Recognizing Cataplexy and Narcolepsy Symptoms

The symptom, Cataplexy and Narcolepsy are often experienced together. Narcolepsy is a chronic sleep disorder characterized primarily by EDS or excessive daily sleepiness. Narcolepsy affects just a small percentage of the population. About 60 to 70 percent, of patients with Narcolepsy show symptoms of cataplexy.

Passing quickly from a state of wakefulness into REM or rapid eye movement sleep or experiencing distinct REM events during the day are considered symptoms of a neurological disorder. Narcolepsy’s cause and origin is still largely unknown, although data from studies conducted on humans and animals suggest that cataplexy and narcolepsy are associated with hypocretin system abnormalities and the loss of active neurons in a person’s hypothalamus.

Cataplexy and Narcolepsy Signs

Usually, excessive daytime sleepiness is the first symptom to appear. It’s characterised by several episodes of sudden sleepiness, lapses and naps during daytime. Although these episodes are most likely to take place in ordinary situations, it may also occur in very unusual situations like when one is eating, walking or even driving; leading to uncontrollable and sudden sleep attacks. The most common symptom of narcolepsy is cataplexy. Generally, it is observed as a dramatic loss of muscle control and tone triggered by sudden strong emotions. Usually, these emotions are positive: surprise, pride, and laughter; however, it may also be negative like anger. Stress, sleepiness, physical fatigue can stimulate factors that can worsen Cataplexy and make the symptom more common.

Cataplexy as a sign of Narcolepsy may be localised, or in worse cases, progressively affect all skeletal muscles; causing a patient to completely collapse on the ground. Commonly affected areas include the face, neck and knees. Fortunately, respiratory muscles cannot be affected. Since Cataplexy as a Narcolepsy symptom can also come in milder forms varying in severity and frequency, diagnosis is sometimes difficult. Mild feelings of weakness, being the sole clinical symptom, may completely pass unnoticed. Probing patients about his/her family members’ experiences involving head drops, sagging facial muscles, weakness of the jaw, slurred speech and knee buckling, is relevant. These narcolepsy and cataplexy symptoms can also vary in duration; ranging from a couple of seconds to minutes. Complete recovery always follows if the person doesn’t injure themselves while affected.

Other Narcolepsy symptoms experienced primarily at night are described in figure 01-02a:

Figure 01-02a – Other Narcolepsy Symptoms Accompanying Cataplexy

Nighttime Cataplexy and Narcolepsy Symptoms

Symptom

Brief Description

Disturbed Nocturnal Sleep

When this symptom is present, it has the tendency to improve in time. It is characterized by the inability or difficulty in having a quality sleep at nighttime, somewhat akin to insomnia. This narcolepsy and cataplexy symptom was underestimated not so long ago, although it is now recognized by most doctors as a major indication of the disorder in patients.

 

Hypnagogic  Hallucinations

This narcolepsy symptom is characterized by vivid hallucinations that may be auditory, tactile, visual or even as multisensory events. These may contain dreamlike elements and occur in the transition between the state of being asleep and wakefulness. The other way around, hallucinations while awake, are referred to as hypnopompic hallucinations.

 

Sleep Paralysis

This narcolepsy sign appears as the lack of movement capability during the same transitions as when the hypnagogic hallucinations occur.

 

 

Effect on a Person’s Life

Narcolepsy symptoms and cataplexy have a great impact on a patient’s quality of life. In the United Kingdom, the biggest problem for patients lies in their limited roles caused by physical difficulties. The time that affected people spend on their work is severely limited by the Narcolepsy signs and cataplexy. Even activities of leisure are shortened since patients usually avoid possible embarrassing situations. Narcolepsy and cataplexy also affect the schooling of many patients, hindering concentration; even making simple daily tasks like cleaning, cooking and watching the children very difficult. Furthermore, it’s been discovered that the accumulation of concern and worry due to narcolepsy and cataplexy can prove harmful to emotional health: greater than 50 percent of all subjects suffering from the disorder are suffering from at least a mild case of depression.

Predisposing Factors and Epidemiology

Narcolepsy’s prevalence is approximately an estimated to be 1:2000 of the American population. It may occur at any age, although it usually happens to people between ages 10 and 25. Also, there are slightly more men affected than women (approximately 1.6/1 relative risk). About 1 to 4 percent of the cases involving narcolepsy and cataplexy have a family member that is also affected. Narcolepsy’s running in families typically shows autosomal-dominant inheritance pattern. Although majority of narcoleptics show non-familial or sporadic narcolepsy, genetic factors remain important. A patient’s first degree relative faces a 1 to 2 percent risk of getting narcolepsy and cataplexy himself.

