What Is Kleine-Levin Syndrome?

Kleine-Levin syndrome, also known as Sleeping Beauty Syndrome, Periodic Drowsiness, and Pathological Hunger Syndrome, is a rare neurological disorder. It is usually seen in male adolescents, with periodic attacks (intervals of several weeks or months), each lasting 3 to 10 days, manifested as lethargy, bulimia and abnormal behavior. The etiology and pathogenesis are unknown, and may be caused by abnormalities in the midbrain, especially in the lower thalamus, or focal encephalitis.

Klein Levine Syndrome

Cause analysis of Klein Levine syndrome

Klein Levine's syndrome, the cause of which is still unclear, and some researchers suspect autoimmune, hypothalamic dysfunction, which is thought to be related to abnormal functional areas of the brain that control sleep and appetite. Causes narcolepsy-the cause of dysphagia. It is also caused by tumors, inflammation or trauma in the posterior hypothalamus. Clinical manifestations of Klein Levine Syndrome.

Features of Kline Levine Syndrome

Klein Levine Syndrome, also known as Sleeping Beauty Syndrome, was originally called Kleine-Levine Syndrome, or KLS for short. This is a disease that causes repeated sleep and behavior changes. No more than 1,000 people suffer from this condition worldwide. Each time, the patient can live a normal life for weeks or months, during which time his sleep patterns and energy levels are normal. But then they need to be careful when it's their turn to sleep. They can sleep for days or weeks at a time.

Symptoms often fall asleep or lethargy during the day and most of the night, and only wake up when you go to the bathroom and eat. Even after waking up, the person's behavior will change, and his mind is still shy or like a child. They also experience unconsciousness, disorientation, general burnout or indifferent emotions. Patients often fail to go to school or work due to illness. Most people lie in bed all day, tired and unable to communicate with others. For some patients, sometimes there are no symptoms for weeks, months or even years, and then recurrence due to some small warning signs. For those affected, their health reports are usually not noticeably abnormal, but the disease will disappear naturally after about 8 to 12 years. ,

Most patients will describe that everything is out of focus, and they are particularly sensitive to sound and light. The status of overeating can be observed in some cases. At the time of onset, some men will have excessive sexual impulses, and some women will Melancholy. The incidence of the disease in adolescents is one in one million, and approximately 70% of patients are men.

Clinical manifestations of Klein Levine syndrome

Lethargy

Drowsiness is a major feature of Klein Levine's syndrome,

Paroxysmal lethargy-strong eating disorder is named after its special manifestations of "sleepiness" and "strong eating". Patients have uncontrollable epileptic sleep, which lasts for several hours to several days each time, regardless of the location and occasion during the attack, and cannot be awakened by various stimuli. Do not eat or drink during the attack. It is several times or even 10 times the usual diet, and it is very easy to starve.

Sleep episodes repeated several times a day occur not only during monotonous, static work or after a full meal, but also during work with high patient activity. Repeated attacks over several months to several years, these patients are not accompanied by endocrine changes, and no symptoms of autonomic dysfunction such as palpitation and sweating. The duration of each attack will vary depending on the body position. If the patient is in an uncomfortable position, it may occur for several minutes, while lying down may reach 1 hour. The patient tried to stay awake during the onset of sleep, but fell asleep in a short time (within 1 to 2 minutes). Symptoms diminish over time but do not completely subside.

Narcolepsy

The clinical manifestations of narcolepsy mainly include irresistible sleep episodes during the day, sleep paralysis, hallucinations before falling asleep, sudden falls, and abnormal sleep at night. The first symptoms usually come close to puberty, the peak age is between 15 and 25 years old, the second smaller peak is between 35 and 45 years old, and women are close to menopause. Daytime drowsiness and irresistible sleep episodes are often the first symptoms that can occur alone or with one or more other symptoms.

