Cataplexy is a symptom of narcolepsy, characterized by sudden episodes of muscle weakness triggered by strong emotions.

How Common is Cataplexy?

Cataplexy affects 1 to 2 out of every 4000 people.

What are signs and symptoms of Cataplexy?

The signs and symptoms of cataplexy include: - Sudden loss of muscle strength, which can be triggered by intense emotions. - Positive emotions such as laughter and excitement are more likely to cause these attacks than negative emotions like anger, fear, and frustration. Laughing is the most common trigger. - During a cataplexy episode, all voluntary muscles may become weak or paralyzed except for the muscles involved in eye movement and breathing. The state of consciousness remains unaffected. - The episodes typically start with the muscles of the face and neck and then spread to the trunk and limbs. - The physical impact depends on the severity of the episode - it can range from facial drooping and slurred speech to a complete collapse. However, the effects usually resolve within two minutes leaving no lingering effects.

How do you get Cataplexy?

Cataplexy can be triggered by intense emotions, particularly positive emotions such as laughter and excitement.

What else can Cataplexy be?

The doctor needs to rule out the following conditions when diagnosing Cataplexy: - Pseudocataplexy, which is part of a larger disorder known as conversion disorder - Lateral hypothalamic lesions accompanied by other neurological problems, such as arteriovenous malformations, cerebrovascular accidents, inflammatory processes, and neoplasms.

What kinds of test are needed for Cataplexy?

The types of tests needed for cataplexy include: - Testing the cerebrospinal fluid (CSF) to check for a decrease in the chemical orexin-A. - Special sleep tests to measure how quickly someone falls asleep and if they enter the REM sleep stage earlier than normal. - The Epworth Sleepiness Scale to track daytime sleepiness and assess the effectiveness of treatment. These tests help doctors confirm the diagnosis of cataplexy and determine the appropriate treatment plan.

How is Cataplexy treated?

Cataplexy is treated with medications called REM sleep-suppressing drugs. These drugs increase the concentrations of norepinephrine and serotonin in the brain. This group of medications includes selective serotonin reuptake inhibitors, norepinephrine reuptake inhibitors, serotonin/norepinephrine reuptake inhibitors, and tricyclic antidepressants. However, tricyclic antidepressants are generally avoided due to their potential side effects. Sodium oxybate is another medication that can be used to treat cataplexy, and it has received FDA approval for this purpose. Its exact mechanism of action is not fully understood, but it is believed to work through a receptor for the brain chemical GABA.

What are the side effects when treating Cataplexy?

The side effects when treating cataplexy with medications that suppress REM sleep include dry mouth, changes in heart rate, confusion, and possible overdoses that can cause serious reactions including heart and brain toxicity. Tricyclic antidepressants, which are sometimes used, can also have side effects such as dry mouth, changes in heart rate, and confusion, among others. Overdoses of tricyclic antidepressants can cause serious reactions including heart and brain toxicity. It is important to note that serotonin syndrome, a potentially life-threatening condition, can occur with the use of medications that affect serotonin. Symptoms of serotonin syndrome include agitation, sweating, and muscle spasms. To avoid serotonin syndrome, it is essential to avoid prescribing multiple serotonergic agents to a single patient.

Are there long term effects of Cataplexy?

The prognosis for cataplexy is that there are various drugs available to help manage the symptoms of cataplexy and excessive sleepiness during the day. However, the only exception is a drug called sodium oxybate, which is used to target all symptoms of narcolepsy type 1.

Cataplexy

What is Cataplexy?

Cataplexy is a symptom of a sleep disorder called narcolepsy, and it involves sudden episodes of muscle weakness triggered by strong emotions. This unique symptom can help in diagnosing narcolepsy, as it is almost exclusively associated with this condition. However, it’s often difficult to notice cataplexy, which leads to it frequently being undiagnosed. Statistics show that while only 19% of patients diagnosed with narcolepsy are also diagnosed with cataplexy, the actual number might be closer to 70%. This means that many people with narcolepsy may be experiencing cataplexy without realizing it.

What Causes Cataplexy?

