Narcolepsy in Children

What is narcolepsy?

Narcolepsy is a neurological (nervous system) disorder that affects the brain’s ability to control sleep and wakefulness. Children with narcolepsy experience excessive sleepiness, which impacts all aspects of their life, including social activities and school performance.

Children with narcolepsy experience:

• Constant excessive daytime sleepiness.
• Ongoing struggles to stay awake.
• Sudden sleep episodes (“sleep attacks”) that occur during any type of activity and at any time of the day.

Who gets narcolepsy?

Narcolepsy is underdiagnosed and misdiagnosed in children, making it difficult to determine how many children have the disorder. However, one estimate is that narcolepsy occurs in slightly less than one in every 100,000 children. The symptoms of narcolepsy have been reported in children as young as five or six, but it is often not diagnosed until adolescence or later. Narcolepsy affects an equal number of boys and girls.

Are there different types of narcolepsy?

Yes, there are two types of narcolepsy:

• Narcolepsy Type 1 (previously called narcolepsy with cataplexy [see symptoms section for definition]). Persons with narcolepsy type 1 have excessive daytime sleepiness plus cataplexy and/or low levels of a chemical in the brain called hypocretin. One unique feature of this type of narcolepsy in children is that those who have it tend to experience rapid weight gain.
• Narcolepsy Type 2 (previously called narcolepsy without cataplexy). Persons with narcolepsy type 2 have excessive daytime sleepiness but do not have cataplexy and have normal levels of hypocretin.

What causes narcolepsy?

Narcolepsy is thought to be related to a disruption in an area of the brain that controls sleep and wakefulness. In many cases, it is thought to be due to a loss of a particular chemical in the brain called hypocretin.

Other possible factors scientists think play a role in narcolepsy include:

• An autoimmune disorder: A person’s immune system attacks the brain cells that produce hypocretin, resulting in a shortage of this chemical.
• Family history: Some persons with narcolepsy have close relatives with similar symptoms.
• Brain injury or tumor: In a small number of patients, the area of brain that controls REM sleep and wakefulness can be injured by trauma, tumor or disease.
• Infections.
• Environmental toxins: Pesticides, heavy metals and secondhand smoke.

What are the signs and symptoms of narcolepsy?

Symptoms of narcolepsy vary during a person’s lifetime. Not all of the following symptoms are present at the start of the disorder or in every single child. The four most common symptoms/signs of narcolepsy are as follows:

• Excessive daytime sleepiness (EDS): This is usually the first sign of narcolepsy in children and occurs in all patients. EDS interferes with normal activities (work, school, home life, social activities) every day. Children have frequent bouts of extreme tiredness, most often during inactive times, such as when sitting in the classroom, reading, or riding in a vehicle. Sleep attacks in young children are nearly constant and they last longer than those experienced in adolescents or adults. In preschool children, afternoon naps can last up to two to three hours, but tiredness returns within one to two hours. One unique sign of narcolepsy in children is consistent napping after the age of five or six. Children with EDS report mental cloudiness, forgetfulness, lack of energy, decreased attention and concentration, and poor school performance. Behavioral issues include irritability, aggressiveness, hyperactivity, social withdrawal and depression.
• Cataplexy: Cataplexy is a sudden, brief loss of muscle tone or strength triggered by stress or a strong emotion, such as laughter (common cause in children), excitement, anger, anxiety or surprise. Cataplexy may be mild, like a brief feeling of weakness in the knees or slackness in the jaw or drooping of the eyelids, to total body paralysis with collapse. Additional features unique to children include facial and/or jaw and eyelid weakness and sticking out of the tongue, plus slurred speech, other abnormal facial movements and expressions. Cataplexy usually lasts a few seconds to several minutes. Cataplexy is seen in about 70% of children with narcolepsy. In young children, cataplexy can be mistaken for clumsiness, seizures, a fainting spell or as an attention-seeking behavior.
• Sleep paralysis: This symptom is the inability to move or speak just before falling asleep or just after waking up. Episodes of sleep paralysis usually go away after a few seconds to a few minutes. Being touched by another person usually causes the paralysis to disappear.
• Hallucinations: These are vivid, dream-like/nightmare events that are difficult to distinguish from reality. They occur just before falling asleep (called hypnagogic hallucinations) or just after awakening (called hypnopompic hallucinations). The "dreams" often involve images or sounds of strange animals or prowlers. The content is generally scary. Many times hallucinations appear in combination with sleep paralysis episodes and are part of the dream (REM) intrusion into wakefulness that occurs with narcolepsy.

