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Education & Practice Online First, published on June 8, 2015 as 10.1136/archdischild-2014-307291 BEST PRACTICE

Approach to a child with excessive daytime sleepiness Keshavamurthy Kallambella, Nahin Hussain Department of Paediatric Neurology, University Hospitals of Leicester NHS Trust, Leicester Royal Infirmary, Leicester, UK Correspondence to Dr Keshavamurthy Kallambella, Department of Paediatric Neurology, University Hospitals of Leicester NHS Trust, Leicester Royal Infirmary, Infirmary square, Leicester LE1 5WW, UK; [email protected] Received 5 August 2014 Revised 30 April 2015 Accepted 6 May 2015

INTRODUCTION Being alert when awake is an essential requisite for a child’s overall development. Sleepiness affects a child’s learning, behaviour, mood and physical health and has larger implications for the whole family.1 It is often an overlooked symptom in the paediatric practice, especially when present with other comorbidities and disabilities which increase its prevalence. A comprehensive exploration of this symptom and an accurate diagnosis of the underlying cause are very important as many of the conditions are treatable or remediable, though some of them will require specialist input. WHY IS IT IMPORTANT? A child who is sleepy is more likely to: ▸ ▸ ▸ ▸ ▸

be bullied by his/her peers at school be labelled as lazy have low self-esteem have poor academic performance have concerns raised over parenting skills

A detailed history and physical examination will guide the clinician when to extend the assessment and when to refer for more specialist review. WHAT CAUSES SLEEPINESS? Conditions causing excessive daytime sleepiness (EDS) can be categorised into three broad groups (see figure 1); conditions that ▸ lead to insufficient sleep ▸ decrease quality of sleep ▸ increase sleep-drive

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The first two groups with overlapping aetiologies will eventually lead to chronic sleep deprivation and account for the majority of cases. Insufficient duration of sleep among adolescents and young adults resulting in daytime sleepiness and impairment is becoming increasingly recognised internationally.2 WHAT TO ASK FOR IN THE HISTORY? Sleepiness is defined as inability to stay awake and alert during the major waking

episodes of the day, resulting in unintended lapses into drowsiness and sleep.3 It is not uncommon for parents to confuse fatigue or excessive tiredness on activity for sleepiness though both can coexist.4 If latter is the main symptom associated with sleep disturbances, then chronic fatigue syndrome and its differentials should be considered. Furthermore, the use of a sleepiness questionnaire as given in table 1 will establish EDS as the main symptom.5 Once this is confirmed, the primary objective of the consultation is to decide whether the child has insufficient sleep or a primary condition causing increased sleep drive. It is useful to send a sleep diary to be completed by the parent/child for the period covering 1–2 weeks prior to the clinic appointment. If there is history of disrupted sleep due to parasomnia (undesirable physical events or experiences during sleep) in the referral, recorded video of the event can be helpful. The medical history should include: ▸ onset of the problem ▸ use of screening tools such as ‘BEARS’6 (see table 2) ▸ any variation between ‘school days and holidays’; weekdays and weekends ▸ external influences: parental separation, bullying, abuse ▸ poor sleep hygiene practices (see box 1) ▸ behavioural problems ▸ psychiatric conditions ▸ comorbid medical conditions ▸ medications ▸ specifically enquire about disorders listed in table 3.

WHAT TO LOOK FOR IN EXAMINATION? Specific examination features of common diagnoses are given in table 4. INITIAL TREATMENT OPTIONS AND FURTHER INVESTIGATIONS The management of EDS depends on the underlying aetiology. Majority of the

Kallambella K, et al. Arch Dis Child Educ Pract Ed 2015;0:1–7. doi:10.1136/archdischild-2014-307291

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Best practice

Figure 1

Causes of excessive daytime sleepiness. ADHD, attention deficit hyperactivity disorder; OSA, obstructive sleep apnoea.

children including those with primary sleep disorders are helped by behavioural modifications and improvement of sleep hygiene. Extension of total sleep time results in resolution of sleepiness in those with chronic sleep deprivation. The use of melatonin can be considered in children with neurodevelopmental disorders to decrease sleep onset latency along with behavioural modifications.7 Those with suspected sleep disordered breathing can be considered for removal of their tonsils and adenoids in liaison with the ENT specialists. If a medical condition or drug side effect is the cause, then appropriate action should be taken. If there is persistence of sleepiness despite these remedial measures or a primary sleep disorder is a possibility, then a referral to the appropriate tertiary centre is advisable. We propose a flow chart of approach in figure 2. WHAT ARE THOSE FURTHER INVESTIGATIONS? These investigations (table 5) are carried out in a sleep lab. They aim to objectively quantify the degree of

