Associated Sleep Disorders and their treatments Flashcards
Delayed Sleep wake phase disorder, characteristics?
stable delay in timing of sleep/wake cycle CBTmin and DLMO are delayed longer tau Altered response to light polymorphism in hPer3 typical schedule 4am-12pm
Delayed sleep wake phase disorder, Treatment
Good sleep hygiene Avoid bright light in evening Bright light in morning (5000 lux) melatonin low dose melatonin 4 - 8 hours before your natural bedtime. If you then get tired earlier, move your schedule an hour earlier and take the melatonin an hour earlier. Let it stabilize. Then move another hour earlier.
Chronotherapy
This technique aims to reset the circadian clock by slowly delaying the bedtime (and hence the sleep period) by about two hours every few days. This strategy is used less commonly than the light therapy method. It invariably disrupts normal schedule of activity during the shift, when day and night are reversed.
Leg cramps and restless legs syndrome (RLS) can mimic each other. What statement is true?
In leg cramps, patients describe an actual cramp or hardening of the muscle
Sometimes it is difficult to distinguish between RLS and sleep related leg cramps. However, there is an actual cramp and hardening of the muscle in sleep related leg cramps.
A 37-year-old patient with BMI of 35 Kg/M2, depression and pain is brought to the clinic by his wife. She is very distressed by his lack of participation in daily chores and family-related activities. She attributes it to his lack of engagement. He used to work as a manager in a retail store but following a car accident 2 years ago associated with loss of consciousness, has not been able to return to his former job. He had incurred serious injuries which required prolonged hospitalization and rehabilitative efforts. Gradually he recovered and was cleared to return to work.
He is currently taking sertraline and naproxen. Wife reports that he is sleeping much longer now, total of about 11-13 hours every night with bedtime of 10:00 PM and arise time of 11:00 AM. He also takes naps during the day. He has had mild snoring for last 10 years but no vivid dreaming or any sudden changes in muscle tone during the day.
What is the most likely cause of his hypersomnia?
Traumatic Brain injury
Patient has post traumatic hypersomnolence which falls into the category of hypersomnia due to medical condition. TBI can cause hypersomnolence which can severely affect quality of life. Wake-promoting drugs can help alleviate the condition. Limited studies have shown a decrease in CSF hypocretin in some post-traumatic cases as compared to normal. In this case his sleepiness started after the accident and persisted despite of recovery. Thus, a temporal association makes that etiology most plausible.
What is ERRT?
Exposure, relaxation and rescription therapy
What is ERRT used for?
Treatment of nightmares
What is the DDSI?
Disturbing Dream and severity Index
Developed by Dr. Barry Krakow
What is the normal human circadian cycle?
24.3 hours is our normal cycle
How do we entrain our circadian clock?
With zeitbebers (time keepers)
What is the most powerful zeitgeber?
Light/dark cycle is the most powerful zeitgeber for entraining our clock
What are social zeitgebers?
Meals
Exercise
Social cues such as work, etc.
What is the prevalence of nightmare disorder?
2-6% of adults meet criteria for nightmare disorder
What is the relationship between nightmares and PTSD?
Presence of nightmares before trauma increases likelihood of developing PTSD
What is the definition of nightmare disorder, DSM 5?
Repeated occurrences extended, extremely dysphoric and well remembered dreams
2nd half of major sleep episode
On awakening, rapidly becomes oriented and alert
Clinically significant distress or impairment
Not attributable to substance
Coexisting mental/medical d/o do not explain complaint
What is the clinical threshold for nightmare d/o and consideration for Tx?
Consider treatment if >1 nightmare per week
Duration: persistent, >6 months
What happens when patient with nightmare d/o avoids sleep (common response)?
Causes REM rebound
What is a common non medication Tx for nightmare d/o?
Imagery rehearsal therapy (IRT)
Recommended for both nightmare disorder and PTSD nightmares
Individual and group
1-3 sessions
Tx very simple
What is the recommended pharmacological Tx for Nightmares?
Prazosin
How does Prazosin work?
Alpha-1 adrenergic receptor antagonist
Decreases CNS sympathetic outflow
1st choice for pharmacologic Tx
What is chronotherapy?
