Narcolepsy or Gélineau disease is a rare disease, but it is very disabling for the people concerned.
Narcolepsy results in a sudden and irresistible urge to sleep that cannot be controlled. These sleeping bouts occur at any time of the day, whatever the circumstances. The narcoleptic feels an extreme drowsiness, it is then impossible for him to remain awake.
Men are more likely to be affected by this sleep disorder than women. On the other hand, there is a peak in the occurrence of the disease around adolescence, Child to young adult. Most often, narcolepsy occurs around 20 years.
There are some family forms, but most often the disease is sporadic. Most people with narcolepsy do not have a specific antecedent.
The symptoms of narcolepsy can be disturbing at first, even dangerous: a person with this disease who do not yet know and do not know how to handle his “sleep attacks” may risk having accidents because of An irrepressible somnolence, or even a sudden loss of vigilance.
The causes of narcolepsy are still poorly understood.
Narcolepsy is due to the destruction of some very specific neurons located at the base of the brain. The neurons that make the neurotransmitter called hypocretin are partially or totally destroyed. The disease only reaches these neurons, it does not touch other parts of the body. However, hypocretin plays a very important role in the maintenance of awakening and in the structuring of sleep.
The cause of the destruction of these neurons is not yet known. It is highly likely that it is an autoimmune mechanism, that is, an inadequate response of the subject’s immune system that will erroneously attack elements of his own organism.
Indeed, the mutation of a gene involved in the immune system has been found in people suffering from narcolepsy. Moreover, a scientific study assumes that the decrease in hypocretin-producing neurons is caused by the increase of hormonal cells producing histamine (a key molecule of the immune system).
For the diagnosis of narcolepsy, hypocretin may be found in the cerebrospinal fluid (liquid that surrounds the central nervous system). The physician may possibly perform a lumbar puncture to dose this substance. The doctor specialist in this kind of disorder is a neurologist, and more specifically a sleep specialist.
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The two main symptoms are diurnal somnolence and cataplexy attacks.
Narcoleptics find it difficult to stay awake during the day. He is a victim of an awakening disorder, a sleepiness. This usually results in irrepressible cravings to sleep on different occasions during the day. There is a possibility of transient recovery through a nap, but somnolence usually returns a few hours later. Narcoleptics may also have cataplexy attacks during the day.
Cataplexy is a significant loss or decline in muscle tone without loss of consciousness. Cataplexy attacks occur in full vigil, usually during a positive emotion such as laughter, surprise, sexual intercourse, etc.
There is an abrupt loss of muscle tone, but the patient does not fall asleep, he is awake and perfectly conscious. Cataplexy may be partial (only the arm or head will be affected) or generalized (in this case, if the patient is standing, he may fall). Then there are other symptoms related to narcolepsy that translate a disorganization of sleep itself:
Of hallucinations hypnagogic, that is to say hallucinatory phenomena falling asleep or awakening
Sleep paralysis, which are moments of inhibition of muscle tone to falling asleep or awakening
There may also be somnambulism-like phenomena, nightmares, etc.
The diagnosis of narcolepsy is suspected as soon as the patient is questioned by the doctor, especially if the patient has cataplexy attacks. Indeed, cataplexy is specific to narcolepsy.
To confirm the diagnosis, the following tests are performed:
A sleep record (polysomnography) that is used to eliminate another cause of drowsiness
An iterative test of latency of sleep, it is a test which consists of recording the sleep of the subject to 5 times in the day, every 2 hours with a controlled environment and instructions well standardized. If the subject falls asleep quickly and finds himself very quickly in REM sleep (a stage that normally occurs late in sleep), he is diagnosed with narcolepsy.
A lumbar puncture is performed for doubtful cases. This allows an assay of hypocretin in the cerebrospinal fluid. If the rate is collapsed, the person is narcoleptic.
Narcolepsy is a chronic disease, which cannot be cured. The causes of this sleep disorder are not known today, as they are still poorly understood.
Fortunately, there are drug treatments that are purely symptomatic. Against drowsiness, medicines can be used to stimulate certain regions of the nervous system. These medicines should be prescribed taking into account possible interactions with other treatments, such as birth control pills and neuroleptics. Waking medicines can sometimes cause symptoms of anxiety.
Against cataplexy attacks, some antidepressants are prescribed at lower doses than for depression. There are also other arousal medications that can be obtained through temporary use (ATU), when other therapies fail. These treatments can be quite effective and lessen the desire to sleep during the day.
People with narcolepsy should have a healthy and regular lifestyle and sleep pattern (always lie at the same time every day, avoid any exciting substances before going to bed – nicotine, alcohol, caffeine, avoid watching television bed).