National Institute of Neurological Disorders and Stroke

What is narcolepsy?

Narcolepsy is a chronic neurological disorder that affects the brain’s ability to control sleep-wake cycles. People with narcolepsy may feel rested after waking, but then feel very sleepy throughout much of the day. Many individuals with narcolepsy also experience uneven and interrupted sleep that can involve waking up frequently during the night.

Narcolepsy can greatly affect daily activities. People may unwillingly fall asleep even if they are in the middle of an activity like driving, eating, or talking. Other symptoms may include sudden muscle weakness while awake that makes a person go limp or unable to move (cataplexy), vivid dream-like images or hallucinations, and total paralysis just before falling asleep or just after waking up (sleep paralysis).

In a normal sleep cycle, a person enters rapid eye movement (REM) sleep after about 60 to 90 minutes. Dreams occur during REM sleep, and the brain keeps muscles limp during this sleep stage, which prevents people from acting out their dreams. People with narcolepsy frequently enter REM sleep rapidly, within 15 minutes of falling asleep. Also, the muscle weakness or dream activity of REM sleep can occur during wakefulness or be absent during sleep. This helps explain some symptoms of narcolepsy.

If left undiagnosed or untreated, narcolepsy can interfere with psychological, social, and cognitive function and development and can inhibit academic, work, and social activities.

Narcolepsy is a lifelong problem, but it does not usually worsen as the person ages. Symptoms can partially improve over time, but they will never disappear completely. The most typical symptoms are:

• Excessive daytime sleepiness (EDS)—All individuals with narcolepsy have EDS, and it is often the most obvious symptom. EDS is characterized by persistent sleepiness, regardless of how much sleep an individual gets at night. However, sleepiness in narcolepsy is more like a “sleep attack,” where an overwhelming sense of sleepiness comes on quickly. In between sleep attacks, individuals have normal levels of alertness, particularly if doing activities that keep their attention.
• Cataplexy—This sudden loss of muscle tone while a person is awake leads to weakness and a loss of voluntary muscle control. It is often triggered by sudden, strong emotions such as laughter, fear, anger, stress, or excitement. The symptoms of cataplexy may appear weeks or even years after the onset of EDS. Some people may only have one or two attacks in a lifetime, while others may experience many attacks a day. In about 10 percent of cases of narcolepsy, cataplexy is the first symptom to appear and can be misdiagnosed as a seizure disorder. Attacks may be mild and involve only a momentary sense of minor weakness in a limited number of muscles, such as a slight drooping of the eyelids. The most severe attacks result in a total body collapse during which individuals are unable to move, speak, or keep their eyes open. But even during the most severe episodes, people remain fully conscious, a characteristic that distinguishes cataplexy from fainting or seizure disorders. The loss of muscle tone during cataplexy resembles paralysis of muscle activity that naturally occurs during REM sleep. Episodes last a few minutes at most and resolve almost instantly on their own. While scary, the episodes are not dangerous as long as the individual finds a safe place in which to collapse.
• Sleep paralysis—The temporary inability to move or speak while falling asleep or waking up usually lasts only a few seconds or minutes and is similar to REM-induced inhibitions of voluntary muscle activity. Sleep paralysis resembles cataplexy except it occurs at the edges of sleep. As with cataplexy, people remain fully conscious. Even when severe, cataplexy and sleep paralysis do not result in permanent dysfunction—after episodes end, people rapidly recover their full capacity to move and speak.
• Hallucinations—Very vivid and sometimes frightening images can accompany sleep paralysis and usually occur when people are falling asleep or waking up. Most often the content is primarily visual, but any of the other senses can be involved.

Additional symptoms include:

• Fragmented sleep and insomnia—While individuals with narcolepsy are very sleepy during the day, they usually also experience difficulties staying asleep at night. Sleep may be disrupted by insomnia, vivid dreaming, sleep apnea, acting out while dreaming, and periodic leg movements.
• Automatic behaviors—Individuals with narcolepsy may experience temporary sleep episodes that can be very brief, lasting no more than seconds at a time. A person falls asleep during an activity (e.g., eating, talking) and automatically continues the activity for a few seconds or minutes without conscious awareness of what they are doing. This happens most often while people are engaged in habitual activities such as typing or driving. They cannot recall their actions, and their performance is almost always impaired. Their handwriting may, for example, degenerate into an illegible scrawl, or they may store items in bizarre locations and then forget where they placed them. If an episode occurs while driving, individuals may get lost or have an accident. People tend to awaken from these episodes feeling refreshed, finding that their drowsiness and fatigue has temporarily subsided.