Severe narcolepsy can cause various comorbidities, with the exception of sleep episodes. Patients may experience abnormal sleepiness and remain in a state of low alert all day, leading to traffic accidents and unconsciousness related to operating machines. Memory loss and slow response Decreased work capacity and forced early retirement, or dismissal, impotence, or even inability to walk or murmur.

Sleep paralysis

Sleep paralysis and pre-sleep hallucinations do not occur in all patients, and they only occur briefly. Nocturnal sleep abnormalities rarely occur during the first stage of sleep. Sleep paralysis is a terrible scene, which often occurs during the process of falling asleep or awake. Suddenly, the patient cannot move, can't talk, and can't even breathe deeply, often accompanied by hallucinations. But I can fully recall it afterwards. In many episodes of sleep paralysis, especially in the first episode, patients often experience extreme anxiety and fear of death. Due to the experience of many times, the patient also knew that this attack was short-lived, the consequences were benign, the duration would not exceed 10 minutes, and it could be relieved automatically.

Pre-sleep illusion

Hallucinations often cause hallucinations before falling asleep, and the shape and size of colored rings or objects vary in front of eyes. Or the color of the object changes from black to white, more often in the form of color. Phantom auditory is also very common. Phantom auditory can be sound or music. Another interesting type of hallucinations is body sensation and feelings (such as traction, friction, and light perception), and changes in the position of body parts (such as changes in the position of upper and lower limbs) have floating or external sensations.

Burst

Sudden falls are sudden, reversible hypotonia or loss. They often occur with abnormal sleep, but they can also appear after 20 years. Sometimes sudden falls can occur before abnormal sleep. This is rare. In most cases, It is induced by emotional changes, such as laughter, anger, surprise, and sudden tension. It can be the involvement of some muscles, or the loss or loss of all voluntary muscle tone.

Typical attacks are sagging jaw, head down, arms down, and knees abducted. Involvement of the diaphragmatic muscle causes intermittent weakness and speech disruption. If only the lower jaw and the articulation system are involved, the patient may experience large chewing movements or stuttering. If the upper limbs are involved, the patient may show awkward movements, and the cup or plate may suddenly fall or spill liquid when laughing or surprised.

In a catastrophic attack, a complete loss of muscle tone can cause serious trauma, such as skull and other fractures. Sudden attacks are very common, but are not noticed. The attack may be just a slight flexion of the knee, and the patient will stop or stand against the wall when they detect this sudden and short attack. With jaw sagging, the symptoms are slightly more pronounced when the head is lowered forward.

The frequency of sudden onset may be only a few times in a lifetime, or it may be several times in a day. The severity and consequences of cataplexy attacks vary widely, from absolute weakness of all voluntary muscles to limited involvement of certain muscle groups, or a mild sense of weakness. There are no records of electromyograms for sudden collapse, transient muscle weakness, and the duration of each sudden onset varies from a few seconds to more than 30 minutes. Seizures may be triggered by mood, tension, fatigue, or a full meal. Laughing or anger is a common trigger, but sometimes it is while listening to music, watching a movie or reading a book. Sudden fallouts sometimes occur when recalling beautiful scenes without the involvement of emotional factors or strenuous exercise.

Other symptoms

Most patients are obese. Mainly due to the reduction in sleep consumption, and a lot of eating after waking, nutrient storage. This type of obesity is also manifested as uniform obesity throughout the body. Although the extraocular muscles were not affected, the patient had weak eyelids and blurred vision. At the time of the attack, language was affected, abdominal muscle strength was reduced and breathing was irregular, and diaphragmatic muscle activity appeared to have a short pause similar to the rapid eye movement sleep period, with no long pause.

Klein Levin Syndrome Prevention

Doctors can only combine stimulants and sleep medications to prevent patients from falling asleep without warning and improve their sleep quality.

