Although narcolepsy without cataplexy happens often, Cataplexy almost never occurs without narcolepsy, except in the rare case that is the result of a major brain trauma.
Some drug therapy and lifestyle changes can take care of mild symptoms of narcolepsy and without cataplexy, it can be managed easily enough to maintain a normal life.
What is Cataplexy?
Cataplexy is exclusively a symptom of narcolepsy that is experienced by seventy percent of the 3 million people who suffer from narcolepsy worldwide.
Narcolepsy with cataplexy has its own biomarker. That is, there is a specific, measurable trait that is exhibited by most narcolepsy patients with the symptom of cataplexy. The peptide hypocretin-1, which is derived from the hypothalamus in patients with healthy sleep patterns and a narcoleptic without cataplexy, tends to be deficient in cataplectic. This unique feature has earned Narcolepsy with Cataplexy its own separate diagnosis from the narcolepsy without cataplexy.
Most Narcolepsy symptoms can be seen as a disassociated part of REM sleep. Cataplexy is no different. One of the things that happen during REM sleep is that the body’s muscles lose tone and go limp. Cataplexy is the same phenomenon, during wakefulness.
Hypocretin-1 plays a major role in the sleep/wake cycles of the brain. A deficiency manifests itself in the symptom of cataplexy. There is no correlation between hypocretin-1 deficiencies without cataplexy in narcolepsy, making it an unreliable test for narcolepsy without cataplexy.
Cataplexy is the uncontrollable loss of muscle tone. It can be a minor annoyance or a major event. Either way, it can be embarrassing to the person suffering from it. Cataplexy episodes are triggered by strong emotional response. Anger and robust laughter are at the top of the list.
A cataplectic episode can range from unnoticeable to a serious event.
- Mild Cataplexy
When cataplexy is mild, it can go unnoticed. Perhaps the patient experiences a little clumsiness or trips. It can manifest in a single tiny muscle group, usually in the face. A droopy eyelid or momentary slackening of the jaw can be from cataplexy. Cataplexy can last as little as a few seconds, hardly giving it enough time to register as ever having happened at all.
- Severe Cataplexy
Cataplexy can last for over an hour. It can affect the arms or legs. It commonly affects the neck, resulting in the head suddenly falling forward. In some cases, the entire body is affected. The result is a total collapse. The subject is awake and alert, but unable to move or communicate. It can be frightening to experience, especially when it is new.
Diagnosing narcolepsy without cataplexy and narcolepsy with cataplexy is done much the same way. There are no simple blood tests that reveal the disorder. Even testing for a hypocretin-1 deficiency is a difficult and painful process. It requires a spinal tap to test the spinal fluids. Because of the risk involved, this kind of test is not likely to become a mainstream diagnostic tool.
Without cataplexy, narcoleptic diagnosis involves a detailed history and the use of sleep studies.
Sleep studies include the nocturnal Polysomnogram and the multiple sleep latency test (MSLT). The MSLT is the currently accepted standard in testing and data collection for the diagnosis without cataplexy of narcolepsy.
The nocturnal Polysomnogram and the MSLT are usually done together, consecutively. This way, a physician can get a complete portrait of sleep habits and REM cycles of his or her patient.
The tests are performed in a sleep clinic. The subject has a continuous electroencephalogram (EEG) while they are settled into a room in which to sleep. The EEG and visual monitoring will be used to record everything the patient experiences during a restful state.
For the nocturnal Polysomnogram, they will spend the night. For the MSLT, the next day they will have 5 scheduled naps that are 2 hours apart and will last for 20 minutes.
With the presence of moderate to severe cataplexy, diagnosis is simple, since cataplexy is a narcolepsy specific symptom, if it is present, a diagnosis of narcolepsy with cataplexy is certain to follow. If the episodes are milder, a detailed history and probably the use of journaling the symptomatic episodes may be required as there is no other way to uncover the existence of cataplexy.
Without cataplexy narcoleptic symptoms can often be managed with careful lifestyle changes such as dietary guidelines, strict adherence of bedtimes and scheduled daytime naps. Often, drug therapies that include the use of stimulants and antidepressants are required.
Narcolepsy patients with cataplexy almost always need to be treated with drugs.
Behavioral and Pharmacological Treatments
avoid sleep deprivation
involve the people you associate with regularly
Selective serotonin reuptake inhibitors
The most effective drug treatment for cataplexy and EDS is gammahydroxybutyric acid (GHB). It is the only drug approved by the FDA for the treatment of cataplexy. It is sold in the USA as sodium oxybate and is produces as the drug Xyrem.
Xyrem works on the part of the brain that controls sleep cycles. At higher levels, as a very effective sedative. Promoting deep restful sleep at lower levels it is a stimulant that keeps EDS at bay.
GHB is a highly regulated substance as it is associated with illegal abuse. IT has a high rate of dependence and serious withdrawal symptoms. It induces a euphoric effect that is similar to the street drug ecstasy and it has been used to facilitate date rape. It has the ability to stimulate the human growth hormone and so it has also been abused by body builders. Overdoses of GHB can result in respiratory depression, bradycardia, seizures and death.
The most common side effects of Xyrem are:
- nausea and vomiting
- urinary incontinence
Sodium Oxybate needs careful consideration with patients that have heart concerns as it raises sodium levels in the blood.