E. Riedel, Vincent Gmeiner, T. Haack, J. Kirschke, M. Deschauer
2026.2.5Clinical Neuroradiology
Abstract
The diagnostic evaluation of myopathies can be challenging, particularly in adults and elderly patients with comorbidities and atypical clinical presentations. While a few hereditary myopathies such as myotonic dystrophy types 1 (DM1) and 2 (DM2) or facioscapulohumeral muscular dystrophy (FSHD) present with sufficiently characteristic phenotypes to allow targeted genetic testing, most patients exhibit limb-girdle weakness with a broad differential diagnosis. In older individuals, acquired conditions—especially idiopathic inflammatory myopathies—are often considered first. But also late-onset hereditary myopathies, including autosomal-dominant and autosomal-recessive limb-girdle muscular dystrophies (LGMDs) as well as the x‑linked rarely late-manifesting Becker muscular dystrophy, which typically presents in adolescence or early adulthood, must be considered.
Citation format
RIEDEL, E., et al. Diagnostic value of muscle MRI in a case of very late-onset becker muscular dystrophy. Clinical Neuroradiology, 2026.