There are two major types of narcolepsy:

1. Type 1 narcolepsy (previously known as narcolepsy with cataplexy)—This diagnosis is based on the individual either having low levels of a brain hormone (hypocretin) or reporting cataplexy and having excessive daytime sleepiness on a special nap test.
2. Type 2 narcolepsy (previously known as narcolepsy without cataplexy)—People with this condition experience excessive daytime sleepiness but usually do not have muscle weakness triggered by emotions. They usually also have less severe symptoms and have normal levels of the brain hormone hypocretin.

A condition known as secondary narcolepsy can result from an injury to the hypothalamus, a region deep in the brain that helps regulate sleep. In addition to experiencing the typical symptoms of narcolepsy, individuals may also have severe neurological problems and sleep for long periods (more than 10 hours) each night.

Who is more likely to get narcolepsy?

Narcolepsy affects both males and females equally. Symptoms often start in childhood, adolescence, or young adulthood (ages 7 to 25), but can occur at any time in life. Since people with narcolepsy are often misdiagnosed with other conditions, such as psychiatric disorders or emotional problems, it can take years for someone to get the proper diagnosis.

Narcolepsy may have several causes. Nearly all people with narcolepsy who have cataplexy have extremely low levels of the naturally occurring chemical hypocretin, which promotes wakefulness and regulates REM sleep. Hypocretin levels are usually normal in people who have narcolepsy without cataplexy.

Although the cause of narcolepsy is not completely understood, current research suggests that narcolepsy may be the result of a combination of factors working together to cause a lack of hypocretin. These factors include:

• Autoimmune disorders—When cataplexy is present, the cause is most often the loss of brain cells that produce hypocretin. Although the reason for this cell loss is unknown, it appears to be linked to abnormalities in the immune system. Autoimmune disorders occur when the body’s immune system turns against itself and mistakenly attacks healthy cells or tissue. Researchers believe that in individuals with narcolepsy, the body’s immune system selectively attacks the hypocretin-containing brain cells because of a combination of genetic and environmental factors.
• Family history—Most cases of narcolepsy are sporadic, meaning the disorder occurs in individuals with no known family history. However, clusters in families sometimes occur—up to 10 percent of individuals diagnosed with narcolepsy with cataplexy report having a close relative with similar symptoms.
• Brain injuries—Rarely, narcolepsy results from traumatic injury to parts of the brain that regulate wakefulness and REM sleep or from tumors and other diseases in the same regions.

In the past few decades, scientists have made considerable progress in understanding narcolepsy and identifying genes strongly associated with the disorder. Groups of neurons in several parts of the brain interact to control sleep, and the activity of these neurons is controlled by a large number of genes. The loss of hypocretin-producing neurons in the hypothalamus is the primary cause of type 1 narcolepsy. These neurons are important for stabilizing sleep and wake states.

The human leukocyte antigen (HLA) system of genes plays an important role in regulating the immune system. This gene family provides instructions for making a group of related proteins called the HLA complex, which helps the immune system distinguish between good proteins from an individual’s own body and bad ones made by foreign invaders like viruses and bacteria.

One of the genes in this family is HLA-DQB1. A variation in this gene, called HLA-DQB1*06:02, increases the chance of developing narcolepsy, particularly the type of narcolepsy with cataplexy and a loss of hypocretins (also known as orexins). HLA-DQB1*06:02 and other HLA gene variations may increase susceptibility to an immune attack on hypocretin neurons, causing these cells to die. Most people with narcolepsy have this gene variation and may also have specific versions of closely related HLA genes.

However, it is important to note that these gene variations are common in the general population and only a small portion of the people with the HLA-DQB1*06:02 variation will develop narcolepsy. This indicates that other genetic and environmental factors are important in determining if an individual will develop the disorder.

Narcolepsy follows a seasonal pattern and is more likely to develop in the spring and early summer after the winter season, a time when people are more likely to get sick. By studying people soon after they develop the disorder, scientists have discovered that individuals with narcolepsy have high levels of anti-streptolysin O antibodies, indicating an immune response to a recent bacterial infection such as strep throat. Also, the h2N1 influenza epidemic in 2009 resulted in a large increase in the number of new cases of narcolepsy. Together, this suggests that individuals with the HLA-DQB1*06:02 variation are at risk for developing narcolepsy after they are exposed to a specific trigger, like certain infections that trick the immune system to attack the body.

How is narcolepsy diagnosed and treated?

Diagnosing narcolepsy

A clinical examination and detailed medical history are essential for diagnosis and treatment of narcolepsy. Individuals may be asked by their doctor to keep a sleep journal noting the times of sleep and symptoms over a one- to two-week period. A physical exam can rule out or identify other neurological conditions that may be causing the symptoms.

