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Narcolepsy (cont.)

What's in the future for narcolepsy?

The discovery that a lack of hypocretins in the cerebrospinal fluid (CSF) may be related to the cause of narcolepsy could lead to the development of tests to determine the level of hypocretins in the CSF. Such tests could help in the diagnosis of narcolepsy. The expectation is that these tests will be simple (drawing blood), and will reflect the level of hypocretins in the CSF. In addition, the discovery of the role of hypocretins in the development of narcolepsy may lead to the development of new drugs for the treatment of narcolepsy.

Narcolepsy At A Glance

  • Narcolepsy is a chronic disease of the central nervous system. The symptoms include excessive daytime sleepiness (EDS), loss of muscle tone (cataplexy), distorted perceptions (hypnagogic hallucinations), inability to move or talk (sleep paralysis), disturbed nocturnal sleep, and automatic behavior.

  • Narcolepsy usually begins in teenagers or young adults and affects both sexes equally.

  • The prevalence of narcolepsy is similar to that of Parkinson's disease and multiple sclerosis. Approximately 125,000 to 200,000 Americans are estimated to suffer from narcolepsy, but only fewer than 50,000 are actually properly diagnosed.

  • Abnormalities in the structure and function of a particular group of nerve cells in the brain called hypocretin neurons are thought to play a role in the development of narcolepsy.

  • The diagnosis of narcolepsy is based on a clinical evaluation, specific questionnaires, sleep logs or diaries, and the results of sleep laboratory tests (polysomnography and multiple sleep latency test).

  • Treatment options for narcolepsy include drug and behavioral modification therapies and disease-specific education of the patient and family members. The treatment should be individualized, depending on the types and severity of the symptoms, the life conditions of the patients, and the specific goals of therapy.

  • Optimal management usually takes weeks to months to achieve and requires continued communication among the physician, patient, family members, and others.

  • Alerting medications are used for the treatment of excessive daytime sleepiness. Amphetamines and methylphenidate (Ritalin) are general CNS stimulants that decrease sleepiness and improve alertness. Modafinil (Provigil) has alerting effects similar to those of the traditional stimulants but has less undesirable side effects.

  • Anticataplectic medications are used to treat cataplexy, hypnagogic hallucinations, and sleep paralysis. Tricyclic antidepressants (TCAs) are often effective in controlling cataplexy, but also frequently produce side effects that could limit their use.

  • Selective serotonin reuptake inhibitors (SSRIs) are also useful in the treatment of cataplexy and their side effects are milder. Sodium oxybate (Xyrem) is a medication with anticataplectic effects that also improves disturbed nocturnal sleep.

  • Behavioral approaches to treating narcolepsy include establishing a structured sleep-wake cycle and planned naps, and involve diet, exercise, and occupational, marriage, and family counseling.


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