Centronuclear myopathy (CNM): management
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Centronuclear myopathy (CNM): management. Large centrally located nuclei, Necklace fibers, Dystrophic change. Fully referenced neurology checklist.
Muscle biopsy: features
- Large centrally located nuclei
- Necklace fibers
- Dystrophic change
- Radial cytoplasmic strands around the central nuclei
Other investigations
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Physical therapy
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Treatment of drooling
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Management of swallowing and feeding
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Bone health management
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Management of contractures
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Scoliosis management
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Mobility aids
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Vaccinations
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Respiratory management
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Other management
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References
- Cassandrini D, Trovato R, Rubegni A, et al; Italian Network on Congenital Myopathies. Congenital myopathies: clinical phenotypes and new diagnostic tools. Ital J Pediatr 2017; 43:101.
- Wang CH, Dowling JJ, North K, et al. Consensus statement on standard of care for congenital myopathies. J Child Neurol 2012; 27:363-382.
- Abath Neto O, Silva MR, Martins Cde A, et al. A study of a cohort of X-linked myotubular myopathy at the clinical, histologic, and genetic levels. Pediatr Neurol 2016; 58:107-112.
- Bevilacqua JA, Bitoun M, Biancalana V, et al. "Necklace" fibers, a new histological marker of late-onset MTM1-related centronuclear myopathy. Acta Neuropathol 2009; 117:283-291.
- Gurgel-Giannetti J, Zanoteli E, de Castro Concentino EL, et al. Necklace fibers as histopathological marker in a patient with severe form of X-linked myotubular myopathy. Neuromuscul Disord 2012; 22:541-545.
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