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sleepiness as well as diagnose conditions such as obstructive sleep apnoea, unusual presentations of seizures and parasomnias, narcolepsy and periodic limb movement disorders. WHAT ARE THE COMMON PRIMARY SLEEP DISORDERS? Delayed sleep phase syndrome

DSPS is a circadian-based disorder in which an individual’s internal circadian clock is not in synchrony with the environmental time. The disorder is thought to represent an extreme manifestation of the normal delay of sleep onset that emerges during adolescence. Though mainly seen as a postpubertal problem affecting up to 16% of teenagers, it can sometimes manifest in childhood.8 These individuals have an inability to fall asleep until early hours of the morning (01:00– 06:00) and are unable to wake up until late morning or early afternoon. Their sleep duration and quality are normal. The diagnosis of this disorder is made mainly by clinical history of sleep patterns and

Kallambella K, et al. Arch Dis Child Educ Pract Ed 2015;0:1–7. doi:10.1136/archdischild-2014-307291

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Best practice Table 1

Paediatric Daytime Sleepiness Scale

Please answer the following questions as honestly as you can by circling one answer only: 1. How often do you fall asleep or get drowsy during class periods? Always Frequently Sometimes Seldom Never 2. How often do you get sleepy or drowsy while doing your homework? Always Frequently Sometimes Seldom Never 3. *Are you usually alert most of the day? Always Frequently Sometimes Seldom

Never

4. How often are you ever tired and grumpy during the day? Always Frequently Sometimes Seldom Never 5. How often do you have trouble getting out of bed in the morning? Very often Often Sometimes Seldom Never 6. How often do you need someone to awaken you in the morning? Always Frequently Sometimes Seldom Never 7. How often do you think that you need more sleep? Very often Often Sometimes Seldom Never Scoring system for the above questions: 4 3 2

1

0

*For question 3 reverse scoring system 20 sleepy

obtaining a sleep diary. Clinicians looking after ‘sleepy’ adolescents should understand this sleep behaviour and not dismiss it as ‘deliberate behaviour’. Other circadian rhythm sleep disorders including the non-entrained type can be present from birth in congenitally blind individuals, and in those with severe neuronal injury. Narcolepsy

Narcolepsy with cataplexy is a lifelong disease characterised by features often referred to as ‘narcolepsy tetrad’: ▸ EDS ▸ cataplexy (sudden loss of muscle tone following an emotional trigger)

Table 2

‘BEARS’: a sample sleep history6

B

Bedtime problems (Do you have any problems falling asleep at bedtime?)

E

Excessive daytime sleepiness (Do you feel sleepy a lot during the day? In school? While driving?)

A

Awakenings during the night (Do you wake up a lot at night?)

R

Regularity and duration of sleep (What time do you usually go to bed on school nights? Weekends? How much sleep do you usually get?)

S

Sleep-disordered breathing (Parent: Does your teenager snore loudly or nightly? Patient: Has anyone ever told you that you snore loudly at night?)

Box 1 Poor sleep hygiene practices 1. 2. 3. 4.

Frequent daytime napping Variable bedtimes or wake-up times Spending excessive amounts of time in bed Routine use of products containing alcohol, tobacco or caffeine in the period preceding bedtime 5. Scheduling exercise too close to bedtime 6. Engaging in mentally stimulating or emotionally upsetting activities too close to bedtime 7. Frequent use of the bed for activities other than sleep (eg, television watching, reading, studying, snacking, planning) 8. Failure to maintain a comfortable sleeping environment Modified from American Academy of Sleep Medicine.

▸ hypnagogic hallucinations (Hallucinations upon falling asleep) ▸ sleep paralysis (transient inability to move or speak during sleep–wake transitions).

Though nearly half of patients will have the disease onset in childhood or adolescence, diagnosis is often delayed by many years.9 This is due to the fact that the full spectrum of clinical manifestations is usually not present at disease onset and may present solely with EDS. Furthermore, childhood cataplexy may present differently compared with that in adults with persistent hypotonia and ‘cataplectic facies’ with repetitive mouth opening, tongue protrusion and ptosis.10 The problem is further compounded by the presence of other features around the onset of the disease such as unexplained weight gain (8–12 kg in 6 months), behavioural problems and mood dysfunction.9 Pathophysiology and classification

Narcolepsy can exist in two forms: with cataplexy (type1) or without (type 2).11 The former, which is more common, is thought to occur as a result of selective loss of hypocretin (orexin)-producing neurons in the hypothalamus. Hypocretins are the neuropeptides known to play an important physiological role in sleep–wake regulation. This selective damage is thought to be mediated by an autoimmune process in genetically susceptible individuals having human leukocyte antigen (HLA) subtype DQB1*0602. The low level of cerebrospinal fluid hypocretin-1 is used as a diagnostic test. Increased incidence of the disease in the 2009–2010 H1N1 vaccination campaign offers further support to the autoimmune hypothesis.12 Weight gain seen around the time of onset is thought to reflect the physiological role of hypocretin-1 in fat and energy metabolism. The current classification of narcolepsy is given in table 6.

