What is Narcolepsy?

Introduction

Note: In this document I will be referring to Person/People With Narcolepsy as ‘PWN’, and sometimes I’ll refer to narcolepsy as N.

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This information will be very useful for the undiagnosed person wondering if they have N, friends, relatives, coworkers, etc., of PWN, and PWN who have been previously diagnosed but don’t really understand what they are dealing with. 

Narcolepsy is very difficult for anyone, especially non-narcoleptics, to understand. It is an ‘invisible disorder’, having no obvious physical manifestation other than cataplexy, which is not experienced in the same way by all PWN, or at all. Additionally, there is nothing in the experiences of others to compare it against.  The best they can do is relate to ‘being tired’, which is not what narcolepsy is about. 

I will explain the symptoms and features of narcolepsy, give you a brief explanation of what causes those symptoms, and provide an introduction to the complex issues narcoleptics face as sleepy, fogbound people in a wide awake world.

Narcolepsy Symptoms 

There are four (some people count five) ‘classical’ features of narcolepsy. Not all PWN experience all of these symptoms, nor do they experience any one symptom in the same way over time. They are:

Excessive Daytime Sleepiness (EDS) 

Cataplexy 

Hypnagogic and/or Hypnopompic Hallucinations

Sleep Paralysis

Excessive Daytime Sleepiness (EDS) is the one symptom universally experienced by all PWN.  It is an intense feeling of sleepiness that persists throughout the waking period regardless of how much sleep the person has gotten during the preceding sleep period. EDS is usually accompanied by cognitive deficits and what is commonly described as ‘brain fog’.  PWN often feel physically exhausted as well.

Why would a person who got a full night’s sleep be so sleepy the next day? The simple answer is that PWN have poor quality sleep that is neither restful nor restorative. Without that restful sleep, PWN are very sleepy throughout the day.  

Not sleeping well night after night is a big problem for anyone.  Sleep deprivation is bad for our brains; it affects cognition, hampers our judgment and causes memory issues, to mention only a few of its many ill effects. Poor sleep also has a cumulative negative effect on physical health.  

PWN aren’t the only people who experience EDS; it occurs with many other illnesses and disorders, usually resulting from things like disordered breathing, reactions to radiation or chemotherapy, stress, aging and so on. The poor sleep of PWN, however, is of neurological origin; that is, it develops because something has gone wrong in the brain. The result is the same, but the cause is quite different.

Cataplexy is defined as a loss of muscle tone (atonia) in one or more limbs or group of muscles or even the entire body. The atonia of cataplexy is the opposite of the high muscle tone (hypertonia) of epilepsy.  Where a person experiencing an episode of epilepsy will go rigid or repeatedly flex certain muscles, the muscles of a PWN having a cataplexy episode (sometimes called an ‘attack’) will go limp. They may slur their speech, drop something they were holding or collapse to the floor. The individual may be unable to communicate or may appear to be unconscious. However, they are conscious and fully aware of what is going on but may be unable to move or respond in any way until the episode passes. 

Cataplexy affects approximately 75% of PWN. The onset of a cataplexy episode is said to be triggered; it is a response to experiencing a strong emotion such as fear, surprise, amusement or even love. While EDS can be severely debilitating and can wreak all kinds of havoc in a PWN’s life, cataplexy is most often the cause of physical harm. Broken bones and concussions are common results of stumbling, dropping things or spontaneously collapsing limply to the ground in response to a triggering stimulus such as a baby’s cry, feelings of love or even the punchline to a joke. 

Hypnagogic and/or Hypnopompic Hallucinations are vivid dreams, usually frightening,  experienced as one drifts off to sleep or begins to wake up, respectively. They are most commonly visual in nature but can involve any of the five senses. Although most people in the general population will have experienced some form of these hallucinations once or even many times over a lifetime, for some PWN they can be daily occurrences. 

Sleep Paralysis is a very frightening and disturbing symptom of narcolepsy. The phase of sleep during which we dream is called REM (Rapid Eye Movement) sleep. In order to protect us from acting out with our bodies what we are experiencing in our dreams and possibly injuring ourselves or others, our brains place our bodies in a sort of ‘lock down’ that prevents us from moving. Most people have had the experience, for example, of waking from a nightmare in which they are trying to scream but can’t get the sound out. That is a brief moment of sleep paralysis. In the sleep paralysis of narcolepsy, the brain gets ‘stuck’ in the physical lockdown of REM sleep even though the PWN has become awake, leaving them aware that they aren’t asleep yet they are unable to move or cry out, all the while experiencing often terrifying hallucinations. 

