Table of Contents
Journal of Neurodegenerative Diseases
Volume 2013 (2013), Article ID 692026, 13 pages
http://dx.doi.org/10.1155/2013/692026
Review Article

Sleep-Wake Cycle and Daytime Sleepiness in the Myotonic Dystrophies

1University of Rome “Tor Vergata”, Policlinico Tor Vergata, Department of Systems Medicine, Section of Neurophysiopathology, Sleep and Epilepsy Centre, 00133 Rome, Italy
2University of Rome “Tor Vergata”, Policlinico Tor Vergata, Department of Systems Medicine, Section of Neurosciences, 00133 Rome, Italy
3Fondazione S. Lucia IRCCS, 00100 Rome, Italy

Received 9 April 2013; Revised 19 July 2013; Accepted 3 August 2013

Academic Editor: Eng King Tan

Copyright © 2013 A. Romigi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.