Type 1 narcolepsy, also known as narcolepsy with cataplexy, is caused by a lack of a certain chemical in the brain called ‘orexin-A.’ This chemical helps to keep us awake. People with type 1 narcolepsy usually have a genetic marker called HLA-DQB1*06:02. This fact suggests that the body attacks its own ‘orexin-A’ producing cells, leading to narcolepsy. This type of narcolepsy often begins in the spring, usually following winter upper respiratory tract infections (like colds). Interestingly, more cases of narcolepsy surfaced after the use of a particular flu vaccine in 2009 (European Pandemrix vaccination), which implies that the body’s immune response to some vaccinations may trigger narcolepsy.

In contrast, ‘secondary’ narcolepsy happens as a result of damage to a part of the brain called the ‘lateral hypothalamus.’ This damage, rare but possible, can be caused by issues such as malformed blood vessels, strokes, inflammation, or tumors, and causes the death of ‘orexin-A’ making cells. People with secondary narcolepsy usually show other neurological problems, as the damage in the brain is not only limited to the ‘lateral hypothalamus.’

Risk Factors and Frequency for Cataplexy

In the United States, narcolepsy is a sleep disorder that affects 1 in every 2000 people, and it affects men and women equally. The symptoms usually start showing up in the teenage years, but it often takes around 15 years for it to be properly diagnosed. Narcolepsy can be of two types: type 1, which includes a symptom called cataplexy, and type 2, which does not include cataplexy. In the U.S., type 1 narcolepsy affects 1 to 2 out of every 4000 people.

Signs and Symptoms of Cataplexy

Narcolepsy is a disorder that requires an accurate and detailed medical history for diagnosis. The doctor will usually ask about any behavior that may cause excessive sleepiness during the day, such as caffeine or tobacco use, inadequate sleep, or poor sleep habits. They will also consider other possible causes of fatigue like anemia, hypothyroidism, or obstructive sleep apnea. People with narcolepsy often feel rested after a short nap or a good night’s sleep, yet they may feel very sleepy within hours of waking up.

Patients with type 1 narcolepsy not only experience excessive sleepiness, but they can also have symptoms related to disturbed Rapid Eye Movement (REM) sleep. These symptoms include cataplexy, hypnagogic hallucinations, and sleep paralysis.

Cataplexy is a sudden loss of muscle strength. These episodes are short and can be triggered by intense emotions.
Positive emotions such as laughter and excitement are more likely to cause these attacks than negative emotions like anger, fear, and frustration. Laughing is the most common trigger.
During a cataplexy episode, all voluntary muscles may become weak or paralyzed except for the muscles involved in eye movement and breathing. The state of consciousness remains unaffected.
The episodes typically start with the muscles of the face and neck and then spread to the trunk and limbs.
The physical impact depends on the severity of the episode – it can range from facial drooping and slurred speech to a complete collapse. However, the effects usually resolve within two minutes leaving no lingering effects.

Testing for Cataplexy

If a person is suspected of having Type 1 narcolepsy, there are some specific signs and tests that doctors look for. The person has to have a common symptom, which is feeling excessively sleepy during the day nearly every day over a period of at least three months. Additionally, there are other physical signs and tests used to confirm the diagnosis.

One of these is testing the fluid that surrounds your brain and spinal cord, known as cerebrospinal fluid (CSF). In people with narcolepsy, there’s normally less of a certain chemical, called orexin-A, in this fluid. Another sign of narcolepsy is a sudden and temporary loss of muscle tone triggered by strong emotions, known as cataplexy.

Finally, special sleep tests are conducted. During these tests, sleep experts measure how quickly someone falls asleep and they see if the person enters a particular sleep phase, called the REM (rapid eye movement) sleep stage, earlier than normal. If the person falls asleep in 8 minutes or less on average and enters the REM sleep stage in 2 or more naps, this could indicate narcolepsy.

Treatment Options for Cataplexy

While lifestyle adjustments like planned naps and adequate night sleep help manage excess sleepiness associated with type 1 narcolepsy, the sudden muscle weakness called cataplexy is treated with medicine. Since there’s no cure for type 1 narcolepsy, this treatment is long-term and often tailored to the individual with a mix of different medications. Doctors can use a tool called the Epworth Sleepiness Scale – which measures daytime sleepiness – to track how well the treatment is working.

