Narcolepsy is characterized by excessive daytime sleepiness chronsische, often with sudden loss of muscle tone (cataplexy). Other symptoms include insomnia and hypnagogic hallucinations and hypnopompic. The diagnosis is made by using the polysomnography and the multiple sleep latency test. Treatment is with modafinil, various stimulants or sodium oxybate against the excessive daytime sleepiness and establishing certain antidepressants against the accompanying symptoms.
The cause is unknown. In Europe, Japan and the US, the incidence is 0.2-1.6 / 1000th Narcolepsy occurs equally in both sexes.
Narcolepsy is characterized by excessive daytime sleepiness chronsische, often with sudden loss of muscle tone (cataplexy). Other symptoms include insomnia and hypnagogic hallucinations and hypnopompic. The diagnosis is made by using the polysomnography and the multiple sleep latency test. Treatment is with modafinil, various stimulants or sodium oxybate against the excessive daytime sleepiness and establishing certain antidepressants against the accompanying symptoms. The cause is unknown. In Europe, Japan and the US, the incidence is 0.2-1.6 / 1000th Narcolepsy occurs equally in both sexes. Narcolepsy is strongly associated with specific HLA haplotypes. Children of patients with narcolepsy have a 40-fold increased risk of narcolepsy, suggesting a genetic cause. However, the concordance in twins low (25%), suggesting an important role of environmental factors that often trigger the disorder. The neuropeptide hypocretin-1 is missing in the CSF narkoleptischer animals and most human patients, which leads to the assumption that the cause could be an HLA-associated autoimmune-mediated destruction of hypocretin-containing neurons in the lateral hypothalamus. Narcolepsy is characterized by a dysregulation of the timing and control of REM sleep. Therefore, the REM sleep intrudes into the waking periods and Einschlafmuster from waking to sleep. Many symptoms of narcolepsy are caused by paralysis of the postural muscles and vivid dreams that characterize REM sleep. There are two types: Type 1: Narcolepsy with hypocretin deficiency and accompanied by cataplexy (current Muskelschwächne or paralysis due to sudden emotional reactions caused) Type 2: Narcolepsy with normal hypocretin values ??and without cataplexy symptoms and complaints The main symptoms are excessive daytime sleepiness ( ETS) cataplexy hypnagogic hallucinations and hypnopompic. Sleep paralysis About 10% of patients have all four of these symptoms. The night sleep is often also disturbed and some patients develop hypersomnia (prolonged rest periods). Symptoms usually begin in adolescence or in young adults with no pre-existing disease, although the onset may be favored by a disease, a stressor or a period of sleep deprivation. Once it has occurred, there is narcolepsy for the entire life. Life expectancy is unaffected. ETS: An ETS can occur at any time. The sleep episodes vary from few to many throughout the day, and each can take minutes to hours. Patients may desire to sleep only temporarily resist, but they may just be awakened as from normal sleep. The sleep is preferred during a featureless environment situations (eg. As reading, watching television, attending meetings), but can also occur during complex activities (eg. As driving a car, speaking, writing, eating). Patients may also experience sleep attacks – episodes of sleep they attacked without warning. Patients can feel refreshed when they are awake and still go back to sleep within minutes. The night’s sleep may not be satisfactory and are interrupted by vivid, frightening dreams. The consequences may include: low productivity, interpersonal relationship crashes, poor concentration, low motivation, depression, a dramatic reduction in the quality of life and the risk of physical injury (especially traffic accidents). Cataplexy: there is a momentary muscular weakness or paralysis without loss of consciousness; this is caused by sudden emotional reactions such as happiness, anger, fear, joy, or, often, surprise. The weakness may be limited to the extremities (z. B. let the patient’s Angel fall when a fish bites), or they can during a hearty laughter [ “weak with laughter”] fall apart or sudden anger. A cataplexy may also affect other muscles: The jaw can hang, facial muscles may flicker, the eyes may be closed, the head can nod, and the language can be indistinct. These attacks resemble the loss of muscle tone that occurs during REM sleep. Cataplexy occurs in about three-quarters in front of patients. Sleep paralysis: Patients are momentarily unable to move while they just fall asleep or immediately after they have woken up. These occasional episodes can be very scary. They resemble the motor inhibition that accompanies REM sleep. Sleep paralysis occurs in a quarter of the patients, but also in some healthy children and rare in healthy adults. Hypnagogic hallucinations or hypnopompic: There may be particularly vivid auditory or visual illusions or hallucinations immediately falling asleep (hypnagogic) or, less commonly, immediately after waking up (hypnopompic). They are difficult to distinguish from an intense dream experience and somewhat resemble the vivid dreams that are normal during REM sleep. Hypnagogic hallucinations occur in about one-third of the patients, they are often in healthy young children and occasionally occur in healthy adults. Diagnostic polysomnography Multiple Sleep Latency Test A time gap of 10 years between the onset of symptoms to diagnosis is common. A cataplexy in medical history speaks in patients with ETS for a very narcolepsy. In patients with ETS a nocturnal polysomnography and a subsequent multiple sleep latency test (MSLT), the diagnosis of narcolepsy confirm if the findings include: Sleep REM episodes while possibilities of at least 2 of 5 naps during the day or a day plus a night in the previous Polysomnogramms. Average sleep latency (time to fall asleep) of ? 