Narcolepsy is a neurological disorder in which people are attacked by sleeping attacks in broad daylight. They often nod in the mouth while talking, eating, at the desk or in the subway, suddenly collapsing. Especially in traffic or when operating machinery can be very dangerous situations arise. Narcolepsy patients can not help with these sleep seizures. Read everything here about the “Schlafucht”!
Narcolepsy: description
Narcolepsy is also popularly referred to as “somnolence” or “sloth addiction”. It can be classified in the group of hypersomnia.
Physicians distinguish the following forms:
- Narcolepsy with cataplexy (muscle relaxation) = classic narcolepsy
- Narcolepsy without cataplexy
- Secondary narcolepsy (due to hypothalamic or brainstem injury, e.g., due to decreased blood flow, tumor, or neurosarcoidosis)
Narcolepsy is a rare neurological disorder that is not curable. It accompanies people for a lifetime, but is not life threatening. Experts estimate the number of people with narcolepsy in Germany to be around 40,000 – although the number of unreported cases should be very high. One reason is that “narcolepsy” often takes several years to arrive at the correct diagnosis.
Narcolepsy: symptoms
In people with hypersomnia, that part of the brain that controls the sleep-wake cycle is disturbed. Typical of narcolepsy is that patients fall asleep suddenly and in the most impossible situation, for example in the middle of a conversation, while eating or in the office chair. In many narcolepsy patients, cataplexy is added to sleep attacks. Here, control of muscle tone is lost and muscles suddenly lose their sleep – patients suddenly collapse, but are fully conscious.
The following symptoms are typical of narcolepsy and must have occurred for more than six months to help diagnose narcolepsy. Symptoms of hypersomnia are:
Extreme daytime sleepiness and heavy sleep urge: This is the main symptom of narcolepsy and affects all those affected. Certain situations that even blind people’s eyes trigger an irresistible need for sleep in narcoleptics. An example is twilight: events in darkened rooms (lectures, conferences, cinema, etc.) are torture or are quite impossible. Particularly critical are boring, monotonous, uniform situations. But sleepiness also makes passivity, such as sitting or listening for a long time. Although you can wake up the narcolepsy. But if he has not slept enough, he immediately falls back to sleep.
When narcoleptics become overwhelmed by extreme drowsiness, their gait becomes unsteady (they waver or stagger), the pronunciation becomes indistinct (they part with) and they get a glassy and pensive look. For outsiders, it often seems that the narcolepsy is alcoholized. Therefore, the environment often has little understanding for people with narcolepsy.
cataplexy: In 80 to 90 percent of those affected, narcolepsy is accompanied by a cataplexy – the second main symptom. The muscles suddenly go to sleep because control over muscle tension (muscle tone) is lost. Although the consciousness is not clouded, the patient can not communicate with his fellow man. Most patients remember everything that happened during the cataplexy. Typical triggers of cataplexy are violent emotions, such as laughter, joy, surprise, fright or fear.
Cataplexy usually lasts only a few seconds. If it affects the entire musculature, the narcoleptic collapses or even falls. In contrast, lighter cataplexes often only affect individual muscle groups. The narcoleptic drops objects because the muscles of the hands or arms are missing. He can also “washed out” and speak indistinctly when the facial and jaw muscles are affected.
Disturbed night sleep: This symptom affects about 50 percent of narcoleptics. It usually does not appear right at the beginning of narcolepsy, but develops gradually during the course of the disease. Patients often wake up at night or lie awake in bed for a long time. In addition, the sleep is relatively easy and not very relaxing – in the morning, the patients are usually tired. Some patients with narcolepsy feel a urge to move in bed (motor agitation) and suffer from nightmares. Some sleepwalk or talk in their sleep. This is often a challenge for the partners as well.
Sleep paralysis occur in about 50 percent of narcolepsy patients. Here patients can not move or speak during the transition from awake state to sleeping or vice versa. Sleep paralyzes take seconds to several minutes and trigger extreme fears. They usually end spontaneously, but relatives can also terminate them by loud speech or touch.
hallucinations have up to 50 percent of the patients. Such hallucinations can also occur during the transition from awake state to sleep (hypnagogic hallucinations) or vice versa when waking up (hypnopompe hallucinations). Mostly they last a few minutes. The contents of the hallucinations can be quite different, but are often quite realistic.
Automatic behavior This can happen if the person is extremely tired and tries to resist the sleep pressure. He simply carries on actions that have been started – this can also lead to dangerous situations. For example, a narcolepsist crosses a street at a red light and does not pay attention to the traffic. In the state of automatic behavior, he no longer registers his environment and dangerous situations. Hazardous situations are also present in the household, for example when people with narcolepsy handle knives or climb a ladder. Injuries are not uncommon. The narcoleptic usually can not remember the time of automated behavior.
