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Lead investigator

Collaborators & Co-Investigators

• Marcos Sampaio, MD (PI)
• Gerd Melkus, PhD (PI)
• Ian Smith, PhD
• Cynthia Gagnon, PhD

Research Sites & Affiliations

• University of Ottawa, Ottawa, Ontario
• Ottawa Hospital Research Institut, Ottawa, Ontario
• Université de Sherbrooke, Sherbrooke, Quebec

Budget: $100,000

Disorders: Oculopharyngeal muscular dystrophy

Research Areas: Understand Diagnosis and Disorder Progression

Abstract: 

Oculopharyngeal muscular dystrophy (OPMD) is a muscle disease and causes swallowing problems with face and limb weakness. OPMD is one of the most common muscular dystrophies in Canada. With new medications being tested, better methods are needed to assess how OPMD worsens or may improve with treatment. Standard MRI of the muscle can show some changes in the muscle and help with diagnosis. However, new ‘quantitative’ MRI (qMRI) can carefully assess the increasing fat in muscle as the muscular dystrophy progresses. However, qMRI has not been used to assess whole body muscle disease progression in OPMD patients.

We will use new qMRI methods to assess muscle involvement and disease progression in OPMD with whole body muscle qMRI over 1 year. We will also assess the link between qMRI and clinical weakness and strength testing. Our study will help patients with muscular dystrophy. We will study one of the largest groups with OPMD with muscle qMRI followed over time and determine if MRI predicts the disease progression. Also, this study will help guide using qMRI in future treatment trials in OPMD.

Impact: