narcolepsy
Introduction
Introduction to narcolepsy Narcolepsy is a disease characterized by irresistible short-term sleep. It is a syndrome with unclear etiology. It is characterized by abnormal sleep tendency, including excessive daytime sleepiness, nighttime restlessness and pathology. REM sleep. More than children or adolescent onset, male and female incidence rates are similar, some patients may have a history of encephalitis or craniocerebral trauma. The pathogenesis of this disease is not clear, and may be related to the decreased function of the ascending activation system of the brainstem network or the reticular nucleus function of the caudal brain. Most patients are accompanied by other symptoms such as cataplexy, sleep paralysis, sleep hallucinations, etc. Quadrilateral narcolepsy. basic knowledge The proportion of illness: 0.003% Susceptible people: more common in children or adolescents Mode of infection: non-infectious Complications: neurasthenia, autism
Cause
Cause of narcolepsy
Functional lesions (25%):
It is related to the decreased function of the ascending activation system of the brainstem network or the hyperfunction of the reticular nucleus of the caudal cerebral ventricle. It is also considered to be an autosomal dominant hereditary disease, although the incidence rate is higher in some ethnic groups such as Japanese. Case reports are reported in various ethnic groups and around the world. Their genetic characteristics are closely related to human HLA minimal tissue-related genes (DR and DQ loci) in various groups. The pathogenesis is a regulatory disorder of REM sleep, followed by NREM. The control of REM sleep after the sleep cycle is lost, and the tendency to enter REM sleep increases both at the beginning of sleep and during the awake period.
Trauma factors (25%):
Sandyk (1995) believes that the dysfunction of the pineal gland and its secretion of melatonin may be related to the occurrence of this disease, and some people believe that it may be related to brain trauma, viral infection, immune dysfunction, brain tumor, multiple sclerosis and other diseases. The occurrence of normal REM sleep depends on the triggering of the serotonin system of the brainstem internuclear nucleus system on other transmitter systems, and this triggered rhythm disorder can occur in this disease.
Genetic factors (20%):
Yoss reported in 1960 that there were 12 (7 males and 5 females) of the 3 generations of a family. Animal experiments showed that when the dogs were both narcolepsy, all the litters were affected. And these dogs are unaffected by puppies bred by unrelated or distantly related dogs. The pattern of transmission may be autosomal recessive, but at the same time it is found that not all types of dogs show seizures. The genetic transmission of sleeping sickness may have different causes.
Environmental factors (15%):
Honda et al reported that 100% of narcolepsy patients in Japan showed positive human leukocyte antigen HLA-DR2, and European and American scholars also confirmed that most Caucasian narcolepsy patients were HLA-DR2, DQWl, DW2 positive. However, Guilleminault et al believe that the transmission of narcolepsy is multifactorial, and environmental factors also play an important role.
Prevention
Narcolepsy prevention
In order to avoid accidents, it is necessary to take a series of prevention and control measures in daily life to reduce seizures. Patients should consciously arrange their life with rich and colorful activities, participate in cultural and sports activities, do some interesting work, and try to avoid monotony. Activities to drink tea or coffee during the day to increase brain excitability.
Maintain optimism, build confidence to overcome disease, avoid depression, sadness, but not too excited, because the loss of excitement can induce a trip, it is best not to travel alone, do not engage in high altitude, underwater operations, but can not engage Drive vehicles, manage a variety of signals and other responsible work to avoid accidents.
Complication
Complications of narcolepsy Complications, neurasthenia, autism
In addition to sleep episodes, some patients complain of fatigue and sleepiness, are in a state of low alertness all day, poor memory, low work efficiency, affecting physical health and work efficiency, and declining quality of life. Long-term lack of sleep can lead to irritability, depression, autonomic dysfunction. Digestive dysfunction, etc., as well as reduced immune function, can also be complicated by hypertension, arrhythmia, cerebrovascular disease, diabetes, kidney disease and so on.
Symptom
Symptoms of narcolepsy common symptoms respiratory irregular anxiety eyelid tremor palpitations fatigue sleep apnea hallucinations powerless sleepiness sleepiness
The onset age is generally from childhood to early adulthood, but it is generally not detected before the adolescence, with 10 to 20 years old as the most, the same incidence rate for men and women, a small number of patients have a history of encephalitis or brain injury, but with this The relationship has not been confirmed.
The first symptoms were mostly in puberty, daytime episodes of sleepiness, squatting and sleep paralysis, and hallucinations before going to sleep were the four main symptoms of the disease.
1. Paroxysmal lethargy : When the patient wakes up, he is usually in a state of frequent and fluctuating alertness, and is more obvious in the afternoon. When the degree of lethargy increases, short sleep occurs. Most patients feel drowsiness before the attack, only a few The patient suddenly falls into sleep from a relative awake state. A monotonous environment, such as during reading and listening, is easy to induce. Typical cases can occur in various activities, such as eating, speaking, operating a machine, driving a vehicle, etc., each episode lasts. From a few seconds to a few hours, most of them last for tens of minutes. Most of the sleep is not deep, and it is easy to wake up. After waking up, you usually feel temporarily awake, and you can have several episodes a day.
