Narcolepsy in Pediatric Patients
Clinical Practice Insights and Educational Resources
Narcolepsy is a chronic and lifelong neurologic disorder characterized by excessive daytime sleepiness and abnormal manifestations of REM sleep. Narcolepsy is of 2 types—narcolepsy type 1 and narcolepsy type 2.2
The onset of narcolepsy usually occurs after 5 years of age, typically between 7 and 25 years of age.2 Narcolepsy type 1 is more common, accounting for ~70% to 80% of cases in pediatric patients.3,4 Patients with narcolepsy type 1 experience cataplexy—a brief, bilaterally symmetrical, sudden loss of muscle tone—in addition to excessive daytime sleepiness.1,2 These patients generally have low or undetectable concentrations of hypocretin, if known, in their cerebrospinal fluid. Patients with narcolepsy type 2 do not have cataplexy, and their hypocretin levels, when known, are higher than in patients with narcolepsy type 1.2
The International Classification of Sleep Disorders, 3rd edition, text revision (ICSD-3-TR) does not specify separate diagnostic criteria for pediatric versus adult patients with narcolepsy.2 However, timely and accurate diagnosis in children and adolescents may be particularly challenging due to differences in symptomology. Further, low prevalence of narcolepsy in pediatric patients along with a general lack of public awareness of the disorder contribute to delays in diagnosis. In these children and adolescents, appropriate recognition of symptoms is key to an effective diagnosis and management plan.1
Know Narcolepsy provides educational information, clinical insights, and resources that may help you in your clinical practice.
Symptoms
- All pediatric patients with narcolepsy have excessive daytime sleepiness, the inability to stay awake and alert during the day, resulting in periods of irrepressible need for sleep or unintended lapses into drowsiness or sleep2
- Excessive daytime sleepiness and impaired sustained attention can seriously impact their ability to function at school and during social situations1,2
- Excessive daytime sleepiness may range from waxing and waning drowsiness to irresistible, unintended lapses into sleep (sleep attacks)2,3
- Sleepiness is more likely to occur in sedentary, boring, and monotonous situations that do not require active participation (eg, sitting in a classroom, reading a book, or being driven in a car)1,2
- In young children with narcolepsy, sleepiness may be difficult to assess and may express itself in different ways, such as inattentiveness, decreased school performance, excessively long night sleep episodes, hyperactive behavior, and behavioral problems2
- Excessive daytime sleepiness may also be associated with memory lapses and complaints of fatigue/lack of energy2
- The need for habitual napping may be present, including the return of previously discontinued naps in older children1,2
- Children with narcolepsy may perform routine tasks without awareness or recollection (automatic behavior), such as brief episodes of sloppy or unintelligible handwriting2,3
- Cataplexy is a generally brief, bilaterally symmetrical, sudden loss of muscle tone with retained consciousness2
- The onset of cataplexy can occur very close to disease onset2,3
- Cataplexy generally manifests as muscle weakness in the face, neck, or legs and is typically triggered by emotions such as laughter2
- Pediatric patients also experience cataplectic facies, a collective term used to describe weakness involving the face, eyelids, and mouth with tongue protrusion.2,3 This form of cataplexy is not triggered by emotion3
Cataplexy occurs in the more common type of narcolepsy—narcolepsy type 1—and is the most specific symptom of narcolepsy. Patients may begin experiencing cataplexy at about the same time as excessive daytime sleepiness, or shortly thereafter.2,3 Cataplexy may be very severe around the time of disease onset and may appear differently than in adult patients.2
- In children with narcolepsy, other cataplexy manifestations include eyebrow raising, perioral movements, and head or trunk swaying3
- Pediatric patients may also experience complete cataplexy, which can result in falling or injuries4
- Cataplexy episodes are usually of short duration, lasting only seconds to typically up to 1 minute, followed by an abrupt return of muscle tone. Consciousness is typically preserved throughout the episode2
- The frequency of cataplexy episodes can be variable, with some patients experiencing more than 20 per day2
- Episodes of cataplexy in children can sometimes be confused with clumsiness, seizures, or neuromuscular disorders and can lead to delayed diagnosis1
Pediatric patients with narcolepsy may also report other, non-specific symptoms, such as disrupted nighttime sleep and signs of REM sleep dissociation (eg, sleep paralysis, hypnagogic/hypnopompic hallucinations).2,3
Hypnagogic/
hypnopompic hallucinations
- Patients may report vivid dreamlike experiences during the transition from wake to sleep (hypnagogic hallucinations) or during the transition from sleep to wake (hypnopompic hallucinations)2
- These hallucinations can have visual, auditory, and tactical characteristics and may be very disturbing2,4
Sleep paralysis
- Patients may experience the temporary inability to move, open their eyes, or speak at sleep-wake transitions, despite being awake, which can last several minutes and be very distressing2
