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Interesting Findings Reported in Recent DM Research Studies on Facial Recognition and Sleep Disturbances

A recently published study from Sweden reported impaired facial recognition in people with DM1, and indicated that there are brain differences that affect how faces are perceived and stored by people with DM1. Dr. Katharine Hagerman, Research Associate at Stanford University Neuromuscular Division and Clinics, provides a summary of the Swedish facial recognition study. Dr. Hagerman also summarized a recent DM2 sleep survey that has drawn criticism from international DM experts in Italy.

Facial Memory Deficits in Myotonic Dystrophy Type 1
J Lundin Kleberg, C Lindberg, and S Winblad (2014) Acta Neruol Scand

Three Swedish researchers recently assessed cognitive differences seen in people with type 1 myotonic dystrophy (DM1). Their previous studies had shown that people with DM1 had a reduced ability to recognize facial emotions, and this correlated with lower sociability. In order to further assess factors affecting sociability in DM1, participants were given pictures of 15 different faces, and were later asked to pick out which faces they had seen before from a set of 30 pictures. Overall, 36% of participants with DM1 had impaired memory of faces, compared to 13% of participants without DM1.

Those with DM1 who had impaired memory of faces tended to falsely recognize faces (false positives), and upon further cognitive testing this group had reduced performance in tests of spatial coordination and motor skills. The researchers believe the impaired facial recognition seen in some people with DM1 indicates deficits in how the information about faces is perceived and stored. They suggest future studies should use eye-tracking to see how people with DM scan pictures to store information. They also recommended conducting MRI studies to see how the brain may differ both in structure and function in those with DM.

For the article abstract click here.  

Restless Legs Syndrome and Daytime Sleepiness are Prominent in Myotonic Dystrophy Type 2
EM Lam et al; 2013, Neurology 81(2):157-64
A recent study headed by Dr. Margherita Milone from the Mayo Clinic in Minnesota outlining sleep disturbances in those with myotonic dystrophy type 2 (DM2) has drawn criticism from a group of international experts in Italy headed by Dr. Gabriella Silvestri.  
Dr. Milone’s study examined the frequency of sleep disturbances by analyzing surveys filled out by 30 people with DM2, and 43 unaffected individuals. Her study found that those with DM2 had more clinically significant reports of daytime sleepiness, fatigue, altered sleep quality, and restless leg syndrome. Surprisingly, the study also found that obstructive sleep apnea was not a frequent sleep disturbance in DM2, going against previous small studies by Dr. Silvestri and others that estimated the prevalence of obstructive sleep apnea to be between 60% and 67% in DM2. 
Though Dr. Milone’s study was able to assess a larger number of affected individuals than other studies, Dr. Silvestri pointed out that it relied on paper-based surveys instead of more reliable clinical measurements from sleep monitoring.  Overall, studies on sleep disturbance in DM2 highlight the need for overnight and daytime sleep studies when individuals have symptoms that may stem from sleep issues, preferably performed in a sleep clinic that is able to differentiate between obstructive and central sleep apnea, along with assessing for sleepiness, fatigue, other forms of hypoventilation, periodic limb movements of sleep, restless leg syndrome, and REM sleep abnormalities.  
For the article abstract click here





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