2. cataplexy: cataplexy is a transient, fully reversible onset of motor depression, manifested as a sudden onset of voluntary muscle strength loss or reduction, can occur in 50% to 70% of patients, often Induced by laughter, anger, emotional, nervous, fatigue or satiety.
Typical hair as the jaw is slack, the head falls forward, the arms fall to one side and the knees open, the complete loss of muscle strength can lead to trauma and even fracture, but the degree of attack is not all so serious, but can only be expressed as a kind A short-lived, partial or systemic sense of powerlessness, the patient may complain of blurred vision (accumulation of the diaphragm), interruption of language (accumulation of the diaphragm), irregular breathing (abdominal or diaphragmatic involvement), slight bending of the knee or sudden holding Falling (upper and lower limb muscle involvement) may be accompanied by involuntary movements, such as head and eyelid tremors, facial paralysis, and may also be accompanied by autonomic symptoms such as palpitations, paleness, and these atypical episodes are often ignored by doctors, and special attention should be paid.
Symptoms often disappear after the emotions return to normal or the body is touched. The collapse is accompanied by the suppression of tendon reflexes, but it is always conscious, the frequency of seizures can occur once every few days or months, and there are also 4 to 5 episodes per day. One episode lasts for a few seconds to 30 minutes (Zancone, 1973).
The relationship between tripping and other symptoms, 50% at the same time, 25% 1 to 5 years after the onset of sleepiness, 15% occurred 10 years after the onset of sleepiness, and it is extremely rare to happen before the onset of sleep. .
3. Sleeping snoring : About 20% to 30% of narcolepsy patients may also appear alone. After waking up or falling asleep, whether it is nap or nighttime sleep, accidental paralysis of the extremities occurs, and the patient's consciousness is clear. , but can not speak or move, often accompanied by anxiety and hallucinations, more than a few seconds to a few minutes to ease, occasionally up to several hours, others can often stop the attack when they touch the patient's body or speak to them, but if they do not act after the relief May relapse.
4. Illusion when falling asleep : It can occur in about 30% of patients, often with sleeping snoring, mainly in the sleepiness period, hallucinations, sight, auditory hallucinations, may also be touch, pain and other physical hallucinations Most of the content is vivid, mostly for the daily experience of patients.
Examine
Examination of narcolepsy
1. Multiple sleep latency test (MSLT) is a test to assess the degree of lethargy. Subjects in a comfortable, quiet, dimly lit room, let them sleep for 20 minutes every 2 hours. 6 times, usually at 10, 12, 14, 16 and 18, while monitoring with PSG, recording the time from the light off to the start of sleep (according to the EEG), the presence or absence of the REM period Time, it is generally believed that the sleep latency of normal people should be more than 10min, such as the average within 8min is pathological; REM period in nap and within 15min of sleep (normal people appear around 90min after the start of sleep) It is considered to be a sleep that starts in the REM phase. If there are more than 2 sleeps in the REM phase in the MSLT, and the overnight polysomnography excludes other diseases that can cause drowsiness, it can be diagnosed as Narcolepsy.
2. Polysomnography (PSG) examination: SAS can be distinguished from narcolepsy, SAS has typical respiratory events, and a few narcolepsy patients also have sleep apnea (Zancone, 1973), with OSAS Identification should pay attention to the presence or absence of pathological REM sleep.
3. PSG examination with anterior tibialis electromyography: PLMS can be diagnosed, characterized by periodic rupture of the tibialis anterior muscle for 0.5 to 5 s with an interval of 15 to 60 s.
Based on the medical history and key neurological examinations, other necessary optional auxiliary examination items include:
1. CT and MRI examinations.
2. Blood routine, blood electrolytes, blood sugar, urea nitrogen.
3. ECG, abdominal B-ultrasound, chest, thyroid function test.
Diagnosis
Diagnosis of narcolepsy
It is generally believed that if there is a typical history of sleepiness and cataplexy, it can be diagnosed. Some people think that it can be diagnosed only by the typical history of sleepiness. With the continuous development of sleep medicine, people realize that many diseases can cause daytime sleepiness. Attacks such as sleep apnea syndrome (SAS) and periodic diastolic syndrome (PLMS) should be differentiated.
Must be identified with the following diseases
First, seizures of seizures, more common in children or adolescents, with consciousness disorders as the main symptoms, often sudden loss of consciousness, eye-opening, standing still, do not fall; or suddenly terminate the ongoing action, such as holding things, Can not continue the original action, lasting a few seconds, the EEG can have a 3Hz spine-slow integrated wave,
Second, fainting, due to short-term transient loss of consciousness caused by cerebral blood circulation disorders, many dizziness, weakness, nausea, black spots and other short-term aura, followed by loss of consciousness and fainting, often accompanied by autonomic symptoms, such as It is pale, with cold sweat, weak pulse, lower blood pressure, and lasts for a few minutes.
Third, Kleine-Levin syndrome, also known as periodic sleepiness and pathological hunger syndrome, usually seen in male adolescents, with periodic episodes (intervals or weeks), each lasting 3 to 10 days, showing drowsiness, Bulimia and behavioral abnormalities, etiology and pathogenesis are still unclear, may be caused by dysfunction of the diencephalon, especially the hypothalamus or focal encephalitis.