Two specialized tests, which can be performed in a sleep disorders clinic, are required to establish a diagnosis of narcolepsy:

1. Polysomnogram (PSG or sleep study)—The PSG is an overnight recording of brain and muscle activity, breathing, and eye movements. A PSG can help reveal whether REM sleep occurs early in the sleep cycle and if an individual’s symptoms result from another condition such as sleep apnea.
2. Multiple sleep latency test (MSLT)—The MSLT assesses daytime sleepiness by measuring how quickly a person falls asleep and whether they enter REM sleep.

Occasionally, it may be helpful to measure the level of hypocretin in the fluid that surrounds the brain and spinal cord. To perform this test, a doctor will withdraw a sample of the cerebrospinal fluid using a lumbar puncture (also called a spinal tap) and measure the level of hypocretin-1.

Treating narcolepsy

Although there is no cure for narcolepsy, some of the symptoms can be treated with medicines and lifestyle changes.

Medications

• Modafinil—The initial line of treatment is usually a central nervous system stimulant such as modafinil. Modafinil is usually prescribed first because it is less addictive and has fewer side effects than older stimulants. For most people these drugs are generally effective at reducing daytime drowsiness and improving alertness.
• Amphetamine-like stimulants—In cases where modafinil is not effective, doctors may prescribe amphetamine-like stimulants such as methylphenidate to alleviate EDS. However, these medications must be carefully monitored because they can have side effects.
• Antidepressants—Two classes of antidepressant drugs have proven effective in controlling cataplexy in many individuals: tricyclics (including imipramine, desipramine, clomipramine, and protriptyline) and selective serotonin and noradrenergic reuptake inhibitors (including venlafaxine, fluoxetine, and atomoxetine).
• Sodium oxybate—Sodium oxybate (also known as gamma hydroxybutyrate or GHB) has been approved by the U.S. Food and Drug Administration (FDA) to treat cataplexy and excessive daytime sleepiness in individuals with narcolepsy. Due to safety concerns associated with the use of this drug, the distribution of sodium oxybate is tightly restricted.
• Histamine 3 receptor antagonist/inverse agonist—Pitolisant was recently approved by FDA as the only non-scheduled product for treating excessive daytime sleepiness or cataplexy in adults with narcolepsy. Pitolisant, which has been commercially available in the U.S. since 2019, is thought to increase histamine levels in the brain. The most common adverse reactions to Pitolisant are insomnia, nausea, and anxiety.

Lifestyle changes

Drug therapy should accompany various lifestyle changes. Remembering the following seven tips may be helpful:

1. Take short naps. Many individuals take short, regularly scheduled naps at times when they tend to feel sleepiest.
2. Maintain a regular sleep schedule. Going to bed and waking up at the same time every day, even on the weekends, can help people sleep better.
3. Avoid caffeine or alcohol before bed. Individuals should avoid alcohol and caffeine for several hours before bedtime.
4. Avoid smoking, especially at night.
5. Exercise daily. Exercising for at least 20 minutes per day at least four or five hours before bedtime also improves sleep quality and can help people with narcolepsy avoid gaining excess weight.
6. Avoid large, heavy meals right before bedtime. Eating very close to bedtime can make it harder to sleep.
7. Relax before bed. Relaxing activities such as a warm bath before bedtime can help promote sleepiness. Also make sure the sleep space is cool and comfortable.

Safety precautions, particularly when driving, are important for everyone with narcolepsy. Suddenly falling asleep or losing muscle control can transform actions that are ordinarily safe, such as walking down a long flight of stairs, into hazards.

The Americans with Disabilities Act (ADA) requires employers to provide reasonable accommodations for all employees with disabilities. Adults with narcolepsy can often negotiate with employers to modify their work schedules so they can take naps when necessary and perform their most demanding tasks when they are most alert.

Similarly, children and adolescents with narcolepsy may be able to work with school administrators to accommodate special needs, like taking medications during the school day, modifying class schedules to fit in a nap, and other strategies.

Additionally, support groups can be extremely beneficial for people with narcolepsy.

What are the latest updates on narcolepsy?

The mission of the National Institute of Neurological Disorders and Stroke (NINDS) is to seek fundamental knowledge about the brain and nervous system and to use that knowledge to reduce the burden of neurological disease. The NINDS, a component of the National Institutes of Health (NIH), along with several other NIH Institutes and Centers, supports research on narcolepsy and other sleep disorders through grants to medical institutions across the country.

