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

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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.

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