What is the potential of paramagnetic rim lesions as diagnostic indicators in multiple sclerosis?

Figures & data

Figure 1. (A) Representative 3-tesla MRI example of a paramagnetic rim lesion (PRL) in a 21-year-old man with relapsing MS. PRL is clearly seen on both 3D seg-T2*-w EPI magnitude and unwrapped filtered phase images and on axial SWI phase images. For each image modality, PRL magnification is shown in the white insets. For each MRI sequence, image resolution is provided. (B) Graph showing the overall percentage of MS cases with at least one PRL by MS clinical phenotypes (number of cases evaluated in the brackets). (C) Graph showing the overall percentage of cases with at least one PRL by diseases (number of cases evaluated in the brackets).

Table 1. Clinical MRI studies assessing the diagnostic specificity and sensitivity of PRL in differentiating between MS and MS-mimic disorders.

Study	Type of study	MS (n)	Non-MS (n)	Non-MS	Specificity ≥1 PRL	Sensitivity ≥1 PRL	MRI field
Wuerfel et al., MSJ 2012	Prospective cross-sectional	10	5	Susac’s syndrome	20%	100%	7 T
Hagemeier et al., JMRI 2012	Retrospective cross-sectional	135	49	NIND	97.9%	21.5%	3 T
Kilsdonket al., Eur Radiol 2014	Prospective cross-sectional	16	16	NIND	100%	25%	7 T
Chawla et al., AJNR 2016	Prospective cross-sectional	21	21	NMOSD	100%	10%	7 T
Sinnecker et al., Neurol Neuroimmunol Neuroinflamm 2016	Prospective cross-sectional	10	10	NMOSD	80–90%	90%	7 T
Hosseini et al., AJNR 2018	Retrospective cross-sectional	17	18	NIND	100%	NA	7 T
Jang et al., J Clin Neurol 2020	Retrospective cross-sectional	32	21	NMOSD	95%	81%	3 T
Clarke et al., AJNR 2020	Prospective longitudinal	112	35	OIND, NIND, & other mimics	100%	59%	3 T
Maggi et al., Ann Neurol 2020	Retrospective multicenter cross-sectional	329	83	OIND, NIND, Susac’s syndrome, HTLV & HIV	93%	52%	3 T
Micheletti et al., Pediatr Radiol 2020	Retrospective cross-sectional	13 pediatric	16 pediatric	NMOSD, ADEM,NMOSD/SLE	100%	92.3%	1.5 T
Meaton et al., MSJ 2022	Retrospective multicenter cross-sectional	254	91 217	NMOSD, OIND, NIND Aging healthy controls	99.7%	24%	3 T
Renner et al., ACTRIMS Forum 2022	Prospective multicenter cross-sectional	58	37	OIND	88%	82%	3 T

Abbreviations:

NIND: noninfectious noninflammatory neurological diseases (headache, migraine, cerebral small vessel disease, and incidental white matter lesions); OIND: other immune-mediated diseases with neurological involvement (systemic lupus erythematosus or SLE, Sjögren disease, primary angiitis of the central nervous system, Behçet disease, neurosarcoidosis, and antiphospholipid syndrome); NMOSD: neuromyelitis optica spectrum disorder; HTLV: HTLV1-2-infected patients; HIV: HIV-infected patients.

Table

Box 1. Proposed revised dissemination in space MRI criteria

The McDonald criteria for dissemination in space are fulfilled if both the following criteria are present in a patient with a clinically isolated syndrome or typical MS attack: An MRI with one or more hyperintense T2 lesions that are characteristic of multiple sclerosis in at least two of four MS-typical regions of the central nervous system: periventricular cortical or juxtacortical infratentorial spinal cord

AND (2) The hyperintense T2 lesions on MRI should satisfy at least one of the following ‘specificity criteria’: presence of at least one paramagnetic rim lesion on susceptibility-based MRI presence of the central vein sign on susceptibility-based MRI (40%-threshold rule, OR 6-rule, OR 3-rule)*

*no international consensus is yet available on the best performing CVS rule in clinical settings [70–73].

Table

Box 2. Desirable goals for future PRL diagnostic and prognostic implementation into clinical practice International consensus for PRL definition and reporting [16] Standardization of susceptibility-based MRI sequences and postprocessing Availability of dedicated susceptibility-based MRI sequences and automated postprocessing for PRL detection by all MRI scanner vendors Validation of the reliability of PRL detection at all magnetic fields, including 1.5 T MRI Revision of current dissemination in space MRI criteria with the inclusion of the PRL biomarker and its validation Clinical training for both neurologists and neuroradiologists on the use of PRL biomarkers

NAIMS cooperative workshop Feb, 2022. NAIMS consensus statement on imaging chronic active lesions. https://www.naimscooperative.org/2022-naims-workshop

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