Cataplexy is a frequent symptom of narcolepsy in which a person has a sudden loss of muscle control while they are awake. Episodes are usually triggered by strong emotions, like excitement, fear, or surprise. Symptoms can be mild or severe and sometimes require a person to take special precautions to keep themselves safe during an episode.
What Is Cataplexy?
Cataplexy causes a person to rapidly lose control of their muscles. Episodes of cataplexy range in severity. Mild cataplexy may cause only slight weakness in some muscles, while severe cataplexy can cause a person to collapse. Regardless of the episode’s severity, the person remains conscious and aware of their surroundings.
Episodes of cataplexy are brief, lasting up to a few minutes. These episodes are not typically dangerous, as long as the person is in a safe area. Most episodes are triggered by emotions, such as:
- Joking
- Laughter
- Excitement
- Anger
- Frustration
What Is the Relationship Between Narcolepsy and Cataplexy?
About two-thirds of people with narcolepsy have cataplexy, including 80% of children with narcolepsy. Narcolepsy is a chronic and lifelong medical condition in which the brain isn’t able to appropriately control when a person sleeps and when they wake up.
There are two types of narcolepsy. While only one type is associated with cataplexy, both involve symptoms such as excessive daytime sleepiness, brief and uncontrollable lapses into sleep, and frequent nighttime awakenings.
- Narcolepsy type 1: People with narcolepsy type 1 typically experience cataplexy and have a deficiency of a brain chemical called orexin. A person may be diagnosed with this type of narcolepsy if they are found to have low levels of orexin, even if they have never experienced cataplexy.
- Narcolepsy type 2: Those with type 2 narcolepsy do not experience cataplexy. When people with this type of narcolepsy are tested, they typically have normal levels of orexin. Sometimes people who were initially diagnosed with narcolepsy type 2 develop cataplexy, which means the disease has progressed.
What Causes Cataplexy?
Scientists don’t completely understand what causes narcolepsy with cataplexy, though they have several theories. People who experience narcolepsy with cataplexy typically have insufficient orexins, which are neurotransmitters that help the body stay awake.
When these brain chemicals are in short supply, the body is less able to regulate sleep and wakefulness. This allows things that usually only occur during sleep to happen while a person is awake, including dreamlike hallucinations and sleep paralysis. The momentary loss of muscle control that occurs in cataplexy is similar to the loss of motor control that happens during REM sleep.
Researchers have also found differences in brain function in people who have narcolepsy with cataplexy. In these people, the area of the brain that processes rewarding or humorous stimuli is abnormally active when stimulated. Notably, laughter is a common cataplexy trigger in adults, and anticipation of a reward often triggers cataplexy in children.
Almost everyone who has episodes of cataplexy also has narcolepsy. However, there are a handful of uncommon exceptions.
- Angelman syndrome: Angelman syndrome is a genetic condition that causes neurological problems that may include cataplexy as well as speech impairment, problems with balance, a small-sized head, and recurrent seizures.
- Niemann-Pick type C disease: This rare genetic condition causes an array of neurological symptoms. In addition to cataplexy, it can cause difficulty swallowing, problems walking, and loss of sight and hearing.
- Norrie disease: Norrie disease occurs only in infants who are male at birth. Its primary symptom is blindness, but it can also cause many other neurological symptoms, including cataplexy.
- Prader-Willi syndrome: People with Prader-Willi syndrome may have issues in growth, weak muscles, and extreme hunger that can contribute to overeating and obesity. People with this syndrome may show signs of cataplexy.
- Drug side effects: Multiple medications can cause cataplexy or similar symptoms. These drugs include some types of anti-seizure medications, anti-psychotics, and sleep aids.
- Brain lesions: Rarely, damage in a part of the brain called the hypothalamus can cause cataplexy. This damage may be due to a stroke, inflammation, tumor, or malformations of the blood vessels. In these cases, cataplexy usually occurs with other neurological problems.
Cataplexy Symptoms
All episodes of cataplexy are characterized by the sudden loss of muscle tone while a person remains conscious and awake.
The symptoms of cataplexy usually begin with the muscles of the face. Facial expressions are typically interrupted and the mouth may fall open. Mild symptoms may be limited to the face and last as little as 30 seconds. Severe symptoms can cause the knees to buckle. People may remain paralyzed for as long as a few minutes.
Symptoms of cataplexy in young children may be different from adults in several ways. Children tend to have more frequent episodes, have episodes triggered by expectations of a reward, and may experience more complex movements. Symptoms in children may also include:
- Drooping eyelids
- Head rolling
- Sticking out the tongue
- Unsteadiness when walking
- Sudden contractions in facial muscles
How Is Cataplexy Diagnosed?
Doctors aren’t typically able to observe an episode of cataplexy directly, so the condition may be diagnosed by having a person or their relatives describe an episode. Identifying whether a person is experiencing cataplexy can be challenging because symptoms are often brief and the type, frequency, and severity of symptoms vary between individuals.
Adults being evaluated for narcolepsy are usually asked if they have muscle weakness when they laugh or tell a joke. Videos can be especially helpful in identifying cataplexy in children since their symptoms may be different from adults and can sometimes be mistaken for seizures or fainting.
Identifying cataplexy helps medical providers diagnose a person with narcolepsy and determine its type. Additional tests used to diagnose narcolepsy include a sleep study, a multiple sleep latency test, and a measurement of orexins.
What Are Treatments for Cataplexy?
The primary approach to managing cataplexy is through medications, which help to reduce the frequency of episodes.
Cataplexy in adults may be treated with medications that inhibit REM sleep. Drugs that may be used include antidepressants and other medicines that alter the release of brain chemicals.
Medication is also the mainstay of treatment for children with cataplexy, though different drugs may be used for children under the age of 7.
Managing Cataplexy in Everyday Life
Depending on how well your cataplexy is managed with medication, you may need to take steps to ensure that you are safe in situations where symptoms may occur. Activities such as driving, swimming, cooking, crossing the street, climbing, or operating machinery may require special precautions.
Your doctor can suggest certain steps to minimize the risks of cataplexy.
- Use caution with triggers: If you know certain things can trigger cataplexy, like tickling or joking, make sure to avoid those activities at times when a cataplexy episode might be dangerous, like when driving or swimming.
- Talk with schools: It’s also important to talk to teachers and school administrators to arrange accommodations like time for taking medication and naps.
- Practice safe driving: Ask your doctor whether it’s safe for you to drive. If so, practices like keeping trips short, driving only during times when you tend to be alert, or driving with a partner may help keep you and other drivers on the road safe.
- Avoid aggravating cataplexy: Recreational drugs and alcohol can aggravate symptoms, and should be avoided.
- Wear a life jacket: People with cataplexy should wear a life jacket when engaged in swimming and other recreational activities around water. It may also be helpful to be in the water only when accompanied by a partner who understands your condition.
Managing cataplexy can require adjustments to daily life. It may be helpful to locate a support group for people with narcolepsy or other syndromes that cause cataplexy. Other people with cataplexy may be able to provide empathy and share helpful coping strategies.
How to Get Quality Sleep
Talk to your doctor about any special measures you should take to ensure quality sleep. People who have cataplexy along with narcolepsy often benefit from practicing good sleep hygiene. The following steps may be helpful:
- Keep a consistent sleep schedule every day
- Don’t drink alcohol or caffeine near bedtime
- Schedule brief naps throughout the day
- Get exercise every day
- Develop a relaxing bedtime routine
References
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