What Is Narcolepsy With Cataplexy? Here’s How Sleep Medicine Experts Explain the Disorder

People who have this form of narcolepsy, now known as type 1, experience temporary bouts of muscle weakness.

Narcolepsy, a complex neurological disorder, is characterized by excessive daytime sleepiness, a trait that often defines the condition. However, lurking beneath the surface of this sleep disorder is another prevalent and intriguing symptom: cataplexy. In the United States, approximately one in 2,000 individuals grapples with narcolepsy, translating to roughly 200,000 Americans, according to the nonprofit organization Narcolepsy Network. Shockingly, about 70% of these individuals experience cataplexy, as reported by the U.S. National Library of Medicine (NLM). Even among children, where narcolepsy is rarer, the proportion afflicted by cataplexy remains at 70%, as revealed by the Cleveland Clinic.

Deciphering Cataplexy: What Is It?

Cataplexy is essentially a sudden, transient loss of muscle tone or strength in one or more regions of the body. Intriguingly, during a cataplexy episode, the person remains fully conscious, and these episodes typically endure for less than two minutes, as described by Dr. Richard Bogan, a medical officer at SleepMed, Inc., and an associate clinical professor at the University of South Carolina School of Medicine and the Medical University of South Carolina in Charleston.

Let’s take a moment to clarify the two primary categories of narcolepsy: type 1 and type 2. The presence of cataplexy is a pivotal factor distinguishing between the two types.

A diagnosis of type 1 narcolepsy is conferred upon an individual when they exhibit cataplexy episodes or possess low levels of hypocretin-1, a neurotransmitter crucial for regulating wakefulness, according to the National Institute of Neurological Disorders and Stroke. Conversely, type 2 narcolepsy shares many symptoms with its type 1 counterpart but typically features milder manifestations and notably lacks cataplexy episodes or reduced hypocretin levels. However, it’s worth noting that individuals with low hypocretin levels, even without cataplexy, may sometimes be classified as having type 1 narcolepsy, as suggested by the American Academy of Sleep Medicine.

While a substantial portion of narcolepsy patients experiences cataplexy, it may not manifest immediately. Dr. Manjamalai Sivaraman, a sleep medicine specialist and neurologist at the University of Missouri, explains that in most cases of narcolepsy, excessive daytime sleepiness or sleep attacks precede the emergence of cataplexy. Furthermore, for those who do eventually develop cataplexy, its onset may be delayed by several years.

Triggers and Underlying Causes of Cataplexy

Cataplexy is often incited by strong emotional responses, according to the Mayo Clinic. These episodes can be precipitated by a gamut of emotions, encompassing both positive sentiments like laughter and happiness and negative feelings such as sadness or grief, as highlighted by Dr. Steven Thau, 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.

While cataplexy is a hallmark of narcolepsy, it is not exclusive to this condition. A 2019 review in Sleep Medicine reported that approximately 30% of cataplexy cases can be attributed to factors other than narcolepsy. These factors encompass various disorders like Niemann-Pick Disease Type C, Angelman Syndrome, and Norrie Disease. Additionally, cataplexy can surface as a side effect of certain medications, including lamotrigine and clozapine.

The Multifaceted Spectrum of Cataplexy Symptoms

It’s imperative to recognize that the manifestation of cataplexy symptoms can vary significantly among individuals. Dr. Bogan underscores that cataplexy presentations span a broad spectrum, encompassing sporadic, partial attacks triggered by laughter to more frequent, complete collapses incited by diverse emotional states. The latter, involving a complete loss of muscle control, is less common.

Dr. Bogan delineates common symptoms of cataplexy, which may include:

• Buckling of the knees
• Dropping of the head
• Sagging of the face
• Slurred speech
• Falls
• A sensation of “melting” as described by some patients

Additionally, individuals may occasionally remain cognizant of their surroundings during a cataplexy episode yet find themselves unable to react to their environment in any way, as elucidated by Dr. Sivaraman.

In children, cataplexy can manifest differently, featuring symptoms such as tongue protrusion, drooping eyelids, slackening of the jaw, facial grimacing, and chewing movements, according to Dr. Sivaraman.

Nurturing Hope through Diagnosis and Treatment

While cataplexy poses unique challenges, once diagnosed, individuals can seek guidance from medical professionals well-versed in narcolepsy and cataplexy, including sleep specialists and neurologists. These experts can provide valuable insights into managing the condition and mitigating associated risks, such as those related to driving during cataplexy episodes.

Although cataplexy remains incurable, both behavioral modifications and medications have proven effective in alleviating its symptoms.

Medications for Managing Cataplexy

Patients grappling with cataplexy frequently turn to medication for relief, according to the NLM. Among the available treatments is pitolisant, a once-daily stimulant approved for cataplexy management.

Antidepressants are another viable option for cataplexy symptom control. The Mayo Clinic reports that selective serotonin reuptake inhibitors (SSRIs), serotonin and norepinephrine reuptake inhibitors (SNRIs), and tricyclic antidepressants have all demonstrated efficacy in managing cataplexy.

For individuals aged seven and above, two nighttime medications, Xyrem and Xywav, offer both sleep promotion and cataplexy treatment. These medications are administered once before bedtime and again a few hours later. Notably, Xywav features significantly lower sodium content compared to Xyrem, a meaningful distinction given the latter’s association with warnings regarding high sodium levels. People with narcolepsy exhibit an increased risk of developing comorbidities such as hypertension and cardiovascular disease, making sodium reduction an important consideration, as emphasized by Dr. Bogan.

Adopting Lifestyle Changes

In addition to medication, lifestyle modifications play a pivotal role in managing cataplexy. Dr. Thau and Dr. Sivaraman emphasize the importance of incorporating scheduled naps into one’s daily routine. Short, 15- to 20-minute daytime naps can offer a refreshing respite and prove highly beneficial for individuals with narcolepsy.

Furthermore, adopting a consistent sleep schedule, with fixed bedtime and wake-up times, contributes to symptom management. Regular exercise, while beneficial for overall health, should be avoided within three hours of bedtime to prevent disruptions to sleep. Abstaining from caffeine, alcohol, or smoking a few hours before sleep can also enhance sleep quality. Engaging in relaxing activities before bedtime can help calm the mind and promote more restful sleep.

In conclusion, narcolepsy and its associated symptom of cataplexy present complex challenges to those affected. Understanding the condition, its triggers, and the array of treatment options available can provide individuals with narcolepsy and their healthcare providers valuable tools for managing symptoms and improving their quality of life. Collaboration with healthcare experts, adherence to prescribed medications, and the adoption of