What Is Narcolepsy With Cataplexy? Here's How Sleep Medicine Experts Explain the Disorder
There's no doubt that excessive daytime sleepiness is the defining trait of narcolepsy. However, there's another prevalent symptom that's important to discuss: cataplexy.
About one in 2,000 people in the US have narcolepsy, or roughly 200,000 Americans, says the nonprofit Narcolepsy Network. Around 70% of those people experience cataplexy, per a review published by the US National Library of Medicine (NLM).
Among children, fewer than one in 100,000 are believed to have narcolepsy; the proportion exhibiting cataplexy is also 70%, according to the Cleveland Clinic.
What is cataplexy?
Cataplexy is essentially a sudden loss of muscle or strength in one or more parts of the body. The person remains conscious during a cataplexy episode, and it typically lasts less than two minutes, Richard Bogan, MD, a medical officer at SleepMed, Inc., and associate clinical professor at the University of South Carolina School of Medicine and the Medical University of South Carolina in Charleston, tells Health.
Let's back up for a second. There are two primary forms of narcolepsy, types 1 and 2. The former type is where cataplexy comes into play.
A person receives a diagnosis of type 1 narcolepsy if they have cataplexy episodes or low hypocretin-1 levels, a chemical that helps regulate wakefulness, according to the National Institute of Neurological Disorders and Stroke. In contrast, a person with type 2 narcolepsy may have similar symptoms to people with type 1—albeit often milder—but does not experience cataplexy or have low hypocretin levels. (Sometimes people may be classified as having type 1 if their hypocretin levels are low, even if they don't have cataplexy, says the American Academy of Sleep Medicine.)
While a majority of people with narcolepsy experience cataplexy, it may not present right away. "Generally, in most narcolepsy cases, excessive daytime sleepiness or sleep attacks occur first," Manjamalai Sivaraman, MD, FAASM, a sleep medicine specialist and neurologist at the University of Missouri, tells Health. For those who do develop cataplexy, it may not be evident for several more years.
What causes cataplexy?
Experiencing a strong feeling can be the thing that sets off a cataplexy episode, per the Mayo Clinic.
"Cataplexy presents after an emotional trigger, which can be a positive emotion such as laughter or happiness, or a negative emotion such as sadness or grief," Steven Thau, MD, division chief of the Pulmonary and Sleep Medicine Department and the medical director of the Sleep Center, Respiratory Therapy and the PFT Lab at Phelps Hospital/Northwell Health, tells Health.
Though cataplexy is regularly associated with narcolepsy, it is not unique to it. A 2019 review from Sleep Medicine reports about 30% of cases occur due to disorders other than narcolepsy, such as Niemann-Pick Disease Type C, Angelman Syndrome, and Norrie Disease. It can also occur as a side effect of drugs such as lamotrigine and clozapine.
What are the symptoms of cataplexy?
How one person experiences narcolepsy with cataplexy may not look like someone else's episodes. "Presentation differs widely among people with narcolepsy, ranging from sporadic partial attacks triggered by laughter to frequent, complete collapse brought about by a variety of emotions," says Dr. Bogan. The latter is less common.
According to Dr. Bogan, symptoms of cataplexy include:
- Knees buckling
- Head dropping
- Face Sagging
- Slurred speech
Patients sometimes describe the experience as akin to "melting," he explains.
At times people may be aware of their surroundings but unable to react to them in any way, adds Dr. Sirvaraman.
Cataplexy can present differently in children. According to Dr. Sivaraman, they may also experience:
- Tongue protrusion
- Drooping of the eyelids
- Jaw slackening
- Facial grimacing
- Chewing movements
How is cataplexy treated?
Once diagnosed, it's important to speak with a medical professional familiar with narcolepsy with cataplexy, such as a sleep specialist or neurologist, about preparing for and mitigating risks (such as driving) during cataplexy episodes.
While there is no cure for cataplexy, or narcolepsy as a whole, behavioral changes and medication can go a long way towards mitigating symptoms.
For patients with cataplexy, medications are the go-to treatment, reports the NLM. One such medication is pitolisant, a once-daily stimulant approved for the treatment of cataplexy.
Antidepressants are another option for managing cataplexy. According to Mayo Clinic, selective serotonin reuptake inhibitors (SSRIs), serotonin and norepinephrine reuptake inhibitors (SNRIs), and tricyclic antidepressants have all shown to help with cataplexy.
Xyrem and Xywav are two nighttime medications available for anyone seven years or older that promote sleep and treat cataplexy. Both are taken once before bed and again a few hours later. A big difference between the two is that Xywav has a much lower sodium content. "This reduction in sodium is meaningful, as Xyrem carries warnings about its high sodium content, and people with narcolepsy have an increased prevalence of developing certain comorbidities, such as hypertension and cardiovascular disease," says Dr. Bogan.
If your schedule allows for it, Dr. Thau and Dr. Sivaraman stress the importance of scheduling naps. Taking 15 to 20 minutes during the day to sleep is "refreshing and always beneficial in narcolepsy patients," says Dr. Sivaraman.
Other potentially helpful habits to adopt, per Cleveland Clinic and others, include:
- Going to bed and waking up at the same time every day
- Exercising regularly, but not within 3 hours of going to bed.
- Skipping caffeine, alcohol, or smoking within a few hours before you plan on sleeping.
- Relaxing before bedtime
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