Other symptoms of narcolepsy can include:

• Disturbed sleep through the night: This is difficulty sleeping through the night because of frequent awakenings.
• Automatic behaviors: This symptom is described as falling asleep for several seconds but continuing to perform routine tasks, such as writing, without any awareness or later memory of ever doing the task.
• Sudden weight gain: This is another symptom or warning sign of narcolepsy in children. It is seen most commonly early in the course of the disorder. Obesity is present in at least 25% of children with narcolepsy.
• Early onset of puberty.

How is narcolepsy diagnosed?

Narcolepsy is underdiagnosed and commonly misdiagnosed in children for many reasons. Often times the behavioral problems that are seen are thought to be psychiatric conditions. Symptoms of cataplexy are mistaken as normal falls in this age group or symptoms of epilepsy or other neurological disorder. Similarly, it is not uncommon to see children slouched over their desk, appear drowsy or even fall sleep during the school day. These factors make diagnosing narcolepsy in children more difficult. Without specific and unique symptoms, young children with narcolepsy may not be diagnosed for 10 to 15 years, until they reach adolescence or early adulthood.

To begin to make the diagnosis of narcolepsy and to rule out other causes of sleep symptoms, your healthcare provider or sleep specialist will collect a detailed medical history and performs a physical exam. A detailed sleep history is also gathered from the child and from the parents (parents may be needed to help clarify the child’s experience). Sometimes an eight question questionnaire, called The Pediatric Daytime Sleepiness Scale, may also be given to collect information on sleepiness and response to treatment. A history of the child’s medications is also collected.

Tests

Actigraphy recordings may be ordered to rule out other types of sleep disorders. Actigraphy is a method of measuring movement. Information is collected via a wrist-watch type of device that is worn for up to 2 weeks. Alternatively, your sleep provider may request that you or your child fill out a sleep log to document sleep and wake times.

Two standard sleep study tests are usually performed. These are done in a sleep disorders center and require an overnight stay. These tests are a polysomnogram (PSG) and a multiple sleep latency test (MSLT).

• The polysomnogram (PSG) is an overnight test that takes continuous measurements, including heart rate, oxygen level, breathing rate, eye and leg movements, and brain waves while the child sleeps. A PSG reveals how quickly the child falls asleep, how often the child wakes up during the night, how often REM sleep is disturbed (a common finding in people with narcolepsy). This study also helps determine if symptoms are caused by another condition, such as obstructive sleep apnea. Most people with narcolepsy show disruptions in normal sleep patterns, with frequent awakenings.
• The multiple sleep latency test (MSLT) is performed during the daytime, the day after the PSG test. During MSLT, the child takes five short naps, usually scheduled two hours apart. The MSLT measures how quickly the child falls asleep and how quickly the child enters into REM sleep.

Another test that can be done is to measure hypocretin levels. However, this test is not commonly ordered since it requires a lumbar puncture.

How is narcolepsy treated?

There is no cure for narcolepsy at this time. The goal of treatment is to reduce daytime sleepiness and improve alertness so that children can experience as near a normal life as possible. Treatment plans typically involve a three-pronged approach: medication, behavioral modification and education.

Medication: Prescription medications are used to treat excessive daytime sleepiness, cataplexy, sleep disturbances and hallucinations. However, many of the following drugs have not been studied in large clinical trials involving children and most have not been approved for use in children. However, many of these medications are used “off-label” in children under the guidance of your physician and with close monitoring.

• Stimulants: Stimulants are prescribed to help patients stay awake during the day. Amphetamine stimulants, such as methylphenidate (Ritalin®), might be tried. Alternatively, wake promoting agents such as Modafinil (Provigil®) or armodafinil (Nuvigil®) are often tried first because they are associated with fewer side effects and are less addictive than other stimulants. These medications can produce side effects similar to that of caffeine, such as agitation, nervousness and palpitations. Other common side effects include headaches, stomach upset, and weight loss. They are generally started at a low dose and increased gradually as needed. Careful monitoring is required, as high blood pressure, heart arrhythmias (irregular heartbeats) and drug abuse have been reported.

Solriamfetol (Sunosi®) and pitolisant (Wakix®) have recently been approved by the Food and Drug Administration and are used to improve wakefulness in adults with narcolepsy. They have not been approved for use in children.