Additionally, the National Heart, Lung, and Blood Institute (NHLBI) manages the National Center on Sleep Disorders Research (NCSDR), which coordinates federal government sleep research activities, promotes doctoral and postdoctoral training programs, and educates the public and health care professionals about sleep disorders.  

Genetics and biochemicals
NINDS-sponsored researchers are conducting studies devoted to further clarifying the wide range of genetic—both HLA genes and non-HLA genes—and environmental factors that may cause narcolepsy. Other investigators are using animal models to better understand hypocretin and other chemicals such as glutamate that may play a key role in regulating sleep and wakefulness. Researchers are also investigating wake-promoting compounds to widen the range of available therapeutic options and create treatment options that reduce undesired side effects and decrease the potential for abuse. A greater understanding of the complex genetic and biochemical bases of narcolepsy will eventually lead to new therapies to control symptoms and may lead to a cure.

Immune system
Abnormalities in the immune system may play an important role in the development of narcolepsy. NINDS-sponsored scientists have demonstrated the presence of unusual immune system activity in people with narcolepsy. Further, strep throat and certain varieties of influenza are now thought to be triggers in some at-risk individuals. Other NINDS researchers are also working to understand why the immune system destroys hypocretin neurons in narcolepsy in the hopes of finding a way to prevent or cure the disorder.

Sleep biology
NINDS continues to support investigations into the basic biology of sleep, such as examining the brain mechanisms involved in generating and regulating REM sleep and other sleep behaviors. Since sleep and circadian rhythms are controlled by networks of neurons in the brain, NINDS researchers are also examining how neuronal circuits function in the body and contribute to sleep disorders like narcolepsy. A more comprehensive understanding of the complex biology of sleep will give scientists a better understanding of the processes that underlie narcolepsy and other sleep disorders.

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Learn About Clinical Trials

Clinical trials are studies that allow us to learn more about disorders and improve care. They can help connect patients with new and upcoming treatment options.

How can I or my loved one help improve care for people with narcolepsy?

The NeuroBioBank serves as a central point of access to collections that span neurological, neuropsychiatric, and neurodevelopmental diseases and disorders. Tissue from individuals with narcolepsy is needed to enable scientists to study this disorder more intensely. Participating groups include brain and tissue repositories, researchers, NIH program staff, information technology experts, disease advocacy groups, and, most importantly, individuals seeking information about opportunities to donate.

Additionally, NINDS supports genetic and immunological research in narcolepsy at the Stanford University Center for Narcolepsy. Blood samples from individuals with narcolepsy can be sent by mail and are needed to enable scientists to study this disorder more intensely.

Consider participating in a clinical trial so clinicians and scientists can learn more about narcolepsy and related disorders. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.

All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.

For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with narcolepsy at Clinicaltrials.gov.

Where can I find more information about narcolepsy?

Information may be available from the following organizations:

Narcolepsy Network
Phone: 401-667-2523 or 888-292-6522

National Heart, Lung, and Blood Institute (NHLBI)
Phone: 301-592-8573 or 800-575-9355

National Library of Medicine
Phone: 301-594-5983 or 888-346-3656

National Sleep Foundation
Phone: 703-243-1697

Wake Up Narcolepsy
Phone: 978-751-3693

Cataplexy: Symptoms, causes, and treatment

Cataplexy is a sudden, brief loss of voluntary muscle tone triggered by strong emotions such as laughter, excitement, anger or fear.

Around one-third of people who experience cataplexy also have narcolepsy, a sleep disorder that also features excessive daytime sleepiness and sleep paralysis. Laughter is the most common trigger of cataplexy.

Narcolepsy is thought to affect 135,000–200,000 people in the United States.

Cataplexy occurs during waking hours. During a mild attack, there may be a barely visible weakness in a muscle, such as drooping of the eyelids. A more severe episode may involve a total body collapse.

Although it is a different condition, cataplexy is sometimes misdiagnosed as a seizure disorder.

There is no cure for cataplexy, but it can be managed with medications and modification of potential triggers.

Fast facts on cataplexy

• During an episode of cataplexy, an individual is awake but temporarily paralyzed.
• Cataplexy is worsened by fatigue and strong emotion and affects males and females equally.
• Cataplexy is linked to narcolepsy and can occur after suddenly stopping an antidepressant medication.
• Sodium oxybate is approved by the U. S. Food and Drug Administration (FDA) for the treatment of cataplexy

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Share on PinterestA person with cataplexy may collapse temporarily as a result of excitement, laughter, or strong emotions.

Cataplexy is a sudden loss of muscle control, typically on both sides of the body, triggered by strong, often pleasant emotions.

