Pearls & Oy-sters: Using Susceptibility-Based Imaging in Highly Active Late-Onset Multiple Sclerosis

Area of Science:

• Neurology
• Radiology
• Immunology

Background:

• The 2024 McDonald criteria incorporate the central vein sign (CVS) and paramagnetic rim lesions (PRLs) as key diagnostic biomarkers for multiple sclerosis (MS).
• These susceptibility-based MRI findings offer moderate (CVS) to high (PRLs) specificity, proving valuable in complex or atypical presentations.
• Diagnosing MS can be challenging, particularly in late-onset or atypical cases, necessitating advanced imaging techniques.

Purpose of the Study:

• To illustrate the diagnostic utility of CVS and PRLs in a challenging case of late-onset multiple sclerosis.
• To highlight how these advanced MRI biomarkers can support diagnosis when conventional criteria are ambiguous.
• To emphasize the potential of these biomarkers to reduce the need for invasive procedures like brain biopsy.

Main Methods:

• A case study of a 61-year-old woman presenting with subacute ataxia and dysarthria.
• Comprehensive cerebrospinal fluid (CSF) analysis and extensive differential diagnosis workup.
• High-resolution 7-tesla MRI to identify CVS and PRLs, alongside histopathological examination of brain biopsy tissue.
• Correlation of imaging findings with clinical presentation and laboratory results.

Main Results:

• The patient exhibited numerous enhancing lesions, atypical CSF findings (lymphocytic pleocytosis, elevated protein, absent oligoclonal bands), and initially ambiguous MS diagnosis.
• Brain biopsy confirmed active demyelination with a macrophage-rich inflammatory infiltrate.
• A 7-tesla MRI revealed the presence of ≥12 CVS and ≥1 PRL, confirming the diagnosis of relapsing late-onset MS.
• The identified biomarkers (CVS and PRLs) were crucial in establishing the diagnosis.

Conclusions:

• Susceptibility-based imaging biomarkers, CVS and PRLs, are instrumental in diagnosing challenging cases of multiple sclerosis.
• Integration of CVS and PRLs into the 2024 McDonald criteria may streamline the diagnostic process for atypical demyelinating diseases.
• These advanced MRI markers can potentially obviate the need for brain biopsy, facilitating earlier diagnosis and treatment initiation in MS.