A main factor to narcolepsy and cataplexy comes from biological factors; according to studies, 25 to 31 percent of all monozygotic twins are prone to the disorder. Also, 90 percent of the patients with narcolepsy and cataplexy show undetectable or low concentrations of hypocretin-1-orexin A in their cerebrospinal fluid. Although this feature is generally specific to narcolepsy and cataplexy, it has also been observed in other CNS (central nervous system) pathologies like Guillain-Barre syndrome.

Recent decades of study have allowed scientist to make progress in understanding the narcolepsy symptoms and cataplexy.  They have identified genes strongly associated with the disorder.  Variant genes in the region of chromosome 6 referred to as HLA complex are strongly although not always, associated with Narcolepsy. This region contains related genes believed to regulate immune function.  It is also known that many of the genetic differences believed to be associated with narcolepsy and cataplexy are not sufficient to cause the disorder. There are many other genes besides those in the HLA complex may contribute to the development of narcolepsy.  Groups of neurons (brain cells) in the brain stem, thalamus, central brain and hypothalamus, interact to control sleep. Many different genes control these neurons’ activity making any of the genes capable of making one develop narcolepsy and cataplexy. Doctors can however look for marked genetic variations associated in narcoleptics to help confirm the diagnosis of narcolepsy. Scientists study narcolepsy in dogs as well and have identified a mutation that causes the disorder in Dobermans.

Cataplexy and Narcolepsy Basics

There are several narcolepsy symptoms and cataplexy is among the most common.   About seventy precent of people who suffer from narcolepsy also report cataplexy as one of their symptoms.   Cataplexy is a condition that although rare, is serious and disabling.  There is no none cure for narcolepsy or cataplexy. But there are treatments that can lessen the frequency and severity of the episodes.

The most common symptom of narcolepsy is cataplexy. When narcolepsy and cataplexy are together, it has its own biological markers that set it apart from narcolepsy without cataplexy. In “Narcolepsy, A clinical Guide” by Meeta Goswami, the predisposition to narcolepsy is looked at in a cross disciplinary fashion with both genetic studies and environmental factors getting equal scrutiny.  Goswami explains that as many advances have been made in recent years in the understanding of narcolepsy and that “in international classifications, narcolepsy with and without cataplexy are now two different diagnostic entries.”

Narcolepsy Diagnosis

Narcolepsy is a chronic sleep disorder; People who have a chronic sleep disorder like narcolepsy have problems sleeping. This nervous system disorder is characterized by the inability to control sleep cycles, extreme daytime sleepiness and a tendency to fall asleep at inappropriate times, like while driving or walking.  Although there are times when an attack is more likely, like after meals, times of impaired health or during high stress situations, a narcoleptic episode can happen at any time.  The disorder itself is not fatal, however, falling asleep while operating Machinery can be. For this reason, Narcolepsy sufferers often seek to control their disorder with drugs and behavior modifications, like scheduled short daytime naps and night sleep insomnia medication.

Cataplexy as narcolepsy Symptom

Experts recognize cataplexy as sign of narcolepsy.  Cataplexy is when a person has a sudden loss of muscle control and becomes weak. Cataplexy attacks are usually triggered by the act of laughter and extreme emotions.  Vigorous laughter is perhaps the most common trigger for a cataplectic attack.  There are several degrees a person can experience cataplexy.  A mild version of the symptom is when just the face goes slack or the eyelids droop.  This can happen for as little as a few seconds.  In extreme cases, control over the entire body is lost and can last for several minutes.  Unlike with plain narcolepsy, Cataplexy is experienced while completely awake, and alert.  So although they may be completely limp, they are aware of everything that is going on around them. This is what makes cataplexy frightening, especially the first time it is experienced.  A similar symptom is experienced in nighttime hours as sleep paralysis.

Protein Deficiency

No one knows what causes narcolepsy, but recent data indicates that the reduction of the Protein, Hypocretin-1 in the hypothalamus may be the cause of cataplexy in narcoleptic patients. A study was conducted in a sleep center in Brazil by Dr. F Coelho, by testing the spinal fluids of narcolepsy patients. It confirms that a reduction Hypocretin-1 is at least a positive biomarker for cataplexy. Patients without Cataplexy did not show reduced Hypocretin-1 levels. This makes it an effective biomarker for Cataplexy but not for Narcolepsy.