Disrupted nighttime sleep
- Pediatric patients with narcolepsy may not report longer-than-normal time to sleep onset, but may have difficulty maintaining continuous sleep at night, often with frequent awakenings2,4
- Hypnagogic hallucinations may occur together with sleep paralysis2
- Hypnagogic hallucinations and sleep paralysis may be difficult to confirm, depending on the patient's verbal ability2
- Patients may experience vivid dreams that can be bizarre and frightening2
- REM sleep without muscle atonia can manifest as movements during sleep, including complex motor behaviors, that may be indicative of acting out dreams (eg, raising the head and reaching or grabbing and pantomime-like events characterized by slow, calm, and repetitive gesturing)2,5
- All pediatric patients with narcolepsy have excessive daytime sleepiness, the inability to stay awake and alert during the day, resulting in periods of irrepressible need for sleep or unintended lapses into drowsiness or sleep2
- Excessive daytime sleepiness and impaired sustained attention can seriously impact their ability to function at school and during social situations1,2
- Excessive daytime sleepiness may range from waxing and waning drowsiness to irresistible, unintended lapses into sleep (sleep attacks)2,3
- Sleepiness is more likely to occur in sedentary, boring, and monotonous situations that do not require active participation (eg, sitting in a classroom, reading a book, or being driven in a car)1,2
- In young children with narcolepsy, sleepiness may be difficult to assess and may express itself in different ways, such as inattentiveness, decreased school performance, excessively long night sleep episodes, hyperactive behavior, and behavioral problems2
- Excessive daytime sleepiness may also be associated with memory lapses and complaints of fatigue/lack of energy2
- The need for habitual napping may be present, including the return of previously discontinued naps in older children1,2
- Children with narcolepsy may perform routine tasks without awareness or recollection (automatic behavior), such as brief episodes of sloppy or unintelligible handwriting2,3
- Cataplexy is a generally brief, bilaterally symmetrical, sudden loss of muscle tone with retained consciousness2
- The onset of cataplexy can occur very close to disease onset2,3
- Cataplexy generally manifests as muscle weakness in the face, neck, or legs and is typically triggered by emotions such as laughter2
- Pediatric patients also experience cataplectic facies, a collective term used to describe weakness involving the face, eyelids, and mouth with tongue protrusion.2,3 This form of cataplexy is not triggered by emotion3
Cataplexy occurs in the more common type of narcolepsy—narcolepsy type 1—and is the most specific symptom of narcolepsy. Patients may begin experiencing cataplexy at about the same time as excessive daytime sleepiness, or shortly thereafter.2,3 Cataplexy may be very severe around the time of disease onset and may appear differently than in adult patients.2
- In children with narcolepsy, other cataplexy manifestations include eyebrow raising, perioral movements, and head or trunk swaying3
- Pediatric patients may also experience complete cataplexy, which can result in falling or injuries4
- Cataplexy episodes are usually of short duration, lasting only seconds to typically up to 1 minute, followed by an abrupt return of muscle tone. Consciousness is typically preserved throughout the episode2
- The frequency of cataplexy episodes can be variable, with some patients experiencing more than 20 per day2
- Episodes of cataplexy in children can sometimes be confused with clumsiness, seizures, or neuromuscular disorders and can lead to delayed diagnosis1
Pediatric patients with narcolepsy may also report other, non-specific symptoms, such as disrupted nighttime sleep and signs of REM sleep dissociation (eg, sleep paralysis, hypnagogic/hypnopompic hallucinations).2,3
Hypnagogic/hypnopompic hallucinations
- Patients may report vivid dreamlike experiences during the transition from wake to sleep (hypnagogic hallucinations) or during the transition from sleep to wake (hypnopompic hallucinations)2
- These hallucinations can have visual, auditory, and tactical characteristics and may be very disturbing2,4
Sleep paralysis
- Patients may experience the temporary inability to move, open their eyes, or speak at sleep-wake transitions, despite being awake, which can last several minutes and be very distressing2
Disrupted nighttime sleep
- Pediatric patients with narcolepsy may not report longer-than-normal time to sleep onset, but may have difficulty maintaining continuous sleep at night, often with frequent awakenings2,4
- Hypnagogic hallucinations may occur together with sleep paralysis2
- Hypnagogic hallucinations and sleep paralysis may be difficult to confirm, depending on the patient's verbal ability2
- Patients may experience vivid dreams that can be bizarre and frightening2
- REM sleep without muscle atonia can manifest as movements during sleep, including complex motor behaviors, that may be indicative of acting out dreams (eg, raising the head and reaching or grabbing and pantomime-like events characterized by slow, calm, and repetitive gesturing)2,5
Clinical Impact
Learn about the different ways in which narcolepsy can impact the daily lives of pediatric patients.
