Interventions helps 30% of myotonic dystrophy patients with sleep issues

Sleepiness and Sleep-related Breathing Disorders in Myotonic Dystrophy and Responses to Treatment: A Prospective Cohort Study

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Provigil Survey Finds Strong Support From Myotonic Dystrophy Patients

For quite a few years, I have been strongly advocating the use of Provigil the brand name for modafinil for helping with sleep related issues n Myotonic Dystrophy. Many patients with myotonic dystrophy have tremendous issues with excessive sleep and sleep disruptions.  A study from France in 2009 concluded that a controlled study showed “no significant effects on daytime sleepiness” This study puzzled me as many patients reported very good results from this drug.

A survey conducted by the Myotonic Dystrophy Support Group,  MDSG in England has concluded that over 80% of survey respondents found that the drug had a marked benefit or a dramatic benefit. This concurs with the informal feedback that I have had here in the USA. This also focuses attention on patient centered approaches versus medical centered approaches. Patient centered approaches are very important to give direct patient feedback rather than just visibility to medical professionals opinions based on limited patient facts.

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Daytime Sleepiness in Myotonic Dystrophy Causes

This is a study (1994)  that looked at the causes of sleepiness in patients with myotonic dystrophy. They concluded that these sleep issues are not caused by sleep apnea. A number of the patients were able to get some relief with Ritalin. Ritalin has a low amount of cardiac side effects. However, this drug may have some cardiac side effects so clear it with your doctor cardiologist before use

Daytime Sleepiness is not caused by Sleep Apnea

J Neurol Neurosurg Psychiatry. 1994 May;57(5):626-8.

Daytime sleep in myotonic dystrophy is not caused by sleep apnoea.


Department of Neurology, University Hospital Rotterdam-Dijkzigt, The Netherlands.


Daytime sleepiness is common in myotonic dystrophy and might be attributed to disturbed nocturnal breathing. Seventeen out of 22 patients complained of excessive daytime sleepiness, resembling “idiopathic hypersomnolence”. Sleep apnoea might have contributed to daytime sleepiness in only three of 17 patients. Treatment with the central stimulant methylphenidate produced sustained benefit in seven out of 11 patients tested. Daytime sleepiness in myotonic dystrophy is usually caused by dysfunction of central sleep regulation and not by disturbed nocturnal breathing.

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Ritalin and Daytime Sleepiness in Myotonic Dystrophy

Sleepiness is a  issue with some adults and children with Myotonic Dystrophy. Sleepiness or Hypersomnia can have great impact on the social life of patients with myotonic dystrophy. Some patients have reported relief with Provigil although studies have indicated that there is no overall benefit. Provigil may work to help mobilize the sleep patterns so that a person can attend an event but then catch up on sleep later. A new study is looking at a different drug.

This study is by one of the worlds experts in the Disease (DM1) Dr. Puymirat in Quebec, Canada. This article  studies Dr. J PuyrimatRitalin (20mg) and Myotonic dystrophy related to sleepiness with some good results. However, it is noted that palpitations are a side effect of the study. The risks with Ritalin are low but make sure to see a cardiologist before using or having Ritalin prescribed.

Clin Ther. 2012 May;34(5):1103-11.
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Sleeping problems and energy myotonic dystrophy

Many individuals with myotonic dystrophy run into problems with excessive sleeping (hypersomnia) or problems with energy levels as the disease causes individuals to lose focus and direction. At the 11th annual conference in Liverpool England they said that a new drug had helped some individuals. This drug is known as Modafinil or in the USA under the trade name Provigil.

The doctors at the conference stated that the drug had worked remarkable well for some patients and that it had let them lead a more normal life. Dr. Miller at the MD clinic in San Francisco had some familiarity with the drug and suggested a trial.

My wife has taken the drug only a few times in the last few weeks but it has made a world of difference. Her energy level has picked up. She previously was unable to do many tasks as she was tired. Now she can do many more tasks. She used to sleep 14-18 hours a day. Now she can function at more normal levels.

Another individual took the drug and it worked for a week but then the effects seemed to wear off and she slept as much as she did before.

Information from the Quest Magazine Vol 7 #5 (October 2000)

In Myotonic Dystrophy the part of the brain that  controls the level of arousal and alertness is often involved. People with myotonic dystrophy even after their respiratory problems have been brought under control with assisted ventilation may need additional help in the form of a stimulation medication.

David Rye a neurologist and sleep specialist at Emory university has used pemoline (Cylert) methylphenidate (Ritalin), and modafinil (Provigil) for this purpose when patients with myotonic dystrophy and daytime sleepiness come to him from the MDA clinic. He says that recent breakthroughs concerning proteins in narcolepsy (a brain disorder that causes people to fall asleep frequently during the day) may have application to the daytime sleepiness of myotonic dystrophy.

Rye uses a multiple sleep latency test in which the subject is asked to take 4-5 naps at two hour intervals as a “way of putting a number” on daytime somnolence. The test measures the kind of daytime sleep experienced (REM sleep is unusual in the normal napper) and the time it takes to fall asleep during the day.

From the same article in Quest

Myotonic dystrophy affects not only the muscles in breathing but also the cells in the brain that control how we breathe. During sleep many people with this disorder can fail to breathe normally because of this brain factor a condition that is known as central sleep apnea (because of the involvement of the central nervous system)

The usual treatment for sleep apnea whether its obstructive or central and for ineffective nighttime ventilation  is noninvasive positive pressure ventilation (NIPPV). This means using a small ventilator that pumps air into the lungs via a mask that fits over the nose or nose and mouth to assist in breathing

The type of NIPPV that is usual used in neuromuscular disease is bilevel positive airway pressure or BiPAP. Bilevel pressure as contrasted to continuous pressure (CPAP) allows lower pressure to exhale against and delivers a higher pressure on inhalation.

FROM MDA Site ask the experts: REPLY from MDA: David Rye, M.D., Ph.D., Emory  University  School of Medicine,Atlanta, GA 30322

Excessive daytime sleepiness is exceedingly common  in myotonic dystrophy (about 85 percent). Unfortunately, it has not been systematically studied and there is a lack of objective data on its prevalence, cause and treatment. Many patients with muscular dystrophies exhibit sleep apnea, which can fragment sleep, resulting in  numerous arousals and unrefreshing sleep with daytime sleepiness. It has been estimated that nearly 50 percent of patients with “neuromuscular” diseases have clinically significant sleep apnea. Therefore, it is wise to have a sleep study performed to rule out the presence of sleep apnea. This can even be a portable study at home for convenience. If sleepiness exists in the absence of sleep apnea or with treated sleep apnea, treatment with wake-promoting drugs like Provigil is certainly warranted. We have observed many favorable responses with Provigil in patients with  myotonic dystrophy and are advocates of its use.  Side effects are minimal compared to other classic psychostimulants such as methylphenidate (Ritalin)and dexamphetamine (dexedrine).

Articles to Review

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Provigil and narcolepsy
Provigil and Multiple sclerosi

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