Autoimmune Disease Implications

It is well known that trauma can bring on both Narcolepsy and Narcolepsy with Cataplexy. Goswami states that in the genetics studies, there is a suggestion of possible autoimmune basis for the disease when cataplexy is present and hypocretin is deficient, because of the hypocretin cell destruction.  If this proves to be true, then scientists will have a new direction to look and new treatment options to explore.  Other Autoimmune diseases include Multiple Sclerosis and Rheumatoid Arthritis.

Cataplexy and Narcolepsy Symptoms in Children

Cataplexy and narcolepsy signs that develop in school age children are very uncommon and are usually misdiagnosed due to cataplexy diagnosis currently being based at 100% specific on medical history. The impact narcolepsy can have on a child’s life can be devastating. E.D.S. or excessive daytime sleepiness can cause sleep attacks at unusual periods throughout the day and is commonly linked to cataplexy. The child experiencing this will lose control of his or her muscle functions while being awake. This is usually triggered by laughter and the end result is a fall, which can cause physical harm.

There are several narcolepsy signs and cataplexy is only one. Sleepwalking, for instance is a common symptom. Sleep paralysis is another symptom that is often reported. This can be a horribly frightening experience for children, as they are totally aware of their surroundings, but are unable to move or speak. Other symptoms that have been noted are depression of various ranges, obesity and sleep disturbances in which the child may not be able to distinguish reality from dreams.

Treatment for narcolepsy in children is a highly demanding effort, which includes medication as well as non-medical treatment. Correct diagnosis is paramount for children to gain benefits from treatment. The medicines include sodium oxybate, stimulants and antidepressants. The extra treatments include scheduled napping, environmental adjustments and understanding. This strategy can help children who suffer from narcolepsy to attain a meaningful life.

Narcolepsy/Cataplexy Studies

Narcolepsy has been extensively studied, but the exact cause remains a mystery. It has been suggested that excessive daytime sleepiness is a genetic factor, but narcolepsy is rarely found in relatives. Narcolepsy studies point to disorders of the cerebral pathways that control a person’s ability to stay awake or asleep.

Cataplexy, although defined, is not very precise by that definition; meaning that the distinction between typical and atypical cataplexy is not well recorded. Recent studies have been aimed at finding a more distinct definition for cataplexy as well as grading it by levels of severity. The patients included in the study were those with recorded cataplexy histories and hypocretin-1 deficiencies. A questionnaire containing items that covered a wide range of cataplexy symptoms, including triggers, duration, associated aspects and limitation in a person’s daily life, was given to these patients.

The results placed 60% of the patients with spontaneous cataplectic attacks, with 45% of these patients having both partial and complete cataplectic attacks. Of the patients as a whole, only 15% had symptoms lasting more than 2 minutes. Of triggers, laughing ranked lower than anger as a whole, with laughing hysterically triggering more attacks, which showed that intensity of certain emotional states has a direct impact on cataplexy. Of all the muscles affected during cataplectic attacks the jaw and facial muscles showed a higher rate of involvement in partial attacks.

Cataplexy and Narcolepsy: What you Might Not Know

Sleep disorders are extremely common. Millions of Americans experience problems sleeping at least three nights a week and many of these suffers have been diagnosed with sleep disorders like Sleep Apnea and Insomnia. Narcolepsy is a less common sleeping disorder, affecting around 200,000 Americans. Of those 200,000 Americans with Narcolepsy, or possible symptoms of Narcolepsy, around 50,000 of them are undiagnosed. One symptom of Narcolepsy is Cataplexy.

Of the small population of narcoleptic victims in the world, there is a very small subcategory of patients who experience a dangerous complication. Cataplexy is uniquely associated with Narcolepsy, and is thought to plague one in every 3,000 Americans. Worldwide, Narcolepsy with Cataplexy affects two percent of adults.

What is Cataplexy?

Narcolepsy and Cataplexy are odd and dangerous neurological conditions associated with sleep. While it is a rather rare side effect of Narcolepsy, medical professionals have dubbed Cataplexy as a Narcolepsy symptom. In fact, it is the first symptom identified by ten percent of all patients diagnosed with Narcolepsy.

Cataplexy, as a sign of Narcolepsy, is defined by sudden attack on muscle tone, and the resulting loss of muscle control. When an episode of Cataplexy occurs during waking hours, patients are unable to move for the duration of the attack. Immobility usually occurs on both sides of the victim’s body. Most attacks of Cataplexy are relatively short, less than thirty seconds, but extreme cases can occur. In extreme cases of Cataplexy, it is possible for a patient to remain paralyzed for several minutes at a time. Because these attacks usually happen so quickly, they are often overlooked by sufferers and medical professionals.