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Best practice Table 3 Features of specific sleep disorders Disorder

Features

Treatment option

Behavioural insomnia of childhood

▸ Lack of limit setting by parents ▸ Inconsistent behavioural management ▸ Special conditions such as rocking or association with specific objects or setting

Behavioural management, sleep hygiene

Obstructive sleep apnoea (OSA)

▸ ▸ ▸ ▸

Snoring with respiratory pauses Snort-arousals Restlessness in sleep Sleeping in abnormal postures: knee–chest position, hyperextended neck. ▸ Day symptoms: morning headache, daytime sleepiness and irritability

Adenotonsillectomy, weight reduction, continuous positive airway pressure during sleep

Parasomnias

▸ Relation of the events to onset of sleep ▸ Review the video recording if available

Reassurance, low dose clonazepam

Periodic limb movement disorder and restless leg syndrome

▸ Uncomfortable sensation in the legs ▸ Irresistible urge to move the legs ▸ Symptoms worse in the evenings/nights

Sleep hygiene, trial of iron supplement, dopamine agonists, clonazepam

Idiopathic hypersomnia

▸ Excessive sleepiness ▸ Absence of other features of narcolepsy ▸ Postawakening confusion (sleep drunkenness)

Sleep hygiene, planned short naps

Narcolepsy

▸ ▸ ▸ ▸ ▸ ▸

Periods of irresistible sleep Cataplexy (loss of tone with giggling) in 40% Sleep paralysis Unexplained weight gain Behavioural problems with inattention Bizarre hallucinations

Good sleep hygiene and planned daytime naps, use of stimulants such as modafinil, methylphenidate or dextroamphetamine

Recurrent hypersomnia

▸ ▸ ▸ ▸

Episodic symptoms Long duration of sleep: 16–18 h Compulsive eating behaviour Hyper sexuality and depersonalisation

Good sleep hygiene and planned daytime naps. Modafinil, carbamazepine, lithium

Delayed sleep phase syndrome

▸ Inability to fall asleep until early hours of the morning ▸ Duration and quality of sleep normal

Idiopathic hypersomnia

This condition is characterised by constant and excessive sleepiness (typically 12–14 h) with prolonged but unrefreshing naps. These patients have difficulty waking up

Melatonin, bright light therapy to reset the clock

to alarm clocks and have postawakening confusion (sleep drunkenness) with automatic behaviours. It is differentiated from narcolepsy by the absence of cataplexy and a normal Multiple Sleep Latency Test.

Table 4 Examination System

Specific features

Possible aetiologies

Assessment of growth

High BMI Unexplained weight gain

OSA Narcolepsy

Assessment of development

Developmental impairment, autistic spectrum disorder, attention deficit hyperactivity disorder, visual impairment

Circadian rhythm disturbance behavioural insomnia

Dysmorphism

Features of Down syndrome, achondroplasia, craniofacial abnormalities, Prader–Willi syndrome, mucopolysaccharidosis

OSA

Neurodisability and additional needs

Pain secondary to contractures, dental diseases, pressure ulcers

Disrupted sleep Circadian rhythm disturbance

ENT examination

Adenoid facies, enlarged tonsils

OSA

Respiratory system

Features of poorly controlled asthma, cystic fibrosis

Disrupted sleep

Neurological examination

Abnormal facial movements, hypotonia, abnormal tongue movements Hypertonia, contractures,

Narcolepsy Disrupted sleep

Endocrine Short stature BMI, body mass index; OSA, obstructive sleep apnoea.

4

Hypothyroidism

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Best practice

Figure 2

Approach to excessive daytime sleepiness. PDSS, Paediatric Daytime Sleepiness Scale; PLMS, periodic limb movements of sleep.

Periodic limb movements and restless legs syndrome

Restless legs syndrome (RLS) is a disorder characterised by uncomfortable sensation in the legs that causes a strong, nearly irresistible urge to move the legs commonly in the evening hours and at night. Though it is commonly perceived as an adult disorder,

a significant proportion of adults recall the onset of their condition to be from childhood, usually 3 months with abnormal MSLT and presence of cataplexy or low CSF hypocretin-1

Type 2 Narcolepsy

EDS of >3 months with abnormal MSLT not explained by other causes and absence of cataplexy and normal CSF hypocretin-1 Type 1 and 2 narcolepsy secondary to a medical condition Examples include ▸ Neuroblastoma ▸ Traumatic brain injury ▸ Niemann–Pick disease type C ▸ Tumours of hypothalamus CSF, cerebrospinal fluid; EDS, excessive daytime sleepiness; MSLT, Multiple Sleep Latency Test.