Laughter is the most typical trigger, but other triggers may include happiness, excitement, annoyance, surprise, fear, or a stressful event.

Cataplexy is most often associated with narcolepsy, but it can occur with other rare disorders such as Niemann-Pick type C disease, Prader-Willi syndrome and Wilson’s disease.

Occasionally, cataplexy can be seen in other medical conditions including stroke, multiple sclerosis, head injury and encephalitis.

The duration of a cataplexy attack is brief, generally lasting anywhere from a few seconds to a few minutes — usually less than 2 minutes — followed by a rapid return of normal muscle tone and function.

The REM sleep disassociation hypothesis suggests that cataplexy is the muscle paralysis that normally occurs during REM sleep intruding into waking hours.

The exact underlying cause of this intrusion is unknown, but a loss of neurons that produce hypocretin (also known as orexin) is thought to be a major contributing factor. Hypocretin is a neurotransmitter involved in the promotion of wakefulness within the sleep/wake cycle,

People with cataplexy have been found to have a certain human leukocyte antigen, variations in T-cell receptors, or dysfunctional immune system responses to certain antigen exposures. It is thought that narcolepsy may be an autoimmune disorder, but more research is needed.

A study published in the Journal of Clinical Investigation in 2010 revealed that the loss of hypocretin is caused by an autoimmune response targeting tribbles homolog 2 (trib 2) autoantigens. This autoimmune response causes trib 2 antibodies to form, which target and kill the neurons in the brain that produce hypocretin.

The frequency of cataplectic episodes varies from less than one per year to several per day. On average, a person with cataplexy will experience one or more episodes each week.

Symptoms of cataplexy associated with narcolepsy often begin in childhood and young adulthood, between the ages of 7 and 25 years, but it can start at any time.

Cataplectic attacks can vary widely. They may be mild and barely noticeable or severe with full body collapse.

Symptoms of a cataplectic episode may include:

• facial twitching, flickering, or grimacing
• unusual tongue movements
• jaw tremor
• dropping the head or jaw
• knee trembling or buckling
• drooping eyelid
• speech difficulty

A person may collapse and be unable to move.

The episode usually lasts a few minutes or less and then passes without intervention. However, it is important to make sure the person will not hurt themselves if they collapse to a fall.

An individual experiencing cataplexy remains conscious, is able to breathe, and can move their eyes.

Tests can help diagnose narcolepsy with cataplexy, but a diagnosis is also based on the patient’s description of events and history.

Often, an individual complains to a doctor about excessive daytime sleepiness (EDS) with a persistent background feeling of sleepiness during waking hours. EDS is commonly the first symptom of narcolepsy. However, if cataplexy occurs with EDS, narcolepsy is likely to be present.

Individuals who have EDS or symptoms of cataplexy should see their medical provider.

A sleep study (a polysomnogram) will most likely be ordered by the provider, followed by a Multiple Sleep Latency Test (MSLT), a series of five naps scheduled to be taken over the course of a day.

Carrying out the MSLT the day after polysomnography enables doctors to find out whether or not the sleep obtained the previous night has any effect on daytime napping.

There is no cure for cataplexy, and treatment is symptom management with good sleep hygiene practices and the use of medication as necessary.

Additionally, safety measures should be put in place to avoid serious injury that can result from falls.

Good sleep hygiene practices include:

• keeping a consistent sleep schedule, including getting up and going to bed at the same time every day, even on weekends or during vacations
• striving for at least 7 to 8 hours of sleep, or the amount that normally makes you feel refreshed
• making the bedroom quiet and relaxing and keeping it at a comfortable, cool temperature
• limiting exposure to light in the evenings
• exercising regularly and maintaining a healthful diet
• avoiding a large meal before bedtime and opting for a light, healthful snack if necessary
• avoiding caffeine in the late afternoon or evening
• avoiding alcohol
• scheduling one or more short naps during the day.

Sodium oxybate at doses between 6 and 9 grams (g) nightly is the medication approved by the U.S. Food and Drug Administration (FDA) for the treatment of cataplexy. Sodium oxybate is effective in reducing both the frequency and intensity of attacks.

Possible adverse effects are dizziness, headache, and nausea. This medication should not be used in conjunction with alcohol or any other central nervous system (CNS) depressant due to the risk of respiratory depression and other significant CNS depressant effects.

Other medications that may help control symptoms are antidepressants. These include venlafaxine, a serotonin-noradrenaline reuptake inhibitor (SNRI) antidepressant, and tricyclic antidepressants such as clomipramine, imipramine, and desipramine.