The loss of muscle tone that occurs during an episode of Cataplexy mirrors REM activity in the brain. The loss of muscle activity that occurs during an attack is almost the same as the interruption that takes place in brain activity when a patient enters the deepest levels of sleep. During the REM stage, a small group of neurons in the brain stem stop moving around and prevents muscle activity. Essentially, it is the same process for Cataplexy patients.

Why is Cataplexy Dangerous?

Cataplexy and its detrimental affects on muscle weakness can range from relatively mild to dangerous and severe. Attacks can range from the loss of muscle tone and slackening of the face; to the dropping of the head, neck, or jaw; to weakness in the knees; and even to total collapse. Attacks can be partial or complete, affecting a variety of different muscular groups. Most patients are in the middle of daily routine activities during attacks of Cataplexy; they are often in standing positions or are holding things in their hands. When a Narcolepsy patient loses all control of their muscular functioning during seemingly mundane routine activities, these activities suddenly become dangerous. Although Narcolepsy patients remain conscious during an episode of Cataplexy, they have absolutely no control over the severity or length of the episode.

Narcolepsy patients who also suffer from Narcolepsy signs and Cataplexy are at a very high risk of being involved in dangerous accidents. Driving, operating machinery, raising children, and performing at work become extremely difficult when you cannot ensure the maintenance of muscle control or wakefulness.

What Causes Cataplexy?

Cataplexy is such a unique and mysterious disease because it is triggered by very odd stimuli. Most cases of Cataplexy are triggered by the patient’s exposure to a strong emotion. Heightened emotional responses like laughing, crying, and shouting can bring on Cataplexy attacks. Although the collapse of muscle tone in response to emotional situations may seem like a laughing matter, it’s anything but funny. Being social means interacting with other people and interactions often include some kind of emotional response that may possibly trigger an attack in Narcolepsy patients with Cataplexy. Emotional triggers can not be easily avoided. When you walk into the grocery store, you can’t plan whether or not strangers will laugh as they walk by you.

What are the Symptoms of Cataplexy?

Narcolepsy symptoms and Cataplexy usually arise in teenage narcoleptics, but can develop at any stage of life. A significant amount of medical research shows that Narcolepsy, and even Cataplexy, are more common in families with members that suffer from the disorder. Keep an especially close eye on teenage narcoleptic patients and the relatives of Narcolepsy sufferers.

There are varying degrees of Cataplexy symptom severity. Some patients experience symptoms so mild that they are hard to even notice. Other patients suffer from severe cases of Cataplexy and often find themselves unable to move or speak. The debilitating symptoms of the sleep disorder can make it difficult to maintain a healthy lifestyle or an acceptable quality of life. Going to school, having a career, raising a family, and staying healthy all become much harder goals to accomplish when you are struggling with an unmanaged medical disorder as severe as Narcolepsy with Cataplexy.

Many patients are too afraid to seek help because they are embarrassed or ashamed of their Cataplexy or situations that were a result of their disorder. Don’t be afraid; if you are struggling with this disorder, or think that you may exhibiting any of the symptoms listed below, seek medical help from a trusted health care provider.

Cataplexy and Narcolepsy Symptoms:

  •  Loss of Muscle Tone- Ranging From Moderate to Severe
  •  Sense of Weakness
  •  Drooping of the Eyelids
  •  Inability to Move, Speak, or Control Their Eyes but Still Conscious
  •  Emotional Triggers
  •  Arm Weakness
  •  Sagging Jaw
  •  Drooping of the Head
  •  Shoulders Slump Over
  •  Speech Becomes Slurred
  •  Vision Becomes Blurry
  •  Knees/Ankles Become Weak or Buckle
  •  Total Collapse

Cataplexy in Narcolepsy Patients can become extremely dangerous to the patients suffering from the rare disorder and the people closest to them. Undiagnosed Cataplexy has a high risk of developing from a mild, over looked disorder to a life-altering accident waiting to happen. If you are experiencing Cataplexy and Narcolepsy signs, or are in fear of developing the disorder, seek medical attention immediately. The sooner your Cataplexy is diagnosed, the sooner it can be treated and managed. Medical professionals, sleep study facilities, and Narcolepsy support groups are ideal resources for seeking Cataplexy information, help, and support.

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