Treatment for delayed sleep phase d/o
This technique aims to reset the circadian clock by slowly delaying the bedtime (and hence the sleep period) by about two hours every few days. This strategy is used less commonly than the light therapy method. It invariably disrupts normal schedule of activity during the shift, when day and night are reversed.
What is Advanced sleep phase disorder (ASWPD)?
Significant advance in phase of major sleep period (>2 hrs relative to socially acceptable time)
Sxs present for at least 3 months
Ad lib sleep improves sleep quality, quantity, consistency
Early morning awakening
Est. 1% of population likely low (less likely to perceive as problematic vs DSWPD)
Tau <24hrs
What is the DSM-5 definition of Chronic insomnia?
o Difficulty initiation sleep (kids: w/o intervention) OR o Difficulty maintaining sleep (kids: w/o intervention) OR o Early morning awakening o Impairment in social, occupational, educational, academic, behavioral, or other area of functioning o 3+ nights a week, 3+ months o Adequate opportunity for sleep o Not purely from a substance o Specify With Non-sleep dx mental comorbidity With other medical comorbidity With other sleep disorder Episodic: 1-3 months Persistent: 3+ months Recurrent: 2+ episodes in a year Substance/medication Other specified Unspecified
ICSD-3 Chronic insomnia disorder definition
o The patient reports or the patient’s parent or caregiver, one or more of the following:
Initiating sleep
Maintaining sleep
Waking earlier than desired
Resistance to going to bed on appropriate schedule
Difficulty sleeping without parent or caregiver
NOTE: dissatisfaction with sleep quality was removed in ICSD-3
o Daytime complaints (fatigue, attention, behavioral probs, motivation, etc.)
o Need adequate time/opportunity
o 3 nights per week for 3 months
o 10% prevalence, adults
o 30M in US with chronic insomnia
o Insomnia high risk for depression
o Insomnia + short sleep, increased HTN, DM, mortality
What is the most common cause of insomnia in teens?
Delayed Sleep phase syndrome
What is definition of Delayed Sleep Phase Syndrome?
o Persistent (>3 months) inability to fall asleep and arise at conventional clock times
o Sleep onset delayed until early morning (3am-6am) with rise-times in early afternoon (11am-2pm)
o Patient complains of sleep onset insomnia
o Often presents in adolescence
o Awakening early because of social/occupational requirements results in daytime sleepiness
o Sleep is normal in pattern and duration
What is a treatment for Delayed sleep. Phase syndrome?
Chronotherapy:
o Bedtime is systematically delayed 2-3 hours each day
o Patients sleep only 7-8 hours without naps
o Chronotherapy is maintained until the desired bedtime is reached (11pm or midnight)
o Bedtime subsequently rigidly maintained
o Consider early morning bright light therapy (2500 lux) for 1-2 hours and light restriction after 4pm
What is the American College of Physicians (ACP) guidelines for Tx of chronic insomnia?
o All get CBTi for chronic insomnia, 1st line recommendation
o But a supply and demand issues with BSM providers
o 60,000 patients per 1 BSM provider
o CBTI helpful for ~2/3 pt but still a high number suffering with sx
o Cons of digital CBT-I
Adoption, appropriateness, feasibility, costs, coverage, etc
o 2nd recommendation – if pt does not respond or does not prefer BSM
Clinicians use a shared decision making approach to add medication
Survey of patients with insomnia
Sleep 2014
87% use zolpidem of Non-BzRA
20% use a med to help them sleep
What are the Neurobiological targets for medication?
o Meds either (flip between sleep and wake)
Enhance sleep (gaba receptor)
Or target arousal cycle
• What are the Classes of Pharmacologic Treatment for Insomnia?
o Benzodiazepine receptor agonist (BzRA)
o Melatonin agonists
o Orexin antagonists (DORA)
o Sedating antidepressants
o Antipsychotics (e.g., dopaminergic antagonists)
o Anticonvulsants (e.g., gabapentin)
o OTC agents (nonselective antihistamines)
GABA-A Receptor
Alpha subunits
6 alpha subunits
Alpha 1 effects: sedative, amnestic, anticonvulsive
Alpha 2 effects: anxiolytic, muscle relaxant
Alpha 3 effects: muscle relaxant
What are typical Benzodiazepine (GABA)-Receptor Agonists (BzRA)?