In addition to these symptoms, other side effects of narcolepsy may occur. These include, for example, headaches or migraines, memory and concentration disorders, accidents, depression, potency disorders and personality changes.
Narcolepsy: causes and risk factors
The causes of narcolepsy are still unclear (idiopathic hypersomnia). Physicians discuss that narcolepsy is an autoimmune disease – here, the immune system is directed against the body’s own structures. Also infectious triggers such as influenza viruses or streptococci could play a role.
Many patients with narcolepsy have lower levels of hypocretin / orexin in the cerebrospinal fluid. These are neuropeptide hormones that are formed in the midbrain (hypothalamus) and influence, for example, the eating behavior as well as the sleep rhythm.
In addition, the genetic test for HLA DRB1 * 1501 and HLA DQB1 * 0602 is positive for almost all narcolepsics (98 percent). HLA stands for the Human Leukocyte Antigen System (HLA System). It is a group of human genes that are extremely important to the functioning of the immune system. For the diagnosis of narcolepsy, however, these loci (alleles) are not specific, since they are also detectable by 25 to 35 percent of the normal population. Therefore, this genetic test is not enough for a single diagnosis.
Narcolepsy: examinations and diagnosis
Narcolepsy belongs in the hands of specialists. It should be diagnosed in a sleep laboratory or by an experienced neurologist or sleep specialist (somnoloologist). First, the doctor will ask you about your medical history (anamnesis). It is also important which complaints they have. In the foreground are the symptoms of daytime sleepiness and cataplexy. Here also close persons and family members can give good information.
Be used Sleep questionnaires and sleep diariesImportant are Epworth Sleepiness Score (ESS), Stanford Narcolepsy Questionnaire, Ullanlinna Narolepsy Score (US) and the Swiss Narcolepsy Score (SNS). Subjectively, they record how good the quality of sleep is and what sleep problems there are.
polysomnography: At night, a wide variety of bodily functions are continuously monitored. As a rule, polysomnography is performed in a sleep laboratory. To measure the biosignals electrodes are glued to the skin. The brain curves, muscle activity, heart function and eye movements during sleep are recorded. With the help of this data, an individual sleep profile can be created and any sleep disorders diagnosed.
Multiple Sleep Latency Test (MSLT): The patient should hold a short sleep of approximately 20 minutes four to five times every two hours. This test tests the tendency to sleep and the premature appearance of REM sleep (SOREM). For narcolepsy, it is typical that sleep latency is very short. Many patients show two or more SOREM phases in the MSLT.
In some patients, hypocretin / orexin levels in cerebrospinal fluid are also determined. In addition, genetic HLA typing can also be performed (HLA DRB1 * 1501 and HLA DQB1 * 0602 by oral swab or blood sampling). If there is a suspicion of secondary narcolepsy, images of the brain are taken.
Narcolepsy: treatment
Narcolepsy is not curable but treatable. The symptoms can be improved to a certain extent. For this purpose, certain medications are used.
Daytime sleepiness can be treated with stimulants. The drugs of choice are modafinil or sodium oxybate (gamma-hydroxybutyric acid). The ADHD drug methylphenidate may also help some patients.
Second-line medications include ephedrine, dextroamphetamine, and sometimes MAO inhibitors, which are used to treat depression. These are not approved for narcolepsy (“off-label use”).
The drugs usually have to be taken permanently, some are subject to the Narcotics Act (BtmG). It is important that patients are regularly checked by a doctor.
Cataplexia, sleep paralysis, and hallucinations are treated with sodium oxybate or antidepressants.
Also non-drug therapies can help narcolepsy patients.
- Important is a regular sleep / wake rhythm. It’s best to get up at the same time every day, and always sleep at the same time.
- Take one to two quick naps during the day when you overpower the need for sleep.
- Avoid situation with increased risk of injury or accident. For example, cook at a time when you are most likely to stay awake. A timetable helps here.
- It is also important to inform your social environment about narcolepsy. If friends and family know about it, there will be less misunderstandings and conflicts. Because many interpret a sleepiness rather than laziness, depression or personal inability.
Narcolepsy is a lifelong companion. Therefore, learn strategies for better acceptance and handling narcolepsy.
Disease course and prognosis
In principle, narcolepsy can occur for the first time at any age. However, researchers have identified two frequency peaks: in the second decade between the 10th and 20th and in the fourth decade between the 30th and 40th year of life. In about 20 percent of those affected, the disease even appears in the first ten years of life.
Narcolepsy can begin creepingly but also suddenly with all symptoms. The severity of the disease can vary considerably from person to person. The quality of life of narcolepsy patients is often very limited. Many can not exercise a profession because of hypersomnia. However, the more experience those affected with the narcolepsy the better they can handle it.