Features of Narcolepsy 

In addition to having the specific symptoms outlined above, narcolepsy can be characterized as having a number of features. Symptoms are ‘official’; they are the precise medical description of narcolepsy. Features are manifestations of narcolepsy that, although acknowledged in medical literature, have not gained the status of symptoms.

Interrupted sleep 

During a typical sleep episode of approximately 7 hours consisting of 3 or 4 sleep cycles, the healthy brain falls and rises in an orderly manner through the various stages of sleep in their correct order and binds those sleep cycles seamlessly together into one continuous sleep episode. In narcolepsy, the individual stages of sleep don’t always occur in the right order and often don’t last for the right amount of time. The result is a hodgepodge of sleep states punctuated by frequent, usually brief, arousals into waking.  PWN typically get too much REM (dreaming) sleep and not enough restful and restorative Non-REM (NREM) sleep. At the conclusion of the sleep episode a PWN may not recall having woken up many times, having just gone right back to sleep each time.  This unbalanced, interrupted and unrefreshing sleep contributes greatly to the development of EDS.

Micro sleep 

A very sleepy person who is sitting quietly or doing something boring might ‘blink out’ into actual sleep, often waking up only moments later. It can happen to anyone; many who have driven a car while drowsy will have experienced this.  A micro sleep doesn’t come upon a PWN with no warning; before having such an experience the PWN will already be feeling quite sleepy.  However, unless they have sufficient awareness that they are drifting off and can somehow rescue the situation by actively breaking away by moving about or doing something different, a PWN in such a situation will fall briefly into a micro sleep. It is possible to experience multiple sequential micro sleeps before surrendering unwillingly to full-fledged sleep. 

Automatic behavior 

Automatic behavior is what happens when a person is engaged in an activity while fighting a losing battle to hold on to being awake. The person will continue to perform the activity, albeit defectively, stopping only when they either enter actual sleep or snap back into wakefulness. The classic example given for this is a person taking notes at a lecture or a meeting. As the sleep pressure builds and they begin to drift away from wakefulness, they keep writing.  When they return to wakefulness they find their notes, which they continued writing automatically, to be illegible scribble. While automatic behavior can happen to anyone who is sufficiently sleepy, it is especially common in narcolepsy, where a PWN can sometimes spend hours or even an entire day ‘stuck’ in a twilight state between arousal and sleep. 

Hallucinations 

PWN routinely experience hallucinations. They can occur in any of the five senses, but not every PWN will experience all of them or even any of them. It is very easy to misunderstand this subject, so please be sure to note the following: 

The kind of hallucinations we are talking about are not in any way like those associated with various psychiatric illnesses or hallucinogenic drugs. Nor are they like the intense, nightmarish hallucinations of sleep paralysis.  We don’t inhabit alternate realities or experience visions. We don’t hear clearly spoken words or feel our limbs being made to move by an unseen force. What we are talking about are generally momentary misfires of the sensory processing areas of our brains.  Most people in the general population experience these things from time to time but not very often, so they just shrug them off and forget that they ever happened, if they even notice them at all. PWN have such experiences more often and a bit more noticeably, so they tend to be more aware of them.

The following list of examples is far from comprehensive.

In general, the visual hallucinations of narcolepsy are in the way of fleeting glimpses out of the corners of our eyes of things that aren’t there.

Anecdotal evidence tells us that the auditory hallucination (something you hear) most commonly experienced by PWN is thinking that they heard someone call their name or say something indiscernible from a distance. Another commonly reported auditory hallucination is becoming aware of a radio (or TV, etc.) playing in the distance but being unable to make out what the announcer is saying or what the music is. 

Olfactory (sense of smell) hallucinations are quite common in narcolepsy. An olfactory hallucination almost universally experienced by PWN is smelling something burning.  An exceptional quality of olfactory hallucinations that they can linger for days. 

Tactile (sense of touch) hallucinations can include feeling something like a non-existent bug crawling on your arm, or feeling that the floor is vibrating or the bed is shaking. 

The last category of sensory hallucinations is gustatory (sense of taste); a person will briefly have the taste of a particular flavor in their mouth. These are apparently quite rare.

What is Narcolepsy?

I have listed the symptoms and features of the disorder, but that doesn’t say anything about what narcolepsy actually is. To be honest, narcolepsy is complicated. A simple explanation wouldn’t actually be very simple. Nevertheless, most PWN, or parents or significant others to PWN, are generally very insistent about wanting to know how they came to have it. What causes it? What’s going on in my brain? So, I will offer a brief explanation.

What causes narcolepsy

Narcolepsy accompanied by any degree of cataplexy is called type 1 (NT1).  Nearly all researchers and clinicians agree that NT1 arises after the body’s immune system mistakenly kills all or most of a small population of specialized brain cells that produce a peptide called orexin (also known as hypocretin). When about 95% of these orexin neurons are destroyed, cataplexy develops. 

