All about Cataplexy

Cataplexy is associated with narcolepsy and is rare. Given that only one in every 2,000 people have narcolepsy and only 75% of these have cataplexy, it’s not at all common. As many as 30,000 people in the UK have narcolepsy, which means that approximately 22,500 suffer from associated cataplexy. In this article, we’ll tell you everything you need to know about cataplexy.

What is cataplexy?

Cataplexy, from the Greek ‘kata’ and ‘plexis’, meaning ‘down’ and ‘strike’ respectively, is a sudden episode of weakness combined with full awareness and consciousness. It is triggered by a strong emotion like surprise, anger, laughter, crying, or fear. More often, an episode of cataplexy will be triggered by positive emotions, but negative emotions can occasionally be a trigger too.

During a cataplexy episode, the person might collapse totally on the floor or they might feel slightly weak in part of their body. Often, eyesight will become impaired (poor focus or double vision) and speech might be slurred. Usually, hearing is undisturbed.

Unlike seizures or fainting episodes, which also cause tonic immobility, a person who experiences cataplexy will remain aware and conscious throughout the episode. Typically, an episode will resolve on its own after a few minutes.

The term was first used in late 19th-century German physiology to describe tonic immobility, which is also known as ‘playing possum’ due to the witnessed playing-dead behaviour of possums.

What are the symptoms of cataplexy?

Cataplexy is characterised by muscle weakness. This can range from complete paralysis and postural collapse to slight muscle weakness in the face.

A cataplexy attack is brief and lasts anywhere between a matter of seconds and a few minutes. A typical attack might involve jaw-dropping, a weak neck and knee buckling. With a full-blown episode, a person is normally able to avoid being injured as they notice an episode coming on. This means that falls are generally slow.

These attacks resolve without medical intervention and are self-limiting. If a person is comfortable when an attack takes place, they might go into REM sleep, experience hypnagogic hallucinations (‘hypnagogic’ refers to the transition from wakefulness to sleep), or general sleepiness.

A famous example of a cataplectic attack was when Bob Beamon learned he had broken the long jump world record by more than half a metre at the 1968 Olympics.

What are the causes of cataplexy?

Though there are continuing studies into the cause of cataplexy, the majority of people who have the condition have fewer hypothalamic neurons in their brains, likely caused by an autoimmune condition. With an autoimmune condition, the body attacks healthy cells, in this case the cells that produce hypocretin.

Hypothalamic neurons produce a substance called hypocretin (otherwise known as orexin). This is known for regulating arousal and plays a part in transitions between wakefulness and sleep. For this reason, it’s extremely rare to experience cataplexy without also having narcolepsy.

Though we don’t yet fully understand the genetic links between cataplexy and narcolepsy, around one in 10 people who have narcolepsy (type 1) will also have a close family member who has similar symptoms.

Another causal factor is a brain injury, an acquired brain disease or a brain tumour.

When cataplexy isn’t related to narcolepsy, it could be caused by a number of other rare diseases and conditions.

These include:

• NPC – Niemann-Pick type C – This is a rare disease and genetic disorder that is characterised by an inability for the body to transport fatty substances within cells. This means that lipids like cholesterol build up in tissues. There are a number of neurological symptoms associated with NPC, including cataplexy, dementia and cognitive impairment.
• Prader-Willi syndrome – This is a genetic disorder that starts in childhood and leads to problems with eating, development and growth. One defining characteristic is having an insatiable appetite. With Prader-Willi syndrome, people can experience cataplexy from food or from excitement.
• Angelman syndrome – Like Prader-Willi syndrome, this is a genetic disorder. It affects the nervous system and causes speech impairment, problems with balance and movement, intellectual disability and cataplexy.

Rarely, cataplexy can occur as a result of medication. An insomnia treatment called Suvorexant, for example, treats insomnia by blocking hypocretin and thus can cause cataplexy. However, unlike other causes of cataplexy, the episodes will disappear once the medication is stopped.

Who is at risk of cataplexy?

Around 75% of people with narcolepsy have cataplexy symptoms, which means that those with the condition have a high chance of having to deal with cataplexy too. Though it isn’t believed to be inherited, 10% of people with both narcolepsy and cataplexy also have relatives with symptoms.

There are other risk factors too.

These include:

• Autoimmune conditions.
• Brain tumours growing near the areas of the brain that control sleep patterns.
• Brain injuries or head injuries.
• Infections like swine flu.

How are cataplexy and narcolepsy related?

Narcolepsy is a disorder that causes excessive sleepiness, hallucinations and sleep paralysis. There are two types. Narcolepsy type 1 includes cataplexy as a symptom while narcolepsy type 2 does not.

When people have narcolepsy-associated cataplexy, the cataplexy symptoms will usually occur after the start of the sleepiness symptoms. Type 1 narcolepsy has causes that are well understood – the loss of the neurotransmitter hypocretin – but the causes of narcolepsy type 2 are not understood.

How is cataplexy diagnosed?

Diagnosing cataplexy is typically done by examining symptoms for narcolepsy. For narcolepsy to be diagnosed, the patient usually needs to have excessive sleepiness during the day, hypnagogic hallucinations, and sleep-onset paralysis. There is often a multiple sleep latency test conducted to quantify how sleepy a person is during the day. This often means taking short, space-out naps to see how quickly a person falls asleep.

Doctors will typically do a full physical exam and assess a person’s health overall. This is in order to make sure the symptoms are not caused by anything else.

