Characteristics of magnetic resonance imaging biomarkers in a natural history study of golden retriever muscular dystrophy

Zheng Fan, Jiahui Wang, Mihye Ahn, Yael Shiloh-Malawsky, Nizar Chahin, Sandra Elmore, C. Robert Bagnell, Kathy Wilber, Hongyu An, Weili Lin, Hongtu Zhu, Martin Styner, Joe N. Kornegay

Research output: Contribution to journalArticlepeer-review

41 Scopus citations


The goal of this study was to assess whether magnetic resonance imaging (MRI) biomarkers can quantify disease progression in golden retriever muscular dystrophy (GRMD) via a natural history study. The proximal pelvic limbs of ten GRMD and eight normal dogs were scanned at 3, 6, and 9-12. months of age. Several MRI imaging and texture analysis biomarkers were quantified in seven muscles. Almost all MRI biomarkers readily distinguished GRMD from control dogs; however, only selected biomarkers tracked with longitudinal disease progression. The biomarkers that performed best were full-length muscle volume and a texture analysis biomarker, termed heterogeneity index. The biceps femoris, semitendinosus and cranial sartorius muscles showed differential progression in GRMD versus control dogs. MRI features in GRMD dogs showed dynamic progression that was most pronounced over the 3- to 6-month period. Volumetric biomarkers and water map values correlated with histopathological features of necrosis/regeneration at 6-months. In conclusion, selected MRI biomarkers (volume and heterogeneity index) in particular muscles (biceps femoris, semitendinosus, and cranial sartorius) adjusted for age effect allow distinction of differential longitudinal progression in GRMD dogs. These biomarkers may be used as surrogate outcome measures in preclinical GRMD trials.

Original languageEnglish (US)
Pages (from-to)178-191
Number of pages14
JournalNeuromuscular Disorders
Issue number2
StatePublished - Feb 2014
Externally publishedYes


  • Biomarker
  • GRMD
  • MRI
  • Muscular dystrophy
  • T2 mapping
  • Texture analysis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Clinical Neurology
  • Genetics(clinical)


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