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This study evaluates the diagnostic usefulness of CEST MRI in a Pompe disease mouse model, focusing on muscle glycogen levels.

May 6, 2026

Detection of Altered Muscle Glycogen‐ and NOE ‐Weighted CEST MRI Signals in an Acid Alpha‐Glucosidase‐Deficient Mouse Model of Pompe Disease Using QUASS ‐Enhanced Multi‐Pool Quantitative CEST ( QCEST ) Imaging

Key Points

This study evaluates the diagnostic usefulness of CEST MRI in a Pompe disease mouse model, focusing on muscle glycogen levels.
Conducted multiparametric MRI at 7 T Bruker scanner
Included T1 and T2 relaxation mapping
Performed densely sampled CEST Z-spectral acquisition on GAAKO and wild-type mice
Analyzed correlation between relaxation signals and glycogen assay
Used spinlock model-based fitting for multi-pool CEST signals
Found significant correlations between CEST metrics and glycogen levels (p < 0.0001)
Demonstrated potential of multi-pool CEST MRI for characterizing muscle pathology in GAAKO mice

Abstract

PURPOSE: Chemical exchange saturation transfer (CEST) MRI is a non-invasive imaging technique that detects dilute labile protons with enhanced sensitivity, aiding in the assessment of tissue properties. This study aimed to evaluate the diagnostic usefulness of CEST MRI in acid alpha-glucosidase knockout (GAAKO) mice, a model of Pompe disease characterized by lysosomal glycogen accumulation in muscles. METHODS: We conducted multiparametric MRI at a 7 T Bruker MRI scanner, including T1 and T2 relaxation mapping and densely sampled CEST Z-spectral acquisition, on adult GAAKO and wild-type control mice. Multi-pool CEST signals were obtained from spinlock model-based fitting of the quasi-steady-state reconstructed Z-spectrum. Using the gold-standard muscle glycogen assay as a biomarker of disease burden, we analyzed the correlation between relaxation and CEST signals and tissue glycogen levels. RESULTS: = 0.57, p < 0.0001) demonstrated significant correlations with glycogen assay measurement. CONCLUSION: Multi-pool CEST MRI metrics show potential for characterizing muscle pathology in GAAKO mice. These findings support using CEST MRI to monitor tissue glycogen levels non-invasively.

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