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Wednesday, May 4, 2016
Narcolepsy and basal ganglia infarction
I have recently been diagnosed with narcolepsy and Idiopathis hypersomnia. I have had both a Sleep Study and MSLT. I have suffered for years without knowing what was wrong with me. I can sleep for hours and then go right back off to sleep. Some weekends I`ll just stay in bed and sleep. I never feel rested. My job as an Assistant Manager of a Bank is extremely difficult. During my consult, I mentioned that I had had Meningitis as a child.
An MRI w/out contrast was done and the impression is: Tiny left basal ganglia lacunar infarction. Along with the symptoms of excessive daytime somnolence, sleep paraylsis. I never feel or get hungry. I also experience extreme variations in my body temperature. At night my body will get so hot and sometimes I have chills for no reason. Could there be a relation with any of this? Could the infarction have caused scarring in the hypothalamic sleep zones?
I am currently on Provogil 200mg bid and adderal 20 mg in the am and 10mg after lunch. Not to mention that I have been on Prozac for 15+ years. Someone who sleeps all the time seems depressed. The Sleep specialist recommended I decrease the dosage of Prozac 20mg to every other day. This has resulted in extreme emotions, irritability and extreme sadness. I have just recently been diagnosed and it all seems overwhelming. Can you share your thoughts? Thank you in advance.
Assuming you have been correctly diagnosed with narcolepsy, then your question really centers on the possible cause and appropriate treatments of this condition. Before answering that, it might be useful to provide a brief overview of this disease and its diagnosis.
Narcolepsy affects about 1 in 2000 people. Narcolepsy is characterized by excessive sleepiness and abnormal intrusions of rapid eye movement (REM) sleep phenomena into awake time. Typically, during REM sleep, our muscles are inactive (except for our breathing muscles and eye muscles) and unable to move. In individuals with narcolepsy, this "paralysis" of muscles may occur during wakefulness (known as cataplexy, usually brought on by strong emotions) or during transitions between sleep and wakefulness (sleep paralysis). In addition, dreams may intrude up in these times as well (known as hypnagogic or hypnopompic hallucinations). Other symptoms of narcolepsy may include poor nighttime sleep and automatic behaviors (performing tasks without being aware of what you are doing or having recall of having done it).
While true cataplexy (brief, sudden, bilateral loss of muscle tone brought on by strong emotions) is considered very strong evidence of narcolepsy, the other symptoms listed above may be seen in individuals without narcolepsy. Furthermore, individuals diagnosed with narcolepsy may not have any or have only some of the other associated symptoms, though they are always sleepy. The diagnosis of this condition is usually determined by a Sleep Specialist after taking a thorough history, performing an examination, and obtaining objective testing - a sleep study and a daytime nap study (known as a Multiple Sleep Latency Study or MSLT).
In your question you mention that you are always sleepy and fatigued and that your sleep is nonrestorative. This is somewhat unusual for narcolepsy. Patients with narcolepsy experience quite restorative sleep. In fact, one of the treatments classically used has been to have the narcoleptic take scheduled naps. Short naps are often very refreshing for narcoleptics and may help to prevent sleepiness for a few hours. You also mention you were told you had “idiopathic hypersomnia” along with narcolepsy. This would be a very unusual combination of diagnoses as there are features to each that tend to exclude the other and diagnosing one in the setting of the other is difficult. It would probably be worthwhile to seek a second opinion about what your true diagnosis is, as this is critical to determining how best to treat you.
If we accept that your diagnosis is narcolepsy, then determining a cause is not easy. In most cases of narcolepsy, we don’t know what the underlying cause is. However, recent research is beginning to provide some clues and certainly problems in the brainstem may be related to the development of narcolepsy and other sleep conditions. It is conceivable, though I don’t think we can say for sure one way or the other, that the lacunar infarct in your hypothalamus (in the brainstem) may be affecting your level of alertness. Part of the wake-sleep control center for the brain runs through the hypothalamus and thus damage to this area could affect sleep and wake control.
There are several available options that are FDA-approved for the excessive daytime sleepiness. The traditional treatment has been with stimulant medications. There are a number of these that are available and FDA-approved, including caffeine, modafinil (i.e. Provigil), methylphenidate (i.e. Ritalin), and dexamphetamine (i.e. Dexedrine). Adderall is a mixture of amphetamines that is FDA-approved for the treatment of narcolepsy. Aside from stimulants, a newer non-stimulant medication, Sodium Oxybate (i.e. Xyrem) is also approved for the treatment of cataplexy and excessive sleepiness related to narcolepsy. It’s a little unusual to use a combination of stimulants to treat the excessive daytime sleepiness, and this may warrant a closer look at your medication usage.
You and your doctor should review your studies and treatments to find the right combination of medications and behavioral modifications to control your symptoms. It may be worthwhile to seek a second opinion to make sure you have the correct diagnosis and are on proper therapy. Simple maneuvers, such as practicing good sleep hygiene (please see the website for details) and scheduled regular naps may help tremendously. Most patients can have their symptoms almost completely controlled with a combination of medications and behavioral modifications.
To learn more about narcolepsy or other sleep disorders, please visit the American Academy of Sleep Medicine's website at. In addition to information, the website contains a list of Sleep Centers across the country so that you may locate one near you. The Narcolepsy Network's website also provides information and links to support groups for those with this condition. Good luck and here's to good sleep!
Dennis Auckley, MD
Associate Professor of Medicine
School of Medicine
Case Western Reserve University