Etiology. Treatment

Etiology. The cause of narcolepsy has not yet been determined. Although a high association of certain class II human leukocyte antigens (HLAs) with narcolepsy accompanied by cataplexy suggests an immunologic pathogenesis, clinical evidence is lacking. The incidence of DR2 or DQwl antigens is estimated to exceed 90 percent in European, Caucasian, African-American and Japanese patients with narcolepsy associated with cataplexy. However, class II HLA typing is not a routine diagnostic test; these antigens may be present in non-narcoleptic patients, and some patients with narcolepsy associated with cataplexy do not carry these antigens.

Diagnosis. The diagnosis of narcolepsy is based on an overnight polysomnogram, which evaluates for sleep apnea, periodic limb movements of sleep or other causes of disturbed nocturnal sleep, and a multiple sleep latency test (MSLT) performed the following day. Positive MSLT results are required to confirm the diagnosis. This test consists of a series of four to five nap trials that objectively measure severity of daytime sleepiness. The primary parameters of interest are sleep latency and the presence of sleep-onset REM. Sleep latency refers to the amount of time that it takes a patient to fall asleep. A sleep latency period of less than five minutes is indicative of a pathologic sleepy state, and a sleep latency period of five to 10 minutes suggests pathologic sleepiness. Sleep-onset REM refers to REM sleep that occurs within 10 minutes after sleep onset.

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An average sleep latency of less than 10 minutes and sleep-onset REM in at least two nap trials are required to establish the diagnosis. Because sleep-onset REM can occur with REM-sleep deprivation from sleep apnea, sleep-wake schedule disturbances or drug or alcohol withdrawal, an overnight polysomnogram is used to help exclude such causes. A two-week period of alcohol or drug abstinence is required before an MSLT; abstinence may be confirmed with a drug screen before the study.

Treatment. Attempts to treat narcolepsy are often unsuccessful. Excessive daytime sleepiness and cataplexy are particularly difficult to treat. The mainstay of treatment for excessive daytime sleepiness is a combination of several planned daily naps and central nervous system stimulants, such as pemoline (Cylert), methylphenidate or amphetamine sulfate. To avoid development of tolerance to these agents, weekly drug vacations of one to two days are recommended. Cataplexy, sleep paralysis and hypnagogic hallucinations may be treated with tricyclic antidepressants, such as clomipramine, imipramine (Tofranil), nortriptyline and protriptyline, and serotonin reuptake inhibitors, such as fluoxetine (cheap Prozac online). Use of these drugs for this indication is not FDA-approved.

Coping with narcolepsy

Learning as much about narcolepsy as possible and finding a support system can help patients and families deal with the practical and emotional effects of the disorder, possible occupational limitations, and situations that might cause injury. A variety of educational and other materials are available from sleep medicine or narcolepsy organizations.

Support groups exist to help persons with narcolepsy and their families.

To imagine what a person with narcolepsy copes with daily, keep in mind that while many are not sleep-deprived (in the classical sense), a major symptom of narcolepsy is akin to sleep deprivation in a normal person; as a normal person, imagine going years functioning off just 3-4 hours of sleep per night. While lifestyle changes and drug therapy can help largely mitigate many symptoms of narcolepsy, there currently exists no complete and permanent solution, therefore patience, empathy and self-education are excellent coping tools.

Individuals with narcolepsy, their families, friends, and potential employers should know that:
Narcolepsy is a life-long condition that may require continuous medication.
Although there is no cure for narcolepsy at present, several medications can help reduce its symptoms.
People with narcolepsy can lead productive lives with proper medical care and lifestyle changes.
A major physiological and physical effect of narcolepsy is roughly akin to the effects of sleep deprivation; such effects can often be controlled and minimized through a combination of lifestyle changes and drug therapy.
Individuals with narcolepsy should avoid jobs that require driving long distances or handling hazardous equipment or that require alertness for lengthy periods (especially where the consequences of falling asleep are dangerous to themselves or others).
Parents, teachers, spouses, and employers should be aware of the symptoms of narcolepsy. This will help them avoid the mistake of confusing the person’s behavior with laziness, hostility, rejection, or lack of interest and motivation. It will also help them provide essential support and cooperation.
Employers can promote better working opportunities for individuals with narcolepsy by permitting special work schedules and nap breaks.
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