6

Test your knowledge You are in a working paediatric outpatient setting in a district general hospital. A 14-year-old boy is referred by his general practitioner for episodes of sleeping for the past 1 month in the class room. Examination shows dyskinetic movements of the face and mild hypotonia in the limbs. His body mass index is 31 and he has recently gained significant weight. Question 1 What is the likely diagnosis? A. Adolescent sleep deprivation B. Delayed sleep phase syndrome (DSPS) C. Narcolepsy D. Obstructive sleep apnoea Question 2 What is the best management of this case? A. Start modafinil B. Advise behavioural therapy and reassess C. Advise weight reduction and reassess D. Refer to a sleep centre for further assessment See after the references for the answers.

Contributors KK performed the literature search and drafted the article. NH guided the literature search and reviewed the article before submission. Competing interests None declared. Provenance and peer review Not commissioned; externally peer reviewed.

REFERENCES 1 Millman RP, Working Group on Sleepiness in Adolescents/ Young Adults; and AAP Committee on Adolescence. Excessive sleepiness in adolescents and young adults: causes, consequences, and treatment strategies. Pediatrics 2005;115:1774–86. 2 Gradisar M, Gardner G, Dohnt H. Recent worldwide sleep patterns and problems during adolescence: a review and meta-analysis of age, region, and sleep. Sleep Med 2011;12:110–18. 3 American Academy of Sleep Medicine. International classification of sleep disorders. 3rd edn. Darien, IL: American Academy of Sleep Medicine, 2014. 4 Working party on sleep physiology and respiratory control disorders in childhood standards for services for children with disorders of sleep physiology. REPORT September 2009. http:// www.rcpch.ac.uk/sites/default/files/asset_library/Research/ Clinical%20Effectiveness/Endorsed%20guidelines/Sleep% 20Physiology%20Disorders%20%28RCPCH%29/Report% 20TextC.pdf (accessed 30 Jul 2014). 5 Drake C, Nickel C, Burduvali E, et al. The pediatric daytime sleepiness scale (PDSS): sleep habits and school outcomes in middle-school children. Sleep 2003;26:455–8.

Kallambella K, et al. Arch Dis Child Educ Pract Ed 2015;0:1–7. doi:10.1136/archdischild-2014-307291

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Best practice 6 Judith AO, Victoria D. Use of the ‘BEARS’ sleep screening tool in pediatric residents’ continuity clinic: a pilot study. Sleep Med 2005;6:63–9. 7 Appleton RE, Gringras P. Melatonin: helping to MEND impaired sleep. Arch Dis Child 2013;98:216–17. 8 Gradisar M, Crowley SJ. Delayed sleep phase disorder in youth. Curr Opin Psychiatry 2013;26:580–5. 9 Adi A, Mali E, Ling L, et al. Clinical and therapeutic aspects of childhood narcolepsy-cataplexy: a retrospective study of 51 children. Sleep 2010;33:1457–64. 10 Prasad M, Setty G, Ponnusamy A, et al. Cataplectic facies: clinical marker in the diagnosis of childhood narcolepsy-report of two cases. Pediatr Neurol. 2014;50:515–17. 11 Baumann CR, Mignot E, Lammers GJ, et al. Challenges in diagnosing narcolepsy without cataplexy: a consensus statement. Sleep 2014;37:1035–42.

12 Barker CI, Snape MD. Pandemic influenza A H1N1 vaccines and narcolepsy: vaccine safety surveillance in action. Lancet Infect Dis 2014;14:227–38. 13 Picchietti DL, Jeffrey SD, Weerd AL, et al. Pediatric restless legs syndrome diagnostic criteria: an update by the International Restless Legs Syndrome Study Group. Sleep Med 2013;14:1253–9.

Answers to the questions 1. C 2. D

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Approach to a child with excessive daytime sleepiness Keshavamurthy Kallambella and Nahin Hussain Arch Dis Child Educ Pract Ed published online June 8, 2015

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Topic Collections

Articles on similar topics can be found in the following collections Drugs: CNS (not psychiatric) (58) Sleep disorders (4) Sleep disorders (neurology) (6) Sleep disorders (respiratory medicine) (6) Child abuse (11) Child and adolescent psychiatry (paedatrics) (56) Child health (292) Attention-deficit hyperactivity disorder (8) Neuromuscular disease (17) Rheumatology (66) Screening (epidemiology) (30) Screening (public health) (30)

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