However, evidence supporting the use of antidepressants for cataplexy is varied. In addition, abrupt withdrawal of venlafaxine can potentially cause rebound cataplexy.

In regards to safety, even if an individual senses the onset of an episode, injury is very possible with cataplexy.

To help avoid injury from occurring with cataplexy:

Share on PinterestInjury can occur if a person falls suddenly.

• Assess for and be aware of potential dangers such as glass, sharp edges, and heights.
• Practice relaxation and stress management techniques.
• Avoid sleep deprivation.
• Avoid situations that are likely to evoke strong emotions, or prepare ahead of time by sitting down or having a companion nearby.
• Do not drive a car or operate heavy machinery until cleared by a medical provider.
• Enlist the help of significant others and friends, and educate them on the seriousness of the disorder.

Cataplexy is a transient, sudden loss of voluntary muscle control brought on by a strong emotional trigger such as laughing or excitement. Cataplexy is almost always associated with narcolepsy.

There is no cure, but education, sleep hygiene practices, and prescription medication can help people with this condition enjoy a better quality of life.

Cataplexy. What is cataplexy?

IMPORTANT
The information in this section should not be used for self-diagnosis or self-treatment. In case of pain or other exacerbation of the disease, only the attending physician should prescribe diagnostic tests. For diagnosis and proper treatment, you should contact your doctor.

Cataplexy is a neurological syndrome characterized by a sudden loss of muscle tone when experiencing strong emotions. The attack occurs against the background of laughter, anger or fear. Focal muscle weakness is accompanied by the loss of functions of a part of the body (arms, legs, neck), complete weakness is accompanied by a fall of a person and short-term immobility. For diagnosis, a clinical and anamnestic method is used: observation, questioning, analysis of video recordings. Treatment involves taking medications, maintaining a sleep-wake regimen.

ICD-10

G47.4 Narcolepsy and cataplexy

• Causes of cataplexy
• Pathogenesis
• Symptoms of cataplexy
• Complications
• Diagnostics
• Treatment of cataplexy
  • Conservative therapy
  • Experimental treatment
• Prognosis and prevention
• Prices for treatment

General

The term “cataplexy” in translation from the ancient Greek language means “horror”, “fear”, “confusion”. Synonymous names of the syndrome are affective loss of muscle tone, blockade of tone, affective adynamia, emotional asthenia. The course is chronic. The debut often occurs in adolescence or adolescence. As you get older, seizures become rarer and less intense. The prevalence is about 20 cases per 100,000 people. Men are more susceptible to the syndrome.

cataplexy

Causes of cataplexy

Cataplexy is based on an organic lesion of the central nervous system, prolonged mental stress, and the effects of toxins on the body. Predisposing factors include chronic intoxication with alcohol or drugs, being in the area of ​​a man-made disaster or military operations, and a strong fright. An attack of loss of muscle tone is a syndrome that occurs in a number of diseases:

• Type I narcolepsy. This pathology is the cause of more than 90-92% of cases of cataplexy. It is characterized by abnormal daytime sleepiness with attacks of sudden falling asleep. The basis of the phenomena is a neurochemical violation of the basic regulation of the sleep-wake cycle.
• Hereditary diseases. In Niemann-Pick type C disease, cataplexy is manifested by weakness of the muscles of the jaws, neck, legs, and is more often found in adolescents. In Prader-Willi syndrome, attacks of atony are rare and develop as manifestations of secondary narcolepsy.
• Diseases of the brain. Cataplexy can be caused by transient ischemia or compression of the brainstem. The attack unfolds in patients with cervical osteochondrosis, stenosing lesions of the vertebral arteries, vascular or tumor formations. Muscle atony occurs after tilting and sharp turns of the head.

Pathogenesis

The etiology of narcolepsy is a violation of the biochemical and neurological foundations of the regulation of sleep-wake cycles. The pathogenetic cause of cataplexy in type 1 narcolepsy is insufficient production of the neurotransmitter orexin A and B. According to its chemical structure, it is a peptide, the precursor of which is the protein pre-prohypocretin. Its physiological function is to provide alertness and maintain appetite.

Orexinergic neurons are few in number, concentrated mostly in the hypothalamus (about 10-20 thousand neurotransmitter-secreting cells). Their activity in patients with cataplexy is reduced. The reasons continue to be investigated, the most likely hypothesis is an autoimmune lesion. According to the data of laboratory tests, the level of orexin A in the cerebrospinal fluid (less than 110 pg/ml) and in the blood (below 0.5 pg/ml) was lowered in the subjects.