- In pediatric patients, excessive daytime sleepiness can lead to decreased performance in school2
- They may often fall asleep in class or while studying1,2
- Pediatric patients may also present with inattentiveness, emotional lability, or hyperactive behavior2
- Attention problems are characteristic of excessive daytime sleepiness and can lead to a psychiatric misdiagnosis of attention-deficit/hyperactivity disorder (ADHD)1,2
- Academic performance in pediatric patients with narcolepsy can deteriorate over time and as schoolwork becomes more complex and challenging1,7
- In one study, the percentages of patients reporting absenteeism, repeating grades, and academic difficulties were higher in children and adolescents with narcolepsy compared with those without narcolepsy1,6
- Narcolepsy can be socially disabling and have a negative impact on peer relationships1,2
- Lower energy/vitality and physical well-being can limit children and adolescents by restricting the type and amount of time spent on school performance, home life, and social activities6
Pediatric patients with narcolepsy have a high burden of both acute and chronic comorbid conditions.1,9
- Depression and anxiety are common psychiatric comorbidities seen in these pediatric patients2,3
- Attention-deficit/hyperactivity disorder (ADHD) and oppositional defiant disorder (ODD) are also seen1,4
- In addition, a US claims data study found that the frequency of claims for fatigue and sleep disorders (ie, circadian rhythm sleep disorders, obstructive sleep apnea, sleep-related movement disorders, insomnia) was higher in children and adolescents with narcolepsy compared with those without narcolepsy9
- Pediatric patients with narcolepsy can experience rapid weight gain coinciding with the onset of narcolepsy symptoms, subsequently resulting in obesity1
- Early onset of puberty is also observed in children with narcolepsy.2,4 Children with earlier age of onset of narcolepsy have a greater risk of precocious puberty4
- In pediatric patients, excessive daytime sleepiness can lead to decreased performance in school2
- They may often fall asleep in class or while studying1,2
- Pediatric patients may also present with inattentiveness, emotional lability, or hyperactive behavior2
- Attention problems are characteristic of excessive daytime sleepiness and can lead to a psychiatric misdiagnosis of attention-deficit/hyperactivity disorder (ADHD)1,2
- Academic performance in pediatric patients with narcolepsy can deteriorate over time and as schoolwork becomes more complex and challenging1,7
- In one study, the percentages of patients reporting absenteeism, repeating grades, and academic difficulties were higher in children and adolescents with narcolepsy compared with those without narcolepsy1,6
- Narcolepsy can be socially disabling and have a negative impact on peer relationships1,2
- Lower energy/vitality and physical well-being can limit children and adolescents by restricting the type and amount of time spent on school performance, home life, and social activities6
Pediatric patients with narcolepsy have a high burden of both acute and chronic comorbid conditions.1,9
- Depression and anxiety are common psychiatric comorbidities seen in these pediatric patients2,3
- Attention-deficit/hyperactivity disorder (ADHD) and oppositional defiant disorder (ODD) are also seen1,4
- In addition, a US claims data study found that the frequency of claims for fatigue and sleep disorders (ie, circadian rhythm sleep disorders, obstructive sleep apnea, sleep-related movement disorders, insomnia) was higher in children and adolescents with narcolepsy compared with those without narcolepsy9
- Pediatric patients with narcolepsy can experience rapid weight gain coinciding with the onset of narcolepsy symptoms, subsequently resulting in obesity1
- Early onset of puberty is also observed in children with narcolepsy.2,4 Children with earlier age of onset of narcolepsy have a greater risk of precocious puberty4
Clinical Resources
Long-term management of narcolepsy in pediatric patients requires measures to monitor severity of symptoms and changes during treatment.10 Although a variety of available subjective tests measure the severity of narcolepsy symptoms as well as treatment response, few have been validated specifically in pediatric patients with narcolepsy.10-13 Some of these scales have been modified to suit activities/language relevant to children and adolescents and are used in clinical practice for identification and monitoring of narcolepsy symptoms.11,12,14
Narcolepsy Assessments
Ullanlinna Narcolepsy Scale (UNS)- 11-item scale assessing the 2 main symptoms of narcolepsy, excessive daytime sleepiness and cataplexy10