Clonazepam (Klonopin), .25-1.0mg, half life: 40 hrs
Temazepam (Restoril), 7.5-30 mg, half life 4-18 hrs (FDA approved for insomnia)
Lorazepam (Ativan), 0.5-2 mg, half life10-20 hrs
Oxazepam (Serax), 10-30 mg, half life 5-10 hrs
Eszopiclone (Lunesta), 1-3 mg, half life 5.5-8 hrs
FDA approved for insomnia
Triazolam (halcion), 0.125-0.25mg), half life 2-3 hrs
FDA approved for insomnia
Zolpidem (Ambien), 3.75-12.5mg, half life 2-3 hrs, CR extends duration of action, FDA approved
Zaleplon (Sonata), 5-10mg, half life 1-2 hrs, FDA approved
What is the biggest discerning factor when comparing BzRA drugs?
the half-life
Klonopin has fallen out of favor, reasonable to suppress anxiety but not ideal for insomnia
long half life = 40 hrs
risk for falls
What is the most common BzRA drug used for insomnia?
Zolpidem
What are the “Z” drugs?
Non-benzo-benzos
Eszopiclone
Zolpidem
Zaleplon
What are characteristics of “Z drugs”?
o Work fundamentally in the same way and bind to same receptor of benzo, but differ in terms of half-life
o Don’t have anxiety reducing effects – so other benzos might be good for pts w/ anxiety that are not interested in behavioral treatment
Are PSG’s required for FDA approval of medications?
Yes
Are sleeping pills addictive?
Tolerance
Physiological dependence
Psych dependence
Non-medical diversion
Do Benzos increase risk of dementia
Do Benzos increase mortality risk
There are no RCT studies that prove benzos increase risk of dementia
No RCT studies that show benzos increase mortality risk
What is the SHARE approach to Shared Decision Making?
Seek your patient’s participation
Help your patient explore and compare treatment options
Assess your patient’s values and preferences
Reach a decision with your patient
Evaluate your patient’s decision
What is Ramelteon?
It’s a melatonin agonist that stimulates melatonin receptor
What are orexin antagonist?
DORA: dual orexin receptor antagonists
Suvorexant (Better for staying asleep/not as great for SL) Belsomra
Lemborexant: FDA approval 2019: better for sleep onset insomnia (Dayvigo)
Daridorexant : FDA approval 2022 (Quviviq)
Blocks the effects of neurotransmitter orexin
Orexin promotes wakefulness
DORAs bind to orexin receptor 1 and orexin receptor 2, blocking the effects of orexin, reducing wakefulness and helping people sleep
Newest classification for insomnia
Stabilize the wake-promoting neurons
Lower abuse potential
What are DORA risks (Dual Orexin Receptor Antagonists) ?
Somnolence Dose-dependent increase in suicidality Complex behaviors Sleep paralysis Abnormal thinking Behavioral changes (history of SUD or can’t take BZRAs)
What sedating antidepressants are used for insomnia?
Doxepin Mirtazapine Trazodone Amitriptyline Nortriptyline
Potential advantages: No abuse, effective for WASO
Potential disadvantages: Anticholinergic at high doses, cardiac effects, falls
What are selective H1 antagonist used for insomnia?
Doxepin (only approved med in this class for sleep maintenance insomnia, 3-6mg) Silenor
Mirtazapine (2-4 mg selective effects)
What sedating antidepressants used for insomnia are working on mixed receptors?
Trazodone Amitriptyline Doxepin (at higher doses) Mirtazapine (higher dose)
Little abuse potential, non-scheduled
Primary problem staying asleep
What is the efficacy data on doxepin?
Dose-dependent effects on sleep efficiency – especially on LAST 3rd of the night
Blocks histamine
What are antidepressant side effects?
Anticholinergic side effects (amitriptyline, Trazodone)
• cardiac conduction issues, urinary retention, dementia risk over time, orthostatic hypotension (BP drops when they stand up)
somnolence (non H1 selective)
complex behaviors
abnormal thinking
behavioral changes
How does Trazodone work for insomnia?