The axons of orexinergic neurons strongly branch, innervate the cells of various parts of the brain that produce acetylcholine, glutamic acid, and serotonin. These neurotransmitters excite the nervous system. Their deficiency is manifested by fatigue, drowsiness, depression.

Symptoms of cataplexy

For the syndrome, attacks of muscle atony are typical, lasting from 5-10 seconds to several minutes. Provoking factors are strong emotions: in 90% of cases – laughter, less often – fear, anger, surprise. Consciousness remains clear, loss of tone is bilateral, may affect isolated muscle groups or muscles of the whole body. Depending on this, two types of cataplexy are distinguished: focal and classical.

With focal cataplexy, the mechanics of the actions performed are violated. If the muscles of the legs weaken, a walking or standing person falls to his knees. With a decrease in tone in the neck, the head involuntarily falls on the chin, nodding movements occur. If the pathology affects the muscles of the face, the lower jaw sags during an attack, the eyelids close, or speech becomes slurred. Sometimes there are muscle twitches.

Focal attacks of cataplexy may remain invisible to others, but the classic version of the syndrome always attracts attention. The tone of all muscle groups is lost, the patient falls, remaining conscious. If an injury occurs during an attack, he cannot respond to pain or change position. Intense emotion can provoke a series of attacks – a cataplexy status lasting from 2-5 minutes to an hour.

Sometimes attacks end with sleep. Their frequency and duration vary considerably even in one patient. For example, a remission can be observed within a month, and during an exacerbation, attacks develop several times a day. With a long course of the disease, patients learn to build their behavior in such a way as to avoid the emotions that provoke an attack.

Complications

The main complication of cataplexy is the physical injury caused by a sudden fall. The situation is aggravated by the fact that in the first minutes after the incident, a person cannot report pain and receive help. In the life of patients there are many restrictions. For example, they are forced to give up driving, as muscle weakness prevents them from continuing to drive and causes traffic accidents.

Attacks interfere with the performance of work duties that require constant concentration. It is impossible to work on the production line, in conditions of increased danger. With frequent attacks that are difficult to treat, patients become socially maladjusted – they lose their jobs, do not leave the house alone.

Diagnostics

There are no specific instrumental examinations to confirm cataplexy. Diagnosis is carried out by clinical methods. The main examination is carried out by a neurologist, in addition, the participation of a psychiatrist, a somnologist may be required. Most patients are diagnosed with type 1 narcolepsy. 65-70% of people with this disorder develop cataplexy, which is described as a state of muscle weakness following overwhelming daytime sleepiness.

A video analysis method can be used. Classic cataplexy attacks have three phases:

• the first is characterized by closing the eyes, bending the knees;
• the second – the disappearance of a smile, lowering of the jaw, violation of posture;
• the third – atonic laying on the floor.

Isolation of these phases, taking into account provoking factors and concomitant disease is important in differential diagnosis. Should be excluded:

• conversion disorder. Individuals with hysterical neurosis may experience conversion symptoms similar to those of a cataplexy. Distinctive features: the presence of a hysteroid character, the absence of narcolepsy and a direct connection between seizures and vivid emotions.
• syncopal state. Fainting caused by a transient slowdown in blood flow in the brain is characterized by a decrease in skeletal muscle tone. Differential signs: short-term loss of consciousness, pallor of the skin, duration – up to 20-30 seconds.
• Atonic absence. Paroxysmal changes in muscle tone with a fall occur with small epileptic seizures. Differences from cataplexy: the presence of an “aura”, unconsciousness, amnesia for a seizure that occurred (often). Atonic absences affect all the muscles of the body, but sometimes they are very short (the patient does not have time to fall).

Treatment of cataplexy

Conservative therapy

Methods that can affect the cause of the syndrome are at the stage of experimental development. To date, pharmacotherapy is used in practice, which does not permanently eliminate attacks of muscle atony, but provides long-term remission and complete relief of symptoms. The key goal of therapeutic and preventive measures is to improve the quality of life. Their basis is the use of the following medicines:

• SSRIs. During an attack, eye movements characteristic of REM sleep are noted. Selective serotonin reuptake inhibitors suppress REM sleep, reduce the frequency of atonic seizures.
• Tricyclic antidepressants. This group of drugs can be prescribed instead of SSRIs. They also suppress the phase of REM sleep, increase the duration of delta sleep. They act more intensively, but more often provoke side effects.
• Anticonvulsants. Anticonvulsants commonly used to treat epilepsy prevent the muscle paroxysms of cataplexy. When they are taken, the state of muscle weakness occurs less frequently, proceeding in a milder form.

