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Investigating the Frequency and Outcome of Central Vein Sign and Paramagnetic Rim Lesions in Children With MOGAD
Riccardo Nistri1,2, Laura Cacciaguerra3, Simone Sacco4
1Queen Square MS Centre, Department of Neuroinflammation, UCL Queen Square Institute of Neurology, Faculty of Brain Sciences, University College London, United Kingdom.
Background And Objectives:
Central vein sign (CVS) is a common feature in multiple sclerosis (MS) lesions, but its frequency in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) varies significantly across studies. Paramagnetic rim lesions (PRLs) are described in MS, but not in MOGAD. Our goals were to evaluate the prevalence of PRLs and CVS in a large multicenter cohort of pediatric MOGAD. We compared CVS frequencies between acute vs remission phases, as well as with longitudinal dynamics of lesion evolution.
Methods:
In this longitudinal retrospective multicenter study, clinical MRIs were assessed from pediatric patients with MOGAD, who had (1) ≥1 brain lesion, (2) susceptibility-based imaging (SBI), and (3) follow-up MRI at least 3 months apart. T2-weighted and fluid-attenuated inversion recovery sequences were analyzed for lesion detection and resolution. SBI was used to assess CVS and PRLs following North American Imaging in MS Cooperative criteria.
Results:
A total of 65 patients and 130 scans were included. A total of 520 lesions were evaluated. The most common reason for lesion exclusion was size (n = 143, large confluent lesions, n = 122, <3 mm, n = 21). CVS was detected in 97 of 327 (29.7%) lesions, with 32 of 65 (49.2%) patients having at least 1 CVS+ lesion. Patients with ≥1 CVS+ lesion (32/65, 49.2%) had a higher number of lesions (p = 0.002) and lesions suitable for CVS analysis (p < 0.001). Of the 31 patients with ≥3 brain lesions, 11 of 31 (35.5%) had >40% CVS+ lesions and 7 (22.5%) had >50% CVS+ lesions. Only 4 patients had ≥6 CVS+ lesions. The proportion of CVS+ lesions was lower in patients who had SBI acquired during an acute attack vs patients scanned during remission (16% vs 33%, p = 0.015). The rate of lesion resolution was higher in CVS- lesions (177/230, 76%) compared with CVS+ (42/97, 42%, p < 0.001). No PRLs were identified.
Discussion:
Lesion pathobiology in MOGAD is heterogeneous. CVS identified persistent rather than transient lesions, with resolution more common among CVS- lesions. The high frequency of confluent or multivein lesions limited the proportion suitable for CVS analysis. MRI timing influenced CVS detection, which was higher in remission, suggesting that time of acquisition contributes to variability across MOGAD studies. No PRLs were found, supporting their potential as biomarkers distinguishing MOGAD from MS.
Insights
Central vein sign (CVS) in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) lesions was present in 49.2% of patients, often indicating persistent lesions. Paramagnetic rim lesions (PRLs) were not identified, potentially distinguishing MOGAD from multiple sclerosis (MS).
Area of Science:
- Neuroimmunology
- Neuroimaging
- Pediatric Neurology
Background:
- The central vein sign (CVS) is a recognized feature in multiple sclerosis (MS) lesions, but its prevalence in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) remains inconsistent across studies.
- Paramagnetic rim lesions (PRLs), observed in MS, have not been consistently reported in MOGAD, suggesting potential differences in lesion pathology.
- Understanding these imaging markers is crucial for differentiating MOGAD from MS, especially in pediatric populations.
Purpose of the Study:
- To determine the prevalence of paramagnetic rim lesions (PRLs) and the central vein sign (CVS) in a large, multicenter cohort of pediatric patients diagnosed with MOGAD.
- To compare the frequency of CVS between acute and remission phases of MOGAD.
- To analyze the longitudinal dynamics of MOGAD lesion evolution in relation to CVS presence.
Main Methods:
- A longitudinal, retrospective multicenter study involving pediatric MOGAD patients with at least one brain lesion and susceptibility-based imaging (SBI).
- Clinical MRI scans were analyzed using T2-weighted and fluid-attenuated inversion recovery sequences for lesion detection and resolution.
- Susceptibility-based imaging (SBI) was utilized to assess for CVS and PRLs according to established criteria.
Main Results:
- Of 520 evaluated lesions in 65 patients, 29.7% showed a central vein sign (CVS), with 49.2% of patients having at least one CVS-positive lesion.
- CVS-positive lesions were associated with a higher overall lesion burden and were less likely to resolve compared to CVS-negative lesions (42% vs. 76% resolution).
- No paramagnetic rim lesions (PRLs) were identified in any of the MOGAD patients.
Conclusions:
- The presence of CVS in MOGAD lesions suggests a more persistent lesion type, while CVS-negative lesions demonstrate higher resolution rates.
- The detection of CVS was influenced by MRI acquisition timing, being higher during remission phases, which may explain study variability.
- The absence of PRLs in this MOGAD cohort supports their potential utility as imaging biomarkers to differentiate MOGAD from MS.