Low dose Trazodone use exerts a sedative effect for sleep through antagonism of 5-HT-2A receptor, H1 receptor and alpha-1 adrenergic receptors
Reduces levels of neurotransmitters associated with arousal effects, such as serotonin, NE, dopamine, ACH and histamine (Mixed receptors)
What are advantages and disadvantages of BzRA drugs (Benzodiazepine receptor agonists)?
Advantages: Efficacious, variety of half-lives
Disadvantages: Cognitive effects, falls, dependence
Examples: zolpidem, zalpelon, Eszopiclone, temazepam
What are advantages and disadvantages of Antihistamines for insomnia
Advantages: widely available
Disadvantages: cognitive effects, limited efficacy data
Examples: diphenhydramine, doxylamine
What are advantages and disadvantages melatonin, receptor agonist
Melatonin, ramelteon
Advantages: “natural” mechanism
Blocks wake signal
Ramelteon reasonable to use in elderly and patients at risk for abuse
Disadvantages: limited efficacy on WASO
What are advantages and disadvantages Orexin antagonist?
Example: suvorexant (belsomra)
Advantage: novel mechanism, blocks wake signal
Disadvantage: limited efficacy, effectiveness data
What are advantages and disadvantages of sedating antipsychotics?
Examples: Quetiapine, olanzapine
Advantages: Not BzRA, efficacy for psychosis, depression
Disadvantages: metabolic, neurological,
CV effects
What are advantages and disadvantages of
Gabapentin, Lyrica
Advantages: Not BzRA, efficacy for pain
Disadvantages: Limited sleep efficacy data
How to choose medications for insomnia
What is the evidence FOR:
o Weak evidence FOR
Ramelteon (Rozerem) - sleep onset
Doxepin (Silenor) – sleep maintenance
Suvorexant (Belsomra) - sleep maintenance
Eszopiclone (Lunesta) - sleep onset, sleep maintenance
Zaleplon (Sonata) - sleep onset
Zolpidem (Ambien) - sleep onset, sleep maintenance
Triazolam (Halcion) - sleep onset
Temazepam (Restoril) - sleep onset, sleep maintenance
How to choose medications for insomnia
Weak evidence AGAINST
Trazodone (Desyrel) Tiagabine (Gabitril) Diphenhydramine (Benadryl) Melatonin Tryptophan Valerian
What should be patient expectations with sleep and sleep aids?
o Sleep is an involuntary biological process influenced by behavior and medication
o Reasonable expectations regarding sleep and medications, it’s not general anesthesia; modest effects, you’re not going to sleep for 8 hrs
20 minutes more of sleep typically with sleep meds
o Detailed instructions – who, what, when, how
o Appropriate timing
What is appropriate follow up for patients on sleep aid?
o Ongoing assessments of effectiveness and side effects
o Use lowest dose for shortest period of time
o Discuss challenges
o Answer questions
o Challenge patients to withdraw hypnotics
o Reassess comorbid conditions
o Education regarding long-term use
What are hypersomnias
Central disorder of Hypersomnolence
Narcolepsy Type 1
Narcolepsy Type 2
Idiopathic hypersomnia
Klein-Levin Syndrome
Hypersomnia due to another medical disorder
Hypersomnia due to a medication or substance
Hypersomnia associated with a psychiatric disorder
Insufficient sleep syndrome
What is the definition of Hypersomnolence?
ICSD-3
symptom of excessive sleepiness
o Inability to stay awake and alert during major waking episodes
o Unintended lapses into sleep/irrepressible need for sleep
o Children: may present as inattentiveness, emotional lability, or hyperactive behavior
o Can be due to disturbed nocturnal sleep, misaligned circadian rhythms, or of central origin
What is the definition of Hypersomnia?
condition or specific disorder with hypersomnolence as the primary symptom
What is chronic Hypersomnia?
Hypersomnias of central origin
Narcolepsy
Idiopathic hypersomnia
What are the characteristics of Narcolepsy Type I and Type 2 ?
