With more experience, people with narcolepsy can learn to better control their cataplexy, but that doesn’t necessarily mean their sleep disorder has become less severe.
As people age, they may be more likely to fall and injure themselves, but patients who suffer from cataplexy—a symptom of narcolepsy that causes loss of muscle tone—have a lifetime to learn about how to stay safe when their bodies fail them.
Since cataplexy is triggered by strong emotions, some patients can be taught how to block out their feelings through meditation techniques or by avoiding triggers, says Lois E. Krahn, MD, a psychiatrist at the Mayo Clinic. So while it may appear like their symptoms are improving with age, they might just be learning to cope better with time.
In some instances, environmental factors can profoundly impact a person’s symptoms. A death of a spouse late in life can trigger severe cataplexy after years without any episodes. After someone retires, they might realize that the stress of work was a significant factor in worsening their cataplexy, says Jed Black, MD, an adjunct professor at Stanford University’s Center for Sleep Sciences and Medicine.
The variability among narcoleptic patients with cataplexy is wide, but the emotional triggers usually remain the same as people age, says Black. Many people report that their cataplexy becomes less severe over time, but these cases could be attributed to changes in behavior or medication compliance, and it’s still debated whether there is a natural evolution of cataplexy over a person’s lifetime.
According to Black, it’s challenging to know what to expect as people age. There will be some patients who will never have a full attack, just experiencing partial paralysis of isolated parts of the body. This could impact the muscles in the face or the neck. The eyes may partially close, their jaws can become slack, and speech may become slurred. In others, only the upper body may be affected, the head and arms may collapse, while the person remains standing.
“When you read in the textbooks about cataplexy, you read about voluntary muscle weakness that is equal on both sides of the body and it is often described as producing a complete collapse,” says Black. “A lot of patients can experience that, but some individuals can have multiple attacks per hour; others can have one every few months or one a year, or less than that. There can be waxing and waning and it has been mischaracterized by some as cataplexy improving over time.”
If someone is going to develop cataplexy, the majority will develop it within a few months to weeks after the development of narcolepsy.
The typical age of onset is in childhood or adolescence. Symptoms usually develop between ages 10 and 20.1 Half of all patients with narcolepsy have symptoms before the age of 15. “We can see symptom onset occurring very early. It can occur in babies as soon as they are able to crawl, which then allows the identification of cataplexy, but it is rare that it is picked up that early,” says Black.
Patients might experience paralysis in some or all of their voluntary muscles. The paralysis can last just seconds or minutes. It can happen several times throughout the day or might vanish for years at a time, says Krahn.
In the most severe cases, a person’s body may collapse suddenly or look like a ragdoll, slowly folding to the ground. In its mildest form, cases of cataplexy may not be noticed for decades, says Julea Steiner, MPH, CHES, assistant professor at the University of North Carolina Gillings School of Global Public Health.
It’s important for physicians to recognize this variability, says Steiner, who was diagnosed with narcolepsy at age 35. “I think a very important point is that milder forms of cataplexy, especially in children, are often overlooked by physicians,” she says.
For instance, Steiner says, she was initially diagnosed with narcolepsy without cataplexy because the screening questions focused on severe manifestations of cataplexy.
“It wasn’t until I connected with other people with narcolepsy and started treatment that I realized I had mild cataplexy—knee buckling, losing my grip and dropping things, and a slight loss of muscle tone in my neck,” she says. “I thought I was just clumsy.” It took 26 years from symptom onset for Steiner to receive a correct diagnosis.
Black says that cataplexy in children can be especially hard to detect because it often looks different than the full paralysis more commonly associated with cataplexy. These children can easily be misdiagnosed with neurological conditions. “To the untrained eye, it looks quite different, but ultimately the same fundamental process of muscle weakness is going on,” he says.
Narcoleptic children with cataplexy can appear to be uncoordinated when they are walking. Their bodies might lean in a certain direction. It may look like they don’t have full control of their movement. They may have partial weakness in various parts of the body that can occur simultaneously, can come and go throughout the day, or can be constant. “They can’t perform athletic activities. Purposeful activities are really challenging for them,” says Black.
Cataplexy in adults typically involves paroxysmal episodes of loss of antigravity muscle tone while the childhood phenotype consists of a complex movement disorder with jaw opening, eyelid drooping, head rolling, or tongue thrusting movements, also called cataplectic facies, according to an article published in the journal Nature and Science of Sleep.2
The loss of muscle tone in cataplexy looks similar to the paralysis that happens during normal rapid eye movement (REM) sleep, so it’s thought that cataplexy could be caused by REM cropping up during the day when the patient is awake. “Cataplexy is probably a fragment of REM sleep that is showing up where it doesn’t belong during the day,” says Krahn.
When patients receive treatment, often antidepressants or Xyrem (sodium oxybate), symptoms may dissipate entirely, but some people can develop a tolerance to their medication over time, and their cataplexy can return. This can give the false appearance that the condition is getting worse, says Black, who is also senior vice president of Sleep and Central Nervous System Development with the drug maker Jazz Pharmaceuticals, which markets Xyrem.
In general, if a patient’s cataplexy is severe enough to need medication, they should remain on medication for the rest of their lives, he says.
If a tolerance to the drugs develops, clinicians can run that patient through a “drug holiday,” removing them from all medications and allowing the brain to come back to baseline function before reintroducing medication.
Black cautions that this method can result in rebound cataplexy for a few days, in which the patient has one attack after another for the entire time that they are awake. In these cases, physicians should make sure to warn patients to take a week off of work and stay home while they are coming off the drugs.
In some individuals, if it is clear that the treatment is working well, physicians only need to screen for cataplexy severity once per year. “Some patients, you get them titrated up on medication and you see that they are stable and tolerating it well and the medication is working well. After a few months, you might not need to see them more than once a year,” Black says.
By the time someone reaches the later decades of their life, they know their cataplexy well, and usually know how to cope. Krahn says she recently had the experience of walking down the hall in her office with a patient and whatever they were talking about triggered the patient’s cataplexy as they were side by side.
“I reached out to support her; she reached out for support and the wall wasn’t too far away. She knew she had just a little bit of time to brace herself. She knew that she needed to move quickly in order to prevent a fall,” says Krahn. “That’s the kind of knowledge of one’s own body that you gain with time.”
Lisa Spear is associate editor of Sleep Review.
1. Division of Sleep Medicine at Harvard Medical School. Narcolepsy: Symptoms. 2018 Feb 21. Available at http://healthysleep.med.harvard.edu/narcolepsy/what-is-narcolepsy/narcolepsy_symptoms
2. Abad VC, Guilleminault C. New developments in the management of narcolepsy. Nat Sci Sleep. 2017;9:39–57.