There might also be written evaluations like the Epworth Sleepiness Scale and the Stanford Narcolepsy Questionnaire as well as sleep studies (a polysomnogram) to record what happens in the brain and to the muscles during sleep. Finally, a patient might undergo a lumbar puncture to draw out cerebrospinal fluid (CSF) from around the brain and spinal cord to test hypocretin levels.

A diagnosis of cataplexy will often come with a diagnosis of type 1 narcolepsy.

Diagnosing cataplexy alone is more of a challenge as there is no specific test. Many patients choose to try and film episodes of cataplexy to show a doctor. There’ll also be a discussion and interview with the patient to try and ascertain what triggers the cataplectic events and which parts of the body are affected.

Diagnosing cataplexy in children

Cataplexy in children often presents differently. Symptoms often present in the walking style and gate or facial muscles. Children also don’t tend to be triggered by emotions. However, when children age, their cataplexy mirrors the symptoms of typical adult cataplexy.

How to deal with cataplexy

Cataplexy can be frightening for both those experiencing the condition and those around them.

Here are some ways in which you can deal with cataplexy:

Dealing with cataplexy in yourself

When you’ve had a few cataplectic episodes, you will begin to know when an attack is coming on. This means you can deal with it safely and sit or lie down. It’s a good idea to create safe areas in places you spend a lot of time in, i.e., your home and at work.

If you plan activities like climbing or swimming, you should take extra precautions and not be alone. The same goes for driving. It’s important that all of your relatives and close friends know how to spot cataplexy and what to do if an attack occurs.

At work or school, you should talk to your employer or school administrator so that they can make provision for your condition. This might mean making time for naps to deal with the narcolepsy side of things, for instance. Whenever you’re out and about, you should be vigilant of any terrain or objects that might harm you if you were to fall. This includes sharp edges and heights.

Lifestyle changes are an important part of managing symptoms and there is a link between sleep deprivation and cataplectic attacks. As such, you should always try to have good sleep hygiene. Things like going to bed at the same time each night and getting up at the same time every day are a priority. Weekends should be the same too.

It’s also important to consider your daytime activities and how they impact upon your sleep. Natural light and regular exercise will help you to maintain a good sleep pattern but things like caffeine, alcohol and smoking won’t. Equally, you should avoid eating a large meal just before bedtime.

Bedtime routines aren’t just for babies and young children! Everyone benefits from a good bedtime routine. This might involve half an hour to one hour of winding down time before bed. You should avoid screens during this time and do something relaxing like taking a bath, stretching or reading.

Another thing to be mindful of is being prepared for a situation if there are likely to be strong emotions. You should have a friend with you or keep a chair close by just in case.

Finally, because cataplexy can feel socially isolating and emotionally draining, it’s important to find an appropriate support network. This can be your family and friends, but it’s also a good idea to seek out support groups with fellow cataplexy patients so that you don’t feel so isolated.

Physical aids for dealing with cataplexy

If you’re finding yourself experiencing severe cataplexy, you can get hold of protective devices to help you to manage any dangers that could happen if you fall.

These include:

• Use of a wheelchair – This can provide a safer way of getting around if you’re having more than one attack each week.
• A protective helmet – This can prevent serious head injuries if you were to fall.
• Crutches or a walking stick – This can help you to keep your balance or slow down a fall.
• Orthoses – These can help support your body and joints and prevent falls.
• Alarms – A wearable alarm or a bed alarm can alert people if you’re about to have an attack.

Dealing with cataplexy in someone you know

The best way to support someone with cataplexy is to ask the person how you can help them. They are the best advocates for their condition.

When you know of someone who suffers from cataplexy, it’s a good idea to know how their attacks generally present themselves so you know what to expect. One thing to be mindful of is that the person might be unresponsive but they are completely aware and conscious of everything that is happening. For this reason, always treat someone as if they are awake and aware.

How is cataplexy treated?

Both narcolepsy and cataplexy can be managed and treated by lifestyle changes and medication. Though there is no cure for either condition, you can manage your symptoms.

Cataplexy medications

There are a number of different medications to alleviate the symptoms of cataplexy.

These include:

• Sodium oxybate (brand name Xyrem). This helps sleepiness as well as cataplexy.
• SSRIs (selective serotonin reuptake inhibitors) or other antidepressants like venlafaxine or fluoxetine.
• Tricyclic antidepressants like clomipramine.

If the cataplexy is a result of narcolepsy, patients might be prescribed:

• Modafinil. This helps people to feel less drowsy and more alert.
• Stimulants resembling amphetamines to keep people alert.

Like many medications, there are often side effects. They might cause changes in mood, abnormal heart rhythms and nervousness. They can also lead to addiction.

Changes to a person’s lifestyle can help make cataplexy and narcolepsy more manageable. Unfortunately, you can’t reverse the low level of hypocretin but you can manage the symptoms.

Lifestyle changes

People with cataplexy often make changes to their lives to try and prevent attacks.

Here are some changes people often make to prevent a cataplectic episode:

• Trying not to think about things that induce strong emotions.
• Taking regular exercise.
• Eating healthily and maintaining a healthy body weight.
• Sticking to a strict sleep schedule.
• Decreasing their intake of caffeine and alcohol.
• Taking daytime naps.
• Not operating machinery or driving.

Conclusion

Narcolepsy and cataplexy often interfere with a person’s daily life. The condition can strain relationships and cause problems at work. However, the condition can be well-managed with medication and changes to your lifestyle. Once under control, it’s possible to lower the risk of having an attack while you’re doing something that could be potentially dangerous, like driving.

Anyone who notices symptoms of cataplexy should call their doctor straight away.