In case of sleep disorders, imidazopyridines, cyclopyrrolones, and melatonin preparations are additionally prescribed. As a rule, adherence to medication prescriptions is necessary throughout life. In addition to drug treatment, patients are advised to strictly observe sleep hygiene: sleep for at least 8 hours at night and no more than 30 minutes during the day at the same time. Night sleep deprivation, night shift work, stressful situations are contraindicated.

Experimental treatment

In the last ten years, the effectiveness of drugs that can affect the pathogenetic link of cataplexy has been actively studied. Orexin (hypocretin) and neorexin preparations have been developed and continue to be tested. The latter group includes H3-antagonists (histamine receptor blockers) and monoaminergic reuptake blockers of various mediators.

Pitolisant is an H3 receptor inverse agonist. It is approved by the European Union in 2016 for the treatment of type I narcolepsy accompanied by cataplexy. Another 8 neorexin drugs are undergoing clinical trials. For example, JZP-110 blocks dopamine uptake and increases daytime wakefulness. Treatment with drugs based on orexin, gene therapy and immune therapy are at the stage of experimental development.

Prognosis and prevention

Subject to the doctor’s prescriptions, patients with cataplexy can achieve a stable remission or a decrease in the severity of seizures. Preventive measures – maintaining the correct mode of wakefulness and sleep, increasing stress resistance, avoiding situations that provoke intense emotions. It is necessary to refuse the use of psychostimulating substances, coffee, alcohol. To prevent another attack before an emotionally intense event, it is recommended to take sedatives.

You can share your medical history, what helped you in the treatment of cataplexy.
/ E.Z. Yakupov, Yu.V. Troshina // Bulletin of modern clinical medicine. – 2015. – Vol. 8, app. 1.

• Deep-brain Imaging Identifies Cataplexy-on Neurons In The Amygdala. Sleep, Volume 42, Issue Supplement_1, April 2019, Pages A1–A2, 12 April 2019.
• Narcolepsy: modern concepts of pathophysiology and treatment methods. A.V. Zakharov, E.V. Khivintseva, I.E. Poverennova, T.V. Romanova.
• Narcolepsy/ Zakharov AV// National Somnological Society. – 2015.
• This article was prepared based on the materials of the site: https://www.krasotaimedicina.ru/

IMPORTANT
Information from this section cannot be used for self-diagnosis and self-treatment. In case of pain or other exacerbation of the disease, only the attending physician should prescribe diagnostic tests. For diagnosis and proper treatment, you should contact your doctor.

Cataplexy: Causes, Symptoms, Causes | doc.ua

Cataplexy is a disease that causes short-term relaxation of the muscles of the body, as a result of which a person can fall or change his body position abruptly.

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Here you can choose a doctor who specializes in the treatment of Cataplexy If you are not sure about the diagnosis, make an appointment with a general practitioner or general practitioner to clarify the diagnosis.

Cataplexy articles:

Causes

Symptoms

Treatment

Which doctor treats Cataplexy

4.9

He deals with the treatment of diseases of the musculoskeletal system and disorders of the nervous system. Proficient in intra-articular injections, paravertebral blockades and acupuncture

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A neurologist of the highest category, candidate of medical sciences, examines patients using methods such as: computer electroencephalography and rheovasography. Also, performs massage (modern techniques) for vertebrogenic diseases of the nervous system

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Neurologist practicing modern approaches to the diagnosis and treatment of acute and chronic pain syndromes based on the experience of leading American and European medical schools in the head, neck, back, lower back, pelvis, limbs. Conducts rehabilitation of patients after bone fractures, injuries of the spine, joints, muscles, ligaments, tendons; restores motor activity after a stroke. He uses myofascial therapy, blockades of varying complexity, kinesitherapy

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Causes

As a rule, the main cause of this disease are excessive emotional experiences, both negative and positive, a high level of emotional stress. But it is possible that the disease can develop in parallel or be the result of disorders of the nervous system.

Prevalence

As practice shows, catalepsy can develop from narcolepsy (sudden sleep disorder), this occurs in more than 50% of cases.

Symptoms

An attack of catalepsy is characterized by complete or partial immobilization of the limbs and head: weakness in the arms and legs, head hanging down. In addition, sweating appears, the face turns red, the pulse slows down, reflexes disappear from the mucous membranes. But with all these symptoms, the person is conscious. The attack lasts no more than 3 minutes, although exceptions are possible – longer seizures. It is possible that after an attack there comes an attack of sleep, although the parallel development of these conditions is also possible.

Treatment

Treatment is prescribed depending on the degree of development of the disease. As a drug prophylaxis, psychostimulant drugs (amphetamine, ephidril, etc.