pector@ihuxw.UUCP (Scott W. Pector) (04/29/84)
<> The following is a response to a request from Denis Johnson for more info on narcolepsy and its treatment. His request appeared in net.med a couple of weeks ago. This is response is from my wife, Beth, who is about to get her MD at the U. of Chicago: Pritzker School of Medicine. Any questions for her should either be posted in net.med or sent to me. I hope this response is informative for you. Scott Pector ihnp4!ihuxw!pector ___________________________________________________________________________ The following is some general information on narcolepsy from Adams and Victor's *Principles of Neurology, pp. 269-271. I'm mentioning some things you undoubtedly already know for the benefit of other net readers, and also their treatment guidelines. I'd strongly recommend further consultation with your wife's primary physician and/or neurologist to help with her individual case. I. Background *Narcolepsy* is a syndrome of recurrent attacks of irresistible sleepiness. It is frequently associated with cataplexy (in about 70% of all narcoleptics and almost all those narcoleptics who have REM sleep attacks demonstrated on EEG monitoring). *Cataplexy* is a strange phenomenon which occurs during hearty laughter or, less often, excitement, sadness, or anger. The patient's head falls forward, jaw drops, knees buckle and he/she may fall to the ground although completely conscious. This is a temporary paralysis of voluntary muscles lasting from a few seconds to minutes. *Sleep paralysis* is a brief loss of voluntary motion during the period of falling asleep. It is often preceded by or associated with *hypnagogic hallucinations*, vivid, terrifying visual, auditory, vestibular (motion) or somatic (enlargement or transformation of a body part) illusions. All four of the above characteristics highlighted by *--* are often part of the narcolepsy disorder, although occasional sleep paralysis or hypnagogic hallucinations occur in otherwise normal individuals. Another characteristic is eisodic lapses of consciousness, akin to sleepwalking but occurring during the day. For a few seconds to an hour, usually in afternoon or evening hours while performing monotonous tasks (e.g., driving), the patient loses track of what's going on. He/she may continue their task, but can't respond to complex questions or demands. A sudden burst of words unrelated to the context may terminate this episode, which is usually not remembered. These attacks occur in over half of narcoleptic- cataplectic patients according to one study. Sleep paralysis and hypnagogic hallucinations occur in about 25% of patients, and all four major features occur in about 10%. Narcoleptic sleep attacks occur two to six times daily as an average, often in unusual circumstances (while standing, eating, conversing). They last up to 15 minutes unless the person is reclining, when they may last an hour or more. The patient is easily aroused. Sleep attacks usually occur after meals or while inactive but may occur in above-mentioned situations, too. The syndrome starts in late childhood, adolescence or early adulthood, affecting males more often than females, and usually with narcolepsy as the presenting symptom. Mayo Clinic sees about 100 new cases of narcolepsy annually. Sleep attacks are usually brief episodes of REM (rapid- eye-movement) sleep, although not all of them are. Hypnagogic hallucinations, cataplexy and sleep paralysis are associated with REM sleep. Normal persons' sleep begins with non-REM sleep, progressing through stages 1,2,3,4, back to stage 2, then into the first REM period about 70-100 min. after the onset of sleep. Much of the preliminary period is spent in stages 3 and 4. After the first REM period the non-REM stages repeat, then REM, and 4 to 6 cycles occur per night. In contrast, narcoleptics' nocturnal sleep characteristically begins with REM sleep, and the sleep pattern is disturbed, with frequent body movements, transient awakenings and decreased stage 3 and 4 sleep. Total sleep time is reduced. In nap situations, sleep latency (time between trying to fall asleep and detection of sleep EEG pattern) is greatly reduced in narcoleptic patients. Thus, many aspects of sleep and wakefulness are disorganized in narcolepsy. II. Treatment "No single therapy will control all the symptoms." Drugs, which presumably work by inhibiting REM sleep, are part of the therapy. You mention an amphetamine (which could be Dexedrine). Other drugs include Ritalin, tricyclic antidepressants (imipramine, clomipramine), and monoamine oxidase inhibitors (phenelzine, pargyline--effective but impractical due to potentially severe side effects). Time of medication is adjusted to the patient's schedule. Dosage of amphetamines is 5 to 10 mg three to five times daily. Ritalin dosage is 10 to 20 mg three times daily. The main effectiveness of amphetamines is in reducing sleep attacks, according to Adams and Victor. Amphetamines reportedly have little effect on cataplexy, although it sounds like your wife's cataplexy has responded well. Tricyclic antidepressants (imipramine = Tofranil, or clomipramine) are given in doses of 25 mg three or four times daily. These can reduce cataplexy and sleep paralysis effectively, and are often combined with Dexedrine or Ritalin therapy to maximize symptom relief. Tolerance can develop to all of the drugs mentioned over 6 to 12 months, and switching between drugs as well as intervals of drug cessation are required in managing narcolepsy with medication. Also important are strategically planned 15 minute naps (e.g., during lunch hour, before or after dinner, and presumably at other times of high risk for sleep attacks). If your wife hasn't tried this, it should hopefully help. Unfortunately narcolepsy is essentially life-long once it appears, although it may become less severe with age. No associated neurolo- gic abnormalities have been known to occur. The cause of narcolepsy is unknown. I hope this information is helpful to you and your wife. Again, I suggest you discuss her problems in detail with a neurologist, who may have additional recommendations or knowledge of experts in the field. Sincerely, Beth Pector