What is cataplexy?
daily periods of hypersomnolence >= 3 months
Gradual or “sleep attacks” (without prodromal symptoms)
EDS
Sleep hallucinations
Sleep paralysis
Disruption of nocturnal sleep (50%)
1 in 2000 incidence, early childhood to 50’s, peaks in 20’s
Daytime naps are refreshing (as opposed to IH where naps are not refreshing)
Cataplexy present in Narcolepsy Type 1, not present in Type II
Cataplexy: brief (<2 min), sudden loss of muscle tone in response to strong emotions (laughter)
Is abrupt, reversible, bilateral
DTR’s absent
Remains conscious
Type 1: cataplexy, orexin <110, EDS
Type 2: no cataplexy, normal orexin, EDS
Narcolepsy Type II, definition
EDS and MSLT findings same as Type I, but without cataplexy
CSF Hypocretin-1 levels are unknown or are above threshold for Narcolepsy Type I
Not better explained by insufficient sleep, OSA, Delayed sleep phase, med or substances
What are associated features of Narcolepsy
Sleep paralysis (temporarily inability to move at sleep-wake transitions)
Hallucinations (visual, auditory, tactile)
Hypnogogic: transition from wake to sleep, vivid dreams at sleep onset
Hypnapompic: transition from sleep to wake
Symptoms can be variable, wax and wane, occur in different frequencies across individuals
Extremely sleepy, fall asleep in places
Disturbed nocturnal sleep-increase arousals
What is the prevalence of Narcolepsy in the population
What is the typical onset?
25-5- per 100,000 people
.025%-.05% of general population
Onset: after age 5, most often between 15-25
Sleepiness, cataplexy and other REM related Sx
What is the etiology and pathophysiology of Narcolepsy?
Hypocretin deficiency syndrome
Decrease hypocretin/orexin in in hypothalamus
?Autoimmune destruction
Genetic predisposition: HLA-DR and HLA-DQ alleles
Environmental triggers: infection, seasonal patterns
How is the diagnosis of Narcolepsy made?
Clinical Evaluation Lab tests Nocturnal PSG Next day MSLT MWT
What is the clinical evaluation for Narcolepsy?
Clinical symptoms (hypersomnolence, cataplexy)
Rule out other causes of hypersomnolence (sleep deprivation)
1 week of actigraphy with diary (ideal)
What are the Lab tests done for evaluation of Narcolepsy?
Nocturnal PSG
Sleep-onset REM (SOREM) <15 min
Next day MSLT (most common in narcolepsy)
Mean SOL < 8 min. And =>2 SOREMS (one can be from previous night PSG)
CSF of hypocretin-1 (involves spinal tap)
How is the MSLT done?
Purpose: Standard measure for objective sleepiness, differential diagnosis
Indications: unexplained EDS, narcolepsy, IH
Includes EEG, chin tone, oxygen, EKG
Protocol:
PSG night prior; let them sleep in when at lab, then get enough sleep, 6 hr TST adult, 7.5 peds
4-5 nap opportunities scheduled at 2hr intervals starting 1.5-3 hrs from end of PSG
Instructions: “try to fall asleep”
Nap session is terminated after 20 min.
Other considerations:
Preceded by nocturnal PSG (>6 hrs TST adults, 7.5 hrs peds)
Ideally, no medications for 2 weeks prior (i.e. no stimulants, stimulant-like meds, REM suppressing meds such as SSRI/SNRI)
Ideally scheduled at patient’s typical sleep/wake schedule
Sleep logs or actigraphy 1 week prior, make sure not sleep deprived
What is the Maintenance of Wakefulness test (MWT) ?
Purpose: measures the ability to stay awake; often used to evaluate response to treatment
Protocol:
4 trials at 2 hr intervals
Instructions: “remain awake for as long as possible”
Trials end after 40 min. If no sleep
Considerations:
PSG prior to MWT?
Unclear if MWT generalizes to occupational safety
Hypersomnolence Diagnostic criteria
ICSD3
Severe sleepiness for >3 months
No cataplexy
<2 SOREMPs (sleep-onset REM periods that occur within 15 min of sleep onset) on MSLT (or no SOREMPs if REM latency on preceding PSG is <15 min.
At least 1 of the following:
MSLT shows mean SL <= 8 min.
Total 24 hr sleep time is >= 660 min (11 hr) on
Either 24 hr PSG or by >= 7 day wrist actigraphy
Insufficient sleep syndrome is ruled out
Can use actigraphy or diary to document increased sleep opportunity
Consider idiopathic unless identified medical, psych, substance underlying
What are the treatments for Narcolepsy?
