AI Article Synopsis
- Myotonic dystrophy is the most prevalent muscular dystrophy in adults, marked by progressive muscle weakness, myotonia, and impact on multiple organs.
- There are two types: DM1 (Steinert's Disease) and DM2, both linked to significant sleep issues.
- DM1 patients often experience various sleep disturbances such as sleep-disordered breathing, periodic limb movements, and central hypersomnia, while research on sleep disorders in DM2 remains limited, highlighting the need for more in-depth studies.
Article Abstract
Myotonic dystrophy is the most common type of muscular dystrophy in adults and is characterized by progressive myopathy, myotonia, and multiorgan involvement. Two genetically distinct entities have been identified, myotonic dystrophy type 1 (DM1 or Steinert's Disease) and myotonic dystrophy type 2 (DM2). Myotonic dystrophies are strongly associated with sleep dysfunction. Sleep disturbances in DM1 are common and include sleep-disordered breathing (SDB), periodic limb movements (PLMS), central hypersomnia, and REM sleep dysregulation (high REM density and narcoleptic-like phenotype). Interestingly, drowsiness in DM1 seems to be due to a central dysfunction of sleep-wake regulation more than SDB. To date, little is known regarding the occurrence of sleep disorders in DM2. SDB (obstructive and central apnoea), REM sleep without atonia, and restless legs syndrome have been described. Further polysomnographic, controlled studies are strongly needed, particularly in DM2, in order to clarify the role of sleep disorders in the myotonic dystrophies.
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|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4437277 | PMC |
| http://dx.doi.org/10.1155/2013/692026 | DOI Listing |
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