CLIPPERS: Multiparametric and quantitative MRI features

Radiol Case Rep. 2022 Nov 17;18(1):368-376. doi: 10.1016/j.radcr.2022.10.043. eCollection 2023 Jan.


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.

Keywords: ADC, apparent diffusion coefficient; CBF, cerebral blood flow; CLIPPERS; CNS, central nervous system; CSF, cerebrospinal fluid; DOT, density of tracts; DTI, diffusion tensor imaging; DWI, diffusion-weighted imaging; Diffusion tensor imaging; FLAIR, fluid attenuated inversion recovery; ITC, International Tomography Center; MPF, macromolecular proton fraction; MS, multiple sclerosis; Macromolecular proton fraction mapping; PWI, perfusion-weighted imaging; Perfusion-weighted imaging; Quantitative MRI; SWI, susceptibility-weighted imaging; WI, weighted image.

Publication types

  • Case Reports