% and in a periventricular white matter lesion by 6.4% (11.3% in the normal-looking contralateral hemisphere). Based on our findings, we argue that quantitative MRI techniques may be a valuable source of biomarkers and reliable diagnostic criteria and can shed light on the pathogenesis and exact nosological position of this disorder.

AB - Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare chronic central-nervous-system inflammatory disorder that became known only recently, and the pathogenesis of CLIPPERS remains poorly understood. This report presents clinical and radiological features of a rare case: a young female patient who rapidly died of suspected CLIPPERS. Helpful multiparametric MRI diagnostic criteria are proposed that can help discriminate CLIPPERS from non-CLIPPERS pathologies. We reviewed clinical history, symptoms, quantitative data from brain multiparametric MRI before and after treatment, and histopathological data. Perfusion-weighted imaging revealed a decrease in regional cerebral blood flow by 31% and in cerebral blood volume by 64%, with a moderate increase in transit time and in time to peak by up to 23% in affected pontine and cerebral white matter. As estimated by diffusion tensor imaging, there was elevated density of tracts (n/mm2) and a decrease of fraction anisotropy (×10−3 mm/s2) in the patient's pons as compared to a healthy control: density of tracts = 13.5 vs 12.4 and fraction anisotropy = 0.32 vs 0.45, respectively. Macromolecular proton fraction values proved to be reduced (15.8% and 14.5% in the control, respectively) in the patient's cerebral peduncles by 3% and in the pons by 4.1% and in a periventricular white matter lesion by 6.4% (11.3% in the normal-looking contralateral hemisphere). Based on our findings, we argue that quantitative MRI techniques may be a valuable source of biomarkers and reliable diagnostic criteria and can shed light on the pathogenesis and exact nosological position of this disorder.

KW - CLIPPERS

KW - Diffusion tensor imaging

KW - Macromolecular proton fraction mapping

KW - Perfusion-weighted imaging

KW - Quantitative MRI

UR - http://www.scopus.com/inward/record.url?scp=85141994396&partnerID=8YFLogxK

UR - https://www.mendeley.com/catalogue/94173f94-415d-3e17-87b5-c408d9af96c2/

U2 - 10.1016/j.radcr.2022.10.043

DO - 10.1016/j.radcr.2022.10.043

M3 - Article

C2 - 36411846

AN - SCOPUS:85141994396

VL - 18

SP - 368

EP - 376

JO - Radiology Case Reports

JF - Radiology Case Reports

SN - 1930-0433

IS - 1

ER -