Medications Stress management CBT techniques Pomodoro Technique Mindfulness and acceptance CBT-H program (Jason Ong)
What medications are used for treatment of Narcolepsy?
Sodium Oxybate, dose 4.5-9 mg, class: GHB. Indication: EDS and cataplexy
Modafinil, dose 200-400 mg, class: Non-amphetamine Stimulant, indication: EDS (nuvigil, shorter half life)
Armodafinil, dose 150-250mg, longer half life, class: Non-amphetamine stimulant, indication : EDS (provigil)
Dextroamphetamine, dose 5-60 mg, class: stimulant, indication :EDS
Methylphenidate, dose: 10-60mg, class: stimulant, indication: EDS
Imipramine, dose: 50-250 mg, class: TCA, indication: cataplexy
Nortriptyline, dose 50-150 mg, TCA, Cataplexy
Fluoxetine, 20-80 mg, SSRI, cataplexy
Venlafaxine, 75-375mg, SNRI, cataplexy
Selegiline, 5-10 mg, MAO-B inhibitor, EDS & cataplexy, (works by increasing dopamine in the brain). Dopamine controls movement; also used in Parkinson’s
What are some special issues with Narcolepsy for patients?
Treatments can reduce sleepiness, but unmet need for improving QOL, improving psychosocial functioning
Patients w/narcolepsy and IH have sig. reduction in health-related QOL
At least 50% w/narcolepsy report symptoms of depression, 15% moderate depression
What are stress management techniques for Narcolepsy and IH?
Chronic sleepiness is a form of stress
CBT for chronic pain: similar unrelating pattern, no cure, sig. impact on QOL
Coping strategies (emotion-focused, problem-focused, avoidance)
What is the Pomodoro Technique used in Narcolepsy and IH?
Key concept: split up large intervals of time into smaller, more manageable components (set time for work, separate short breaks)
Re-conceptualize wakefulness
Split wakefulness and naps up
The Pomodoro technique is a time management method developed by Francesco Cirillo in the 1980’s. uses a timer to break work into intervals, typically 25 min. In length, separated by short breaks. Each interval is known as a Pomodoro.
Pomodoro means tomato in Italian. Cirillo used a tomato shaped kitchen timer as a university student.
How do we apply mindfulness and acceptance to Narcolepsy and IH?
Mindfulness and acceptance:
Being mindful of physical and mental state
Is it possible to accept without giving up? Acceptance is an active (not passive) process
Avoid the “second dart or arrow” (1st dart comes from enemy, part of living)
2nd dart or arrow we throw on ourselves
Comes from Buddhism-suffering from attaching from outcomes
What are the details of CBT-H program?
o Cognitive: emotion reg, cope with symptoms, value living
o Behavioral: behave changes to improve structure and efficiency, min impact of symptoms of functioning, reg nighttime sleep
o Interpersonal: how to deal with it
What is Kleine-Levin Syndrome?
Rare disorder w/recurrent episodes of excessive sleep along with cognitive and behavioral changes.
May sleep up to 20 hrs per day during episode
A few days to a few weeks
May start abruptly or sometimes preceded by an upper respiratory infection
Additional sx: hyperphagia (excessive food intake), irritability, childishness, disorientation, hallucinations, abnormally uninhibited sex drive
Affects primarily adolescent males
Unknown origin
Stimulants (modafinil, methylphenidate, amphetamine) and mood stabilizers (lithium) may be prescribed
Insufficient Sleep syndrome
Working too much!
Hypersomnia in older adults
o Not necessarily more in older adults o Daytime sleepiness vs naps o ESS difficult measure because not all older adults do all tasks on ESS o Treatment Bright light therapy Naps Exercise
What are the pediatric hypersomnias?
Excessive daytime sleepiness
Hypersomnolence
Narcolepsy
Fatigue
What is the definition of EDS in pediatrics population?
Increased in sleep duration Resumption of naps Falling asleep inappropriately Inability to awaken in the morning Daytime inattention Misbehavior Mood lability Hyperactivity
What is REM Behavior Disorder (RBD) ?