- Consists of 7 items evaluating sleepiness and 4 items evaluating cataplexy11
- Initially designed as a screening tool to distinguish narcolepsy from other disorders with similar symptoms11
- Validated for screening for narcolepsy in children aged 6 to 17 years11
- Adapted from the Narcolepsy Severity Scale12
- Brief self-report questionnaire designed to measure frequency, severity, and consequences of the 5 key symptoms of narcolepsy type 1 in children and adolescents12
- Consists of 6 items on symptom frequency and 8 items on symptom consequences on daily life12
- Validated in children and adolescents ≥10 years of age12
Excessive Daytime Sleepiness Assessments
The Pediatric Daytime Sleepiness Scale (PDSS) and Epworth Sleepiness Scale for Children and Adolescents (ESS-CHAD) are scales measuring daytime sleepiness.11
Pediatric Daytime Sleepiness Scale (PDSS)- Self-assessment instrument used to evaluate daytime sleepiness in children and adolescents10,11
- Includes questions based on daily life situations related to sleep habits, waking time, and sleep problems10
- 32-item questionnaire related to daily sleep patterns, school achievement, mood, sleepiness, quality of life, and extracurricular activities10
- Validated in children and adolescents with narcolepsy aged 5 to 17 years11
- Developed from the adult Epworth Sleepiness Scale (ESS) to evaluate daytime sleepiness in children and adolescents14
- Modifications to the ESS were made to incorporate age-appropriate language and ensure that questions reflect activities in which children and adolescents are more likely to participate14
- Given as an example by the International Classification of Sleep Disorders, 3rd edition, text revision (ICSD-3-TR) for the subjective quantification of severity of daytime sleepiness2
- Validated in children and adolescents with narcolepsy with cataplexy aged 7 to 16 years13
Cataplexy Assessments
- Cataplexy questionnaires are generally diaries designed for specific clinical studies and assess the frequency of cataplexy attacks. They are not specifically validated for use in clinical practice10
- However, the Ullanlinna Narcolepsy Scale – Cataplexy subscore is used to measure the frequency of cataplexy attacks, including knees unlocking, mouth opening, head nodding, and falling down15
- Plazzi G, Clawges HM, Owens JA. Clinical characteristics and burden of illness in pediatric patients with narcolepsy. Pediatr Neurol. 2018;85:21-32.
- American Academy of Sleep Medicine. International Classification of Sleep Disorders. 3rd ed, text revision. American Academy of Sleep Medicine; 2023.
- Maski K, Kotagal S. Clinical features and diagnosis of narcolepsy in children. Clinical Decision Support | UpToDate | Wolters Kluwer. Updated November 28, 2023. Accessed March 13, 2024. https://www.uptodate.com/contents/clinical-features-and-diagnosis-of-narcolepsy-in-children
- Chung I-H, Chin W-C, Huang Y-S, Wang C-H. Pediatric narcolepsy: a practical review. Children (Basel). 2022;9(7):974. doi:10.3390/children9070974
- Antelmi E, Pizza F, Vandi S, et al. The spectrum of REM sleep-related episodes in children with type 1 narcolepsy. Brain. 2017;140(6):1669-1679.
- Inocente CO, Gustin M-P, Lavault S, et al. Quality of life in children with narcolepsy. CNS Neurosci Ther. 2014;20(8):763-771.
- Wise MS, Lynch J. Narcolepsy in children. Semin Pediatr Neurol. 2001;8(4):198-206.
- Quaedackers L, van Gilst MM, van Mierlo P, et al. Impaired social functioning in children with narcolepsy. Sleep. 2019;42(2). 10.1093/sleep/zsy228
- Carls G, Reddy SR, Broder MS, et al. Burden of disease in pediatric narcolepsy: a claims-based analysis of health care utilization, costs, and comorbidities. Sleep Med. 2020;66:110-118.
- Ouyang H, Gao X, Zhang J. Symptom measures in pediatric narcolepsy patients: a review. Ital J Pediatr. 2021;47(1):124. doi:10.1186/s13052-021-01068-7
- Lehert P, Plazzi G. Comparing symptom measurement tools in pediatric narcolepsy. Sleep Epidemiology. 2022;2(100032):2667-3436.
- Barateau L, Lecendreux M, Chenini S, et al. Measurement of narcolepsy symptoms in school-aged children and adolescents: the Pediatric Narcolepsy Severity Scale. Neurology. 2021;97(5):e476-e488.
- Wang YG, Menno D, Chen A, et al. Validation of the Epworth Sleepiness Scale for Children and Adolescents (ESS-CHAD) questionnaire in pediatric patients with narcolepsy with cataplexy aged 7-16 years. Sleep Med. 2022;89:78-84.
- Johns M. About the ESS-CHAD. Accessed April 18, 2024. https://epworthsleepinessscale.com/about-the-ess-chad/
- Hublin C, Kaprio J, Partinen M, Koskenvuo M, Heikkilä K. The Ullanlinna Narcolepsy Scale: validation of a measure of symptoms in the narcoleptic syndrome. J Sleep Res. 1994;3(1):52-59.