Clinical correlation between disease progression and central vein sign in pediatric onset multiple sclerosis: A binational study

• Published in: European journal of paediatric neurology 2024-05, Vol.50, p.81-85
• Main Authors: Menascu, Shay, Halusková, Simona, Pollak, Amir, Ryska, Pavel, Angelucci, Francesco, Magalashvili, David, Guber, Diana, Yosef, Arthur, Kalron, Alon, Valis, Martin, Gurevich, Michael
• Format: Article
• Language: English
• Subjects: Adolescent; Age of Onset; Brain - diagnostic imaging; Brain - pathology; Cerebral Veins - diagnostic imaging; Cerebral Veins - physiopathology; Child; Czech Republic; Disability Evaluation; Disease Progression; Female; Follow-Up Studies; Humans; Israel; Magnetic Resonance Imaging; Male; Multiple Sclerosis - diagnostic imaging; Multiple Sclerosis - physiopathology
• ISSN: 1090-3798; 1532-2130; 1532-2130
• DOI: 10.1016/j.ejpn.2024.04.007

The central vein sign (CVS) has been proposed as a novel MRI biomarker to improve diagnosis of pediatric-onset MS (POMS). However, the role of CVS in POMS progression has yet to be discovered. To investigate the appearance of CVS and its correlation with POMS disease progression. One hundred fifty-six POMS from two MS centers in Israel and Czech Republic MS centers were followed for five years. Patient assessment was performed by the Expanded Disability Status Scale (EDSS) and Annual Relapse Rate (ARR). Patients in whom at least 40 % of brain MRI lesions had CVS (“rule of 40”) were determined as CVS-positive. The total group of POMS consisted of 96 CVS-negative (61.5 %), aged 14.6 ± 1.9 years, EDSS 2.0, 75 % Interquartile Range (IQR) 1.0–3.0, disease duration (DD) 6.28 ± 0.38 years, and 60 CVS-positive (38.5 %), aged 15.1 ± 0.3 years, EDSS 2.0, IQR 1.5–3.0, DD 5.62 ± 0.13 years, were analyzed. After a three and five-year follow-up, the CVS-positive patients had higher EDSS scores than those who were CVS-negative, 2.0, IQR 1.0–2.5, vs 1.0, IQR 1.0–2.0, (p = 0.009) and 2.0, IQR 1.0–3.25 vs 1.0, IQR 1.0–2.0, (p = 0.0003), respectively. Patients with CVS-positive POMS were characterized by a significantly higher ARR (0.78 ± 0.08 vs 0.57 ± 0.04, p = 0.002). These results were confirmed in subgroups of Disease Modifying Treatments (DMT) untreated and treated patients. CVS-positive POMS is characterized by higher disability progression than CVS-negative, indicating the importance of CVS in disease pathogenesis. •CVS-positive POMS is characterized by higher disability progression, indicating the importance of CVS in MS pathogenesis.•The effect of CVS on POMS disability progression appears independent of patient demographics and DMT treatment status.•CVS could serve as a marker for disease progression in POMS.