Violent/frightening dreams “acted” out by patient
PSG shows REM sleep without atonia
Typically male >60 years old
What diseases is RBD associated with?
Parkinson’s disease
Lewy Body disease
Multisystem atrophy
RBD is associated with withdrawal from what substances?
ETOH
barbiturates
Meprobamate (miltown)
What medications is RBD associated with?
SSRI's TCA's Fluoxetine venlafaxine MAOI
SSRI’s may also be associated with increased muscle activity in REM sleep even in absence of symptomatic RBD
How is the Diagnosis of RBD established?
Dx of RBD is based on clinical symptoms of disturbing behaviors during sleep with dreaming and violent behavior.
Findings of REM without atonia only on PSG doesn’t establish dx of RBD
What is the Dx criteria for Hypersomnolence in pediatric pop (ICSD3)
Severe sleepiness x 3 mos
No cataplexy
<2 SOREMPs on MSLT (or no SOREMPs if REM latency on preceding PSG is <=15 min
At least 1 of the following: MSLT shows MSL <=8 min, 24 hr TST >=11 hrs on 24 hr PSG or by 7 day actigraphy
Insufficient sleep is ruled out (actigraphy or sleep diary)
o Consider idiopathic unless identified medical, psych, substance underlying
What is the diagnostic criteria for Narcolepsy diagnosis in Pediatric population?
o At least 3 months of severe sleepiness
o Children may sometimes present with excessively long nighttime sleep or as resumption of previously discontinued daytime naps
o MSL <= 8 min and 2+ SOREMPs on MSLT
o Hypersomnolence and/or MSLT findings are not better explained by other causes such as insufficient sleep, obstructive sleep apnea, delayed sleep-wake phase disorder, or the effect of medication/substance or withdrawal
o Type 1
Cataplexy
CSF hypocretin-1 concentration is either <= 110
pg/mL or <1/3 of mean values
o Type 2
No Cataplexy
Either CSF hypocretin-1 concentration has not been measured or CSF hypocretin-1 is either >110 pg/mL or > 1/3 of mean values
What is the definition of Fatigue in pediatric population?
o Physical sensation of extreme tiredness
o Related to mental or physical exertion
o Relieved by rest
o May be “unrelenting exhaustion”- chronic fatigue (not relieved by rest)
o Chronic fatigue may be improved by exercise
What are the features of EDS in pediatrics pop?
o Differentiated from fatigue o Propensity to fall asleep o Difficulty remaining awake from activities o Increased in napping o Longer sleep duration o Difficult waking the morning o May vary from mild to severe
MSLT in pediatric population
4-5 nap opportunities
2 hr intervals
Quiet, dark room (no TV, electronic devices)
EEG, EOG, EMG, EKG
If sleep onset occurs, permitted to sleep for 15 min
No sleep occurs in 20 min, nap opportunity ends
Record # of naps, mean sleep onset minutes, SOREMPs
EDS in preteen <= 15 min
EDS in teens <=10 min
What are Associated features w/ hypersomnia and narcolepsy ?
o Increased risk of accidents o Cognitive difficulties Uneven cog profile Poor decision making o Poorer academic performance Failures o Behavioral dysregulation Inattention Impulsivity o Depression o Anxiety o Low self-esteem o Reduced QOL
What are the treatments for narcolepsy in pediatrics?
stimulants
antidepressants for cataplexy, etc.
What is the role of BSM in narcolepsy?
Education Improve sleep habits structured/planned naps relaxation as indicated CBT/ACT Patients have expressed interest in BSM services
How should structured naps be carried out in narcolepsy and pediatric population?
15 min duration
Schedule between 12:30pm-5pm
depending on severity, may need 2
School consultation for 504 planning
What is the spectrum of SDB?
No snoring Primary snoring/ "mild SDB" OSA, mild OSA, mod OSA, severe OSA + hypoventilation
What moves a person down the SDB spectrum?
weight gain
alcohol
hypnotics
sleeping pills
What are the Central Disorders of Hypersomnolence?
