The sarcoglycanopathies pattern in whole-body muscle imaging

  • An international research consortium has reported magnetic resonance imaging (MRI) data (whole-body MRI in this case) from 64 patients diagnosed with sarcoglycanopathy:
  • 43 had LGMD-R3 (alpha-sarcoglycanopathy), 8 had LGMD-R4 (beta-sarcoglycanopathy), 12 had LGMD-R5 (gamma-sarcoglycanopathy) and one had LGMD-R6 (delta-sarcoglycanopathy)
  • in each case, images of 65 target muscles were examined, most often longitudinally,
  • fairly characteristic patterns were identified, particularly in the upper body, which had previously been poorly studied,
  • early-onset forms often showed a rapid rate of deterioration compared with late-onset forms.

The authors provide a comparative table with other muscle disorders affecting the girdles, which will prove useful for clinicians.

 

Whole-Body Pattern of Muscle Degeneration and Progression in Sarcoglycanopathies. Costa-Comellas L, Monforte M, Sanchez-Montañez A et al. Ann Clin Transl Neurol. 2025 Dec