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Abstract Details

Integration of Muscle Fat Fraction and Apparent Diffusion Coefficient Analyses to Track Disease Progression in Oculopharyngeal Muscular Dystrophy Using Whole-body Quantitative MRI
Neuromuscular and Clinical Neurophysiology (EMG)
S7 - Updates on Muscle Disorders (5:06 PM-5:18 PM)
009
To evaluate 97 muscles cross-sectionally and longitudinally, quantify fat fraction (FF), and test whether diffusion related MRI markers like Apparent Diffusion Coefficient (ADC) precede or correlate with fat infiltration.
Oculopharyngeal muscular dystrophy (OPMD) a slowly progressive disorder marked by ptosis, dysphagia, and proximal weakness. Quantitative MRI (qMRI) is an emerging biomarker that may support severity assessment and monitoring.

We studied 31 patients with OPMD and 10 controls. All underwent whole body 3T qMRI with two-point Dixon for FF, ADC for diffusivity . Seven patients had follow-up scans after 22.7 ± 4.1 months. We segmented 97 muscles for FF and ADC, using ITK SNAP 3.8.0. Analyses used Prism and R using Wilcoxon, Mann Whitney tests and Pearson or Spearman correlations, p<0.05.

Adductor Magnus (AM)  Soleus (SO), Biceps Femoris (BL), Semi Membranous (SM), Tibialis Posterior (TP)  showed higher FF, and FF increased with age (<55 y 17%, 55–65 y 34.9%, >65 y 54.3%) and disease duration. Within muscles (AM, BL, SM, SO), higher FF was linked to a negative correlation between FF and ADC (spearman rho=-0.55, p value =<0.05). Muscles (AL, TP, AM, Serratus Anterior, SM) showed significant within subject FF increases (range= +2.1 to +5.7 % absolute change). Muscles with higher mean FF and wider between patient ranges of FF and ADC have a stronger negative (FF-ADC Pearson correlation co-efficient R²=0.49, 0.59, 0.30). Baseline ADC did not predict later FF change (Spearman rho range: -0.35 to +0.30 p value>0.05).
FF is sensitive marker of progression and shows a robust within-muscle negative relationship with ADC. ADC is poor standalone marker and does not predict future FF change in this cohort. Future analysis would aim to provide muscle-specific context (e.g., mean-or control-subtracted ADC), assess the presence of edema and increase the sample size to potentially enhance ADC’s effectiveness in monitoring OPMD.
Authors/Disclosures
Tanay Satarkar, MD, MSc candidate
PRESENTER 		Dr. Satarkar has nothing to disclose.
Ian C. Smith, PhD Dr. Smith has nothing to disclose.
Gerd Melkus, PhD Dr. Melkus has nothing to disclose.
Marcos L. Sampaio, MD Dr. Sampaio has nothing to disclose.
Fadi Esttaifo, BHSc, MSc Mr. Esttaifo has nothing to disclose.
Kaitlynn Meier-Ross Ms. Meier-Ross has nothing to disclose.
Sono Khan (University of Ottawa) No disclosure on file
Jodi Warman, MD (The Ottawa Hospital) The institution of Dr. Warman has received research support from Muscular Dystrophy Canada. The institution of Dr. Warman has received research support from CIHR. Dr. Warman has received research support from OHRI. The institution of Dr. Warman has received research support from Department of Medicine.