Secondary/associated:
Insufficient sleep syndrome
Hypersomnolence due to a medical disorder
Hypersomnolence due to medication/substance
Hypersomnolence associated with psychiatric disease
Recurrent:
Kleine-Levin syndrome
Primary and persistent:
Narcolepsy Type 1
Narcolepsy Type 2
Idiopathic hypersomnia
What is insufficient Sleep syndrome?
ICSD-3 criteria
Behaviorally induced, not really a central disorder of hypersomnolence
Daily sleepiness (in preteens, can be behavioral problems due to sleepiness)
Sleep shorter than expected for age
Sleep pattern present most days x 3 months at least
Uses measures to shorten sleep (alarm, person); sleeps longer w/o these measures
Sx resolve with sleep extension, but takes some time to see results!
Not better explained
Is behavioral issue fundamentally!
Large piece of differential dx in workup for hypersomnolence
Hypersomnia due to a medical disorder (ICSD-3)
Daily sleepiness x 3 months
Not due to other sleep d/o, psych cond, or medication
If MSLT done, MSL “usually” <8 min. With 0-1 SOREM
Implies the medical condition is causal to sleepiness
What are the medical conditions commonly implicated in hypersomnia?
Medical: Metabolic encephalopathies (hepatic) Systemic inflammation (rheum, cancer, chronic infection) Genetic syndromes (Niemann Pick type c, prader-willi, fragile X) Endocrine disease (hypothyroidism)
Neurological: Parkinsonism Myotonic dystrophy TBI Insult to hypothalamus, bilat. Thalamus, midbrain (stroke, tumor, sarcoidosis)
What is the criteria for Residual Sleepiness after OSA treatment as dx of hypersomnia due to a medical condition?
Requires FULLY ADEQUATE treatment of OSA
3 mos or more
PAP download showing 7+ hrs of use/night
PSG showing control of AHI at prescribed settings (Don’t rely on AHI from CPAP machine if possible)
Hypersomnia due to drug/substance
Dx requires daily sleepiness, no duration criteria
Caused by addition of sedating medication or withdrawal of alerting medication
Not better explained by
Which medications are involved with Hypersomnia due to drug/substance?
Sedative-hypnotics (Rx or Non)
Neurological: anti-epileptics, dopamine agonists
Psychiatric: antidepressants, antipsychotics (dopamine antagonists), Benzos/barbiturates
Anticholinergics
Antihistamines
Muscle relaxants/pain medications
Anti-arrhythmias
beta-blockers
Also includes substance abuse of sedating meds and d/c of wake-promoting meds
Hypersomnia Comorbid to psychiatric d/o (ICSD3)
What disorders?
Most commonly: Mood disorders Atypical depression Bipolar2 SAD Anxiety d/o
Somatoform d/o
Less commonly:
Thought d/o
Adjustment d/o
Personality d/o
What is the ICSD-3 criteria for Kleine-Levin Syndrome?
At least 2 episodes of Excessive sleepiness (2 days-5 weeks, median duration is 13 days)
Recurrences once every 18 mos (usually more than once per year)
Median frequency every 3-6 months
Median duration 15 years
Triggers: infection, ETOH, sleep deprivation, stress, head trauma
In addition to excessive sleepiness, bouts must demonstrate at least one of:
cognitive dysfunction
altered perception (de realization, depersonalization)
disordered eating (anorexia or hyperphagia)
disinhibited behavior
Normal alertness, cognition, behavior and mood between bouts
No MSLT criteria, no requirement to measure sleep duration
Kleine-Levin syndrome, typical episode symptoms
Neurological sx always present: hypersomnia (15-22 hr/d) Altered cognition (mental slowness, confusion, post-episode amnesia) Neurological sx occasionally present: Meningitis like headache photophobia painful hyperacousia Neuropsychiatric derealization/altered perception (always) Apathy (always) disinhibition (megaphagia, hypersexuality), occasionally present Regressive behavior, puerility (occasionally) Psychiatric irritability (always) depressive or flat mood anxiety Psychotic symptoms reference ideas hallucinations delusion Derealization >90% Apathy 100% Hyperphagia/increased intake 66% Hypersexuality: boys 58% girls 35%
What is the epidemiology of Klein-Levin Syndrome?
Rarest of central d/o of hypersomnolence 1-5/million Onset typically age 12-20 2/3 of patients are boys ~5% have FH of KLS