8 minutes No other diagnostic abnormalities in the nocturnal polysomnography narcolepsy Type 1 is diagnosed when patients also have cataplexy; Type 2 is diagnosed when the patients do not have cataplexy. The guard live test does not contribute to diagnosis, but it helps in monitoring the treatment efficiency. Other disorders that can cause chronic ETS are usually suspected based on history and physical examination; a brain imaging and laboratory tests of blood and urine can confirm the diagnosis. These disorders include lesions affecting the hypothalamus or upper brainstem, raised intracranial pressure and certain forms of encephalitis. Hypothyroidism, hyperglycemia, hypoglycemia, anemia, uremia, hypercapnia, hypercalcemia, liver failure and seizure disorders can also cause an ETS with or without hypersomnia. Acute systemic diseases such as influenza are usually accompanied by an acute, relatively short ETS and hypersomnia. The Kleine-Levin Syndrome, a rare disorder in adolescent boys, causes episodic hypersomnia and hyperphagia. The etiology is unclear, perhaps it is an autoimmune response to infection. Modafinil treatment methylphenidate and its derivatives, amphetamine derivatives or sodium oxybate Certain REM sleep-suppressing antidepressants Some patients with occasional episodes of sleep paralysis or hypnagogic hallucinations and hypnopompen, rare and partial cataplexy and lighter ETS do not need treatment. In other stimulants and antikataplektische drugs are used. In addition, patients should get enough sleep at night and make daytime naps short (<30 min) always at the same time of day (typically in the afternoon). Modafinil, a long-acting alertness promoting drug can help patients with mild to moderate EDS. The mechanism of action is unclear. Typically, modafinil is 100-200 mg p.o. given in the morning. The dose is increased to 400 mg as needed. Doses> 400 mg have not been shown to be more efficient. If the effect is not until well into the evening pause, a small second dose (z. B. 100 mg) can be used for 12 or 13 hours, although this dose may sometimes interfere with the night’s sleep. Alternatively, a short-acting preparation of methylphenidate can be added in the afternoon on a regular basis or as needed. Adverse effects of modafinil include nausea and headache, they can be mitigated by a low initial dose and a slower titration. Modafinil may reduce the effectiveness of oral contraceptives and has addictive potential, is however low. Rarely, severe skin rashes and Stevens-Johnson syndrome, have developed in patients taking modafinil. If serious reactions develop, the drug should be permanently discontinued. Armodafinil, the R-enantiomer of modafinil, having similar benefits and side effects; the dosage is 150 mg or 250 mg p.o. once in the morning. Methylphenidate or amphetamine derivatives are usually used instead of, or modafinil, when patients do not respond to modafinil. Methylphenidate 5 mg p.o. 2 times a day up to 20 mg p.o. 3 times daily is particularly favorable as immediately effective treatment, since the onset of action of modafinil is delayed. 5-20 mg of methamphetamine p.o. 2 times daily or dextroamphetamine 5 mg po 2 times a day up to 20 mg p.o. may be prescribed three times daily; All substances are available as prolonged-release formulations and can therefore be administered once daily in many patients. You can patients who are already taking modafinil, even if necessary to achieve, as their effect occurs rapidly and lasts relatively short. The adverse effects include agitation, hypertension, tachycardia, and mood changes (eg manic reactions.); the potential for abuse is high. Pemoline, which although has a lower dependence liability than amphetamines is not recommended, because it can be hepatotoxic and liver enzymes every 2 weeks must be controlled. Sodium can also be used to treat ETS and cataplexy. A dose of 2.25 g p.o. is taken at bedtime in bed, followed by the same dose of 2.5 to 4 hours later. The maximum dose is 9 g / night. Adverse effects include headache, nausea, dizziness, nasopharyngitis, somnolence, vomiting, urinary incontinence and sometimes sleepwalking. Sodium oxybate is BTM-charge and has abuse and dependency potential. It is contraindicated in patients with succinic semialdehyde dehydrogenase deficiency and should not be used in patients with untreated respiratory diseases. Tricyclic antidepressants (v. A. Clomipramine, imipramine, and protriptyline) and SSRI (z. B. venlafaxine, fluoxetine) are useful in the treatment of cataplexy, sleep paralysis and hypnagogic hallucinations and hypnopompen. Clomipramine 25-150 mg p.o. once daily in the morning, the most effective drug antikataplektische seems to be; However, it should only be given during the day to reduce nocturnal awakening. Conclusion narcolepsy may be caused by autoimmune destruction of hypocretin-containing neurons in the lateral hypothalamus in genetically predisposed patients. The cardinal symptoms are excessive daytime sleepiness (ETS), cataplexy, hypnagogic hallucinations and hypnopompic and sleep paralysis. The diagnosis is made using polysomnography and multiple sleep latency test. ETS usually speaks to modafinil of (sometimes with methylphenidate, an amphetamine derivative, is used) or on sodium oxybate. Tricyclic antidepressants and SSRIs may be useful in cataplexy, sleep paralysis and hypnagogic hallucinations and hypnopompen.