Daytime sleepiness can be managed successfully using medications that promote alertness, as lifestyle changes often cannot completely relieve this symptom. Modafinil and armodafinil are often used as the first-line treatment. These medicines work by increasing the activity of dopamine, a chemical in the brain involved in wakefulness, though the exact way they work is not fully understood. In 2019, the FDA approved two new medications – pitolisant and solriamfetol – for excessive sleepiness. Pitolisant works on a different brain chemical called histamine, while solriamfetol increases the activity of both dopamine and a related chemical, norepinephrine. For those who still experience excessive daytime sleepiness despite primary treatment, certain stimulant medications such as dextroamphetamine-amphetamine and methylphenidate may be considered.

To manage cataplexy, medications called REM sleep-suppressing drugs are used. These drugs boost the concentrations of two other brain chemicals: norepinephrine and serotonin. This group includes selective serotonin reuptake inhibitors (often used as antidepressants), norepinephrine reuptake inhibitors, serotonin/norepinephrine reuptake inhibitors, and tricyclic antidepressants. However, doctors tend to avoid tricyclic antidepressants because of their possible side effects, including dry mouth, changes in heart rate, and confusion, among others. Overdoses can cause a serious reaction that includes these side effects plus heart and brain toxicity.

Serotonin syndrome, a potentially life-threatening condition with symptoms such as agitation, sweating, and muscle spasms, can occur with the use of medications that affect serotonin. These symptoms can occur if a patient takes too much of one medication or combines several medications that affect serotonin, so it’s essential to avoid prescribing multiple serotonergic agents to a single patient.

Sodium oxybate is a unique medication that has received FDA approval for treating both excessive sleepiness and cataplexy. Its exact mechanism of action is unclear, but it is thought to work through a receptor for the brain chemical GABA. However, its high salt content should be noted.

Treatment of type 1 narcolepsy in children is slightly different from adults. For excessive sleepiness, stimulants such as dextroamphetamine-amphetamine or methylphenidate are often the first-line treatment instead of wakefulness-promoting agents. For cataplexy in children over seven, sodium oxybate is typically the first choice. For children under seven who need treatment for cataplexy, certain REM sleep-suppressing drugs may be used.

Cataplexy is a condition almost exclusively linked to type 1 narcolepsy. There are very few other conditions that show the same symptoms, making it fairly easy to diagnose. However, there is a condition called “Pseudocataplexy”, part of a larger disorder known as conversion disorder, where individuals experience symptoms similar to cataplexy. In these cases though, the other key symptoms of narcolepsy are usually not present.

In rare cases, cataplexy may accompany lateral hypothalamic lesions, which are involved in the development of secondary narcolepsy. These lesions could be linked to things such as:

Arteriovenous malformations (abnormal connections between arteries and veins)
Cerebrovascular accidents (strokes)
Inflammatory processes (the body’s response to harmful stimuli)
Neoplasms (tumors)

However, in these cases, it’s likely that other neurological problems would be noticeable. This is because the damage usually isn’t just limited to the hypothalamus, a region of the brain.

What to expect with Cataplexy

For a doctor to diagnose narcolepsy type 1, it’s essential for patients to effectively communicate their symptoms. Narcolepsy type 1 is a neurological disorder that can’t be cured, but there are several treatment options available that can manage its symptoms.

In patients who have narcolepsy with cataplexy (a condition where strong emotions cause a person to suddenly feel weak or fall down), there are various drugs to help manage the symptoms of cataplexy and excessive sleepiness during the day. The only exception is a drug called sodium oxybate, which is used to target all symptoms of narcolepsy type 1.

Possible Complications When Diagnosed with Cataplexy

Narcolepsy’s most severe symptom, cataplexy, can lead to total collapse. This collapse can cause harm to the body’s musculoskeletal system and may result in bleeding within the skull. The severity of injuries from a cataplectic fall can be influenced by several factors.

Factors Influencing the Severity of Fall Injuries:

Age of the individual
The type of surface they fall on
The height from which they fall
Existing bone diseases
Objects around them at the time of fall

Preventing Cataplexy

People who have type 1 narcolepsy should understand that they have a higher chance of having accidents while driving due to sleep-related symptoms. It’s important for them to avoid alcohol and recreational drugs as these substances can make their symptoms worse. Patients, as well as their parents, should be encouraged to maintain a healthy diet and participate in daily exercises.

When taking part in activities involving water, these patients should wear a life jacket for safety. It’s also recommended that they do these activities with a partner who knows about their medical condition. This allows for an extra safety measure and quick response if something goes wrong.