• Antidepressants: Cataplexy, hallucinations, disrupted nighttime sleep and sleep paralysis are often treated with two types of antidepressant medications: tricyclic antidepressants and selective serotonin reuptake inhibitors (SSRIs). Examples of tricyclic antidepressants include protriptyline (Vivactil®), clomipramine (Anafranil®) and desipramine (Norpramin®). Examples of SSRIs include fluoxetine (Prozac®), atomoxetine (Strattera®) and sertraline (Zoloft®). SSRIs generally have fewer side effects than tricyclic antidepressants.
• Sodium oxybate: Sodium oxybate (Xyrem®) is the only FDA-approved medication used to treat daytime sleepiness and cataplexy in patients age 7 years and older with narcolepsy.

The choice of medication(s) depends on the specific symptoms present in each individual child. It may take several weeks and/or several trials of different medications to find which one(s) work best and which dosage works best. Your healthcare provider may also recommend avoiding antihistamine products (an ingredient in many cold products), as these products block the action of a substance in the blood (histamine) that helps a person stay awake.

Behavior modification: Suggestions that are often given to both adults and children with narcolepsy include:

• Follow a regular sleep/wake schedule. Go to bed and wake up at about the same time every day.
• Take short naps (20 to 30 minutes) at times when you are feeling most sleepy, if possible.
• Keep your bedroom quiet, dark, cool, and comfortable. Do not watch TV or bring computers or phones into bed with you.
• Avoid caffeine (colas, coffee, teas, energy drinks, and chocolate) for several hours before bedtime.
• Exercise at least 20 minutes per day. Do not exercise within 3 hours of bedtime.
• Don’t eat large, heavy meals or a lot of liquids close to bedtime.
• Relax before bedtime. Take a warm bath, meditate, perform some gentle yoga moves, listen to soft music, or immerse yourself in relaxing scents such as peppermint, eucalyptus or lavender.
• Avoid activities that can be a danger to your health or life, such as driving, swimming, or cooking, except during times when you know you will be alert.

Education: It is important to work with your child’s teachers and coaches and to educate family members and close friends about your child’s disorder and how it may affect his or her functioning. For example, teachers who may not be familiar with narcolepsy may misjudge the child as disinterested, lazy or inattentive. In young children, narcolepsy symptoms may be mistaken as hyperactivity or as a learning disability.

On the other hand, sometimes teachers and coaches may be the first to approach parents out of concern over symptoms they see in their students and players such as:

• Poor attention and concentration.
• Irritability.
• Poor memory.
• Poor school performance.
• Showing up late for classes; being absent from school.

Children with narcolepsy may also need support from mental health professionals or support groups (see resources section). Children with narcolepsy report that they:

• Feel helpless and/or ashamed about their symptoms.
• Feel a loss of self-esteem.
• Get bullied or teased by other children; feel a lack of acceptance in school, athletics and social activities.
• Feel embarrassed for falling asleep.
• Are thought of as lazy by their classmates or other children.
• Fear falling at social events due to cataplexy.
• Avoid involvement in athletics and other after-school activities due to sleepiness or cataplexy.
• Fear others believe their symptoms are due to drug abuse.

What's the outlook for a child with narcolepsy?

There is no cure for narcolepsy. It is a life-long sleeping disorder. However, it usually does not worsen with age. The use of medications and behavior changes can help improve narcolepsy symptoms.

What should parents of school-aged children tell school officials?

Meet with school teachers and counselors about making adjustments to meet your child’s needs. For example, is it possible to change the classroom schedule to fit in a nap, can teachers’ lessons be recorded, and can your child get extra time or take breaks when taking tests?

When should I call my child’s healthcare provider?

If you believe your child has a sleeping disorder, see your pediatrician. He or she may refer you to a sleep specialist or sleep center for additional evaluation.

Narcolepsy is associated with poor school performance, which is often made worse by misdiagnosis and delayed diagnosis. School performance continues to decline as the schoolwork becomes more complex and challenging. Early diagnosis and treatment is important so that children do not fall behind in school.

Resources to turn to include:

• Narcolepsy Network. Support Groups.
• Narcolepsy Network. Facebook page.
• Find a Sleep Center.
• Wake Up Narcolepsy Online Support Group.
• Wake Up Narcolepsy Podcasts.