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).
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