A large series of patients with VCP gene-related myopathy studied with muscle imaging

An international consortium of clinicians coordinated by the Barcelona Neuromuscular Center has published the results of the analysis of nuclear magnetic resonance (MRI) images acquired in patients with VCP myopathy, a myopathy characterized by inclusion bodies and, inconstantly, Paget’s disease and/or fronto-temporal dementia :

  • the images of 80 patients from the large international cohort of 255 patients with VCP myopathy were usable,
  • they were analyzed and compared with MRI images of other neuromuscular diseases and with those of control subjects,
  • a relatively specific pattern emerged from this analysis, with predominant involvement of the periscapular, paraspinal, gluteal and quadricipital muscles, with a positive STIR sequence in 67% of cases.

 

Analysis of muscle magnetic resonance imaging of a large cohort of patient with VCP-mediated disease reveals characteristic features useful for diagnosis. Esteller D, Schiava M, Villar-Quiles RN, Dibowski B et al. J Neurol. 2023 Aug 21. doi: 10.1007/s00415-023-11862-4. Epub ahead of print. PMID: 37603075.