Photo Credit: magicmine

The following is a summary of “Spinal cord gray matter atrophy is associated with disability in spinal muscular atrophy,” published in the January 2025 issue of Neurology by Kesenheimer et al. 

With the approval of disease-modifying treatments for 5q-spinal muscular atrophy (SMA), biomarkers for monitoring disease progression and therapeutic response are essential. Radially sampled Averaged Magnetization Inversion Recovery Acquisitions (rAMIRA) MR-imaging allows in vivo delineation and quantification of spinal cord (SC) gray matter (GM). 

Researchers conducted a prospective study to assess SC GM atrophy in patients with 5q-SMA and its association with clinical disability.

They assessed 21 patients with 5q-SMA and 21 age- and sex-matched healthy controls using 3T axial 2D-rAMIRA MR-imaging at intervertebral disc levels C2/C3-C5/C6 and T _max (lumbar enlargement level). Associations between spinal cord gray matter areas and muscle strength, Motor Function Measure (MFM), revised upper limb module (RULM), revised hammersmith scale (RHS), and SMA-functional rating scale (SMA-FRS) were analyzed using spearman rank correlations and linear regression. 

The results showed significantly reduced spinal cord gray matter areas in patients vs healthy controls at C3/C4 (relative reduction (RR) = 13.6%, P < 0.0001), C4/C5 (RR = 16.7%, P < 0.0001), C5/C6 (RR = 17.1%, P < 0.0001), and Tmax (RR = 17.4%, P < 0.0001). Significant correlations were found between cervical spinal cord gray matter areas and muscle strength, RULM, MFM, RHS, and SMA-FRS. GM area at C3/C4 explained 33% of RHS variance in linear regression. 

Investigators found that spinal cord gray matter atrophy in 5q-SMA patients was linked to clinical disability measures, highlighting its potential as a marker for disease progression and therapeutic response. 

Reference – link.springer.com/article/10.1007/s00415-024-12740-3