Major cases of Klein Levin syndrome

Klein Levin syndrome UK

Samuel Hilton

At the end of the 17th century, the Royal Society published a very sleepy man named Samuel Hilton. Originally on May 13, 1694, this sturdy and robust male "Sleeping Beauty" began to fall asleep and did not wake up until a week later. On April 9, 1695, Hilton fell into a long and deep sleep. People asked the doctor to give him bloodletting treatment, use fire to burn and apply various stimuli, and it was completely useless. The big sleep lasted 17 weeks, and Hilton did not wake up until August 7.

Bethany-Ross Goodill

In November 2010, 18-year-old British girl Bethany Rose Goodall suffered from the rare "Klein-Levin syndrome" and slept intermittently for a year, the longest of which was "sleeping" 6 months. During this time, her family and boyfriend have been standing by her, taking care of her 24 hours a day. From then on, I slept more than 22 hours a day. This really frightened Besani's family.

Louisa Ball

Luisa Ball, from the southwestern city of Worthing, suffers from Klein Levine Syndrome, and she often wakes up for several days without waking up, sometimes sleeping for two weeks at a time. She even fell asleep at any time when she was talking to someone. In fact, Louisa was not born to be a "sleeping beauty". According to her mother, she developed this drowsiness after suffering from the flu in 2008. The doctor didn't know what went wrong at first, but said that it might be caused by hormones.

In March 2008, Louisa came to a hospital in south London before the doctor finally confirmed her diagnosis. The doctor prescribed her some medicine, but it didn't seem to help, and she went to sleep longer and longer. In the summer, Louisa's family went on a tour. On the plane home, Louisa couldn't sleep, and the family had to wait for all the passengers to leave before taking her off the plane. After going home, she went to sleep for another 7 days. Another family planned a 7-day camping holiday trip, and as a result, Louisa was sleeping during the entire process and missed all activities. When she woke up, she was very angry about it, but everyone could do nothing about it.

By November 2008, Louisa had been able to sleep for 10 days at a time. After she usually slept for 22 hours, her parents called her up to eat and go to the bathroom, and then she would return to bed and sleep. Because she slept, Louisa missed school exams and dance lessons.

Now the doctor has changed another medication for Louisa, hoping to reduce her sleep time. Luisa's father said that this method looks promising. She has not had symptoms of lethargy for 7 weeks, and hopes that she can return to normal as soon as possible.

Lily Clark

British college girl Lily Clark suffers from Klein-Leven syndrome and is a live-action version of "Sleeping Beauty". Clark is 21 years old and lives in Hasselmere, Surrey. Her mother Adele recalled. She often doesn't wake up and can sleep for two full months at the worst, so she misses important exams such as exams, Christmas, New Year and even her 18th birthday party.

In November 2007, Clark first became ill. On the day of the attack, the whole family went skating and eating together. Lily felt uncomfortable on the head, so she fell asleep on the chair. The family couldn't wake her up, and finally had to lift her from the restaurant go. For the next 25 days, Clark slept heavily and woke up just to eat and drink. Before Christmas that year, Clark finally woke up, but had no impression of his illness. But just a month later, she fell into a state of sleep again for up to 3 weeks and missed the mock exam. For the third time, Clark slept for two weeks and spent his 18th birthday in sleep.

David Nat of the Westminster Hospital in the United Kingdom confirmed that Clark did suffer from Klein-Levin syndrome. Because of the illness, Clark was often lethargic. He was sleeping for 23 hours a day, and the lights and noise would not wake her up. The only sober hour was enough for her to drink and eat. At this stage, she has to go to deep sleep every 7 months and sleep for a full two months at a time. Being awake and not waking up missed important life days like birthdays, school exams, Christmas, and New Years, and she often woke up and didn't know she had slept that long. After college, her grades were poor due to absences.

Klein Levine Syndrome China

In 2011, a woman in Guangdong has been asleep since junior high school, and she has not been awake for one month, two months for two months, and three years for twenty years. This "Sleeping Beauty" was a little vague while sleeping, and could walk in the bed confusedly, but she never refused to leave the house, and so far she has not spoken to her family. ^[1]