Oculopharyngeal muscular dystrophy (OPMD): management
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Oculopharyngeal muscular dystrophy (OPMD): management. This shows fatty replacement of muscles. Fully referenced neurology checklist.
Magnetic resonance imaging (MRI) muscle
- This shows fatty replacement of muscles
- It is especially marked in the adductor magnus and the soleus
- It correlates with disease duration and severity
Muscle biopsy: features
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Swallowing assessments
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Cardiac investigations
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Other investigations
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Treatment of dysphagia
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Other treatments
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Experimental treatments
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References
- Hill ME, Creed GA, McMullan TFW, et al. Oculopharyngeal muscular dystrophy: phenotypic and genotypic studies in a UK population. Brain 2001; 124:522-526.
- Blumen SC, Bouchard J-P, Brais B, et al. Cognitive impairment and reduced life span of oculopharyngeal muscular dystrophy homozygotes. Neurology 2009; 73:596-601.
- Bertorini TE. Neuromuscular Case Studies. Butterworth Heinemann Philadelphia 2008 pp494-495.
- Alonso-Jimenez A, Kroon RHMJM, Alejaldre-Monforte A, et al. Muscle MRI in a large cohort of patients with oculopharyngeal muscular dystrophy. JNNP 2019; 90:576-585.
- Tomé FM, Fardeau M. Nuclear inclusions in oculopharyngeal dystrophy. Acta Neuropathol 1980; 49:85-87.
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