Narcolepsy without cataplexy is called type 2 (NT2). There is much debate about the extent to which orexin neuron death contributes to the development of NT2 or even if it is a factor at all. It is generally believed that some degree of orexin neuron death below the 95% threshold results in  NT2. Some research suggests that sustaining a traumatic brain injury can cause the development of NT2 by affecting dopamine transmission. Since only about 25% of PWN have NT2 much less research has been done in that area.  The question remains open.  

The bottom line is that you probably came to have narcolepsy because you had an illness, often involving a high fever (the flu, strep throat, etc.) or some other insult to the body such as a concussion and your immune system was activated, got confused and attacked and killed or somehow disabled some or all of your orexin neurons.

When brain cells die, they don’t grow back. True, the brain can to some degree rewire itself to make up for the now-missing neurons, but orexin neurons are quite specialized and not all of their functions can be picked up by other neurons. I don’t mean to shock you, but there is no way around this: narcolepsy is the visible result of brain damage, and because it is brain damage, it is incurable. 

The symptoms of N can be somewhat ameliorated by pharmaceutical means.  I’m not going to address that here for two reasons. The first is that pharmaceutical therapy for N is something for the patient or care provider to discuss with the treating clinician. The second reason is that as of this writing the range of available treatments is in flux, with both revised and additional ‘traditional’ coming on line and an entirely new class of drugs waiting on the horizon.  

In addition to drugs, good sleep hygiene, scheduled naps, accommodations in the workplace, etc., can all be helpful.  I can not understate the importance of the compassionate support of family, close ones, friends and workmates. 

Learning the science behind narcolepsy can be very interesting for those who are able to pursue such research on their own.  To that end, as I continue to redevelop this website I will later on add some links and some advice on how to do that.  In the interim, there are many N support groups on FaceBook that you can participate in through which you can learn a lot. Be careful of what you read on the internet; not everything is accurate.

The Hard Part 

The behavioral consequences of narcolepsy 

Orexin neurons regulate or modulate many cognitive functions and behaviors including memory formation, fear and risk determination, reward and addiction, and stress response. Without the participation of orexin neurons those processes become dysregulated. 

There are countless many ways that a PWN can appear to be ‘a bit off’. The slurred speech of someone suffering a mild cataplexy episode might be interpreted as drunkenness or drug use, as falling asleep at an inappropriate time or in an inappropriate place might also be. The grogginess and brain fog of EDS often leaves narcoleptics with little presence of mind. They might miss social cues or misunderstand the questions or directions of others, responding oddly as they blunder about their lives in a bleary haze. 

Because PWN are often clueless as to what is going on around them they can have difficulties with interpersonal relations. They often appear to others as looking quite sad because most people can’t tell the difference between a sleepy face and a sad one, so they are dismissed as depressed.  Narcoleptics are often said to be angry but they’re not; they’re simply a bit cranky at times, as you would be if you were that sleepy. Often PWN are accused of being lazy, but that’s not the case; apart from being ‘crippled’ with regard to certain motivated behaviors, they are often just too sleepy to get up and do anything. Poor judgment is common. PWN often don’t succeed at education or employment. They don’t do well at social events, either. Family outings fill many with dread. They aren’t even good at telling you that they love you. The list goes on and on. 

Some PWN are fortunate enough to have an early diagnosis and grow up in a supportive environment. Some find an enduring and supportive spouse. Some find careers that allow them the flexibility to cope well with their illness. Many, however, have a very different story to tell. While reliable statistics are not available it is well understood that the divorce rate among PWN is much higher than the national average. Studies have shown that the suicide rate among PWN is many times greater than that of the general population. 

Unemployment, poverty and homelessness often accompany PWN as they struggle through their fogbound and catastrophic lives, wandering from one train wreck to the next. 

As difficult as narcolepsy is for a person to have, for many that difficulty is compounded by the long time it takes to get diagnosed and get proper treatment. Today, a greater awareness of narcolepsy among clinicians and PWN alike has contributed to reducing the length of the diagnostic journey, although a time-to-diagnosis in excess of 10 years is still common. Older PWN often went 40 years or longer before they finally learned what was wrong with them and why they had so much trouble in their lives. 

In Conclusion 

Narcolepsy is an enormously complex and mostly invisible disorder which can be catastrophically destructive to the lives of those who suffer with it.  It is a disorder about which most people, including most clinicians, know nothing, and where much of what people think they know is wrong.  Hopefully after reading this you will have a better understanding of yourself or someone close to you.