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 chronic neurological disorder affecting approximately one in 2,000 individuals in the United States, is characterized by excessive daytime sleepiness, often the most visible and distressing symptom. However, there exists another less-known yet equally significant hallmark of narcolepsy – cataplexy. This comprehensive exploration delves into the intriguing world of cataplexy, unraveling its definition, causes, symptoms, and potential treatments.

Cataplexy Unveiled: A Sudden Loss of Muscle Control

Cataplexy is a distinctive symptom that sets narcolepsy apart from other sleep disorders. It manifests as a sudden and temporary loss of muscle strength or control in specific parts of the body while the person remains fully conscious. Typically, a cataplexy episode lasts for less than two minutes, during which the individual may be acutely aware of their surroundings but unable to move or react as usual.

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, elucidates that cataplexy episodes are often initiated by intense emotional triggers. These emotions can range from positive feelings like laughter or happiness to negative ones such as sadness or grief. While cataplexy is most commonly associated with narcolepsy, it can also occur independently or be linked to other underlying conditions.

Two Types of Narcolepsy: Differentiating Type 1 and Type 2

To understand cataplexy’s role in narcolepsy, it is essential to differentiate between the two primary forms of the disorder – type 1 and type 2 narcolepsy. Type 1 narcolepsy, which is often associated with cataplexy, is diagnosed when an individual experiences cataplexy episodes or has low hypocretin-1 levels. Hypocretin-1 is a neurotransmitter that regulates wakefulness. On the other hand, type 2 narcolepsy may display similar symptoms to type 1, albeit usually milder, but does not involve cataplexy or low hypocretin levels. In some cases, individuals with low hypocretin levels, even in the absence of cataplexy, may be classified as having type 1 narcolepsy.

The Spectrum of Cataplexy Symptoms

Cataplexy does not follow a uniform pattern in all individuals with narcolepsy. The way it manifests can vary widely among patients. While some may experience partial attacks triggered by laughter, others may encounter complete collapses induced by various emotions. Dr. Bogan highlights the following symptoms associated with cataplexy:

1. Knees buckling: A sudden weakness in the knees, leading to buckling or giving way.

2. Head dropping: The head may suddenly droop forward.

3. Face sagging: Facial muscles may lose control, resulting in sagging features.

4. Slurred speech: Speech may become incoherent or slurred.

5. Falls: Complete loss of muscle control may cause individuals to fall.

6. Feeling of “melting”: Some describe cataplexy episodes as feeling like they are melting, as if their body is losing its structural integrity.

In children, cataplexy symptoms can also encompass tongue protrusion, drooping eyelids, jaw slackening, facial grimacing, and chewing movements. It’s important to note that the severity and frequency of these symptoms can vary from person to person.

Navigating Cataplexy: Diagnosis and Treatment

Diagnosing cataplexy often requires consultation with medical professionals well-versed in narcolepsy and its associated symptoms, such as sleep specialists or neurologists. Once diagnosed, individuals should work closely with healthcare providers to develop strategies for managing cataplexy, especially in situations that pose risks, like driving.

While there is currently no cure for cataplexy or narcolepsy, several treatment options can significantly alleviate its symptoms:

1. Medication: Medications are a cornerstone of cataplexy treatment. Pitolisant, a once-daily stimulant, has been approved to manage cataplexy. Additionally, various antidepressants, including selective serotonin reuptake inhibitors (SSRIs), serotonin and norepinephrine reuptake inhibitors (SNRIs), and tricyclic antidepressants, have shown efficacy in cataplexy management.

2. Nighttime Medications: Xyrem and Xywav are two nighttime medications available for individuals aged seven and older. These medications promote sleep and effectively treat cataplexy. Xywav, in particular, offers a lower sodium content, addressing concerns associated with the high sodium content in Xyrem, which can lead to certain comorbidities in narcolepsy patients.

3. Behavioral Changes: Incorporating behavioral changes into daily routines can complement medication-based treatments. Scheduled naps, lasting 15 to 20 minutes, can provide refreshing breaks during the day. Establishing a consistent sleep schedule, engaging in regular exercise (while avoiding vigorous activity within three hours of bedtime), and refraining from caffeine, alcohol, and smoking in the hours leading up to sleep can also contribute to better symptom management.

In conclusion, cataplexy is a distinctive and challenging aspect of narcolepsy, affecting a significant percentage of individuals with the disorder. While there is no cure, a combination of medication and lifestyle adjustments can significantly improve the quality of life for those living with cataplexy. By seeking professional guidance and developing effective strategies for symptom management, individuals with narcolepsy can navigate the complexities of cataplexy and lead fulfilling lives.