Sleep walking

BTS sleep Course
Module 2
Disorders of Sleep
(prepared by J Stradling)
•  What are sleep
disorders
•  Organisation of sleep services
•  Classification of sleep disorders
•  Non-REM disorders
•  REM disorders
•  Other sleep disorders
BTS sleep Course
Modules 1, 2 and 3
Acknowledgements
With many thanks to Richard Horner, Toronto University for
permission to use some material from his sleep course
Some images have come from
http://www.sleephomepages.org/sleepsyllabus
Some images came from internet searches via Google
Others have come from published articles/books
Most of the images are therefore copyright and cannot be used other
than for personal study
Others come from Prof J Stradling who wrote these modules
What is Sleep Medicine?
•  Study of disorders with a significant sleep related component
•  Is it any more valid than an awake medicine speciality?
•  Two camps – sleep issues belong to their host specialities
(e.g narcolepsy/neurology, OSA/respiratory)
– sleep issues require sleep specialists
•  Good arguments for both approaches, examples of
both/hybrids in the UK and abroad.
How common are sleep disorders?
•  1 in 7 people complain of a chronic sleep problem
•  1 in 3 complain intermittently of a sleep problem
•  One of the commonest reasons for presenting to GP
•  Sleep apnoea – between 1 and 4% adults, approx 1% in children
•  Restless legs – 10%
•  Sleep walking – 10%
•  Narcolepsy – 0.15%
•  Circadian disturbances – 3%
•  Sleep often disturbed by other problems, e.g. arthritis, depression
No shortage of work!
Division of sleep into stages
•  Many sleep disorders are
specific to either Non-REM or
REM sleep (eg leg movements)
•  Others can occur in any sleep
stage (eg sleep apnoea)
•  Some disorders are not really
related to sleep stage (eg jet lag)
Classification of
Sleep Disorders
!
Common disorders of Sleep
Non-REM -
Sleep Walking
Night terrors
Periodic leg movements
Sleep automatism
REM -
Nightmares
REM sleep behaviour disorder
Cataphrenia (REM sleep groaning)
Narcolepsy
Both -
Obstructive sleep apnoea, central sleep apnoea
Unrelated -
Circadian rhythm disorders
Drug effects
Sleep in other medical disorders (e.g. heart failure, depression)
Sleep walking
•  Subject recurrently arises from deep slow wave
sleep, typically 1st third of night, and shows
complex automatic behaviour including
leaving the bed and walking for some distance.
Minor violence is common (adult males). Usually
not remembered the following morning.
Common, 20% of children occasionally sleep
walk, 5% repeatedly
Vincenzo Bellini, La
Sonnambula (Amina
walking across a plank
above a water wheel)
•  Strong family history (DBQ1*05 association), no real psychiatric
association, worse at times of stress, worse with alcohol/caffeine.
Partial arousal disorder – the cortex remains asleep when the rest
of the brain wakens . Happens more when other arousing stimuli
occur – e.g. reported secondary to sleep apnoea and disappears with
its treatment. Some believe related to SWS/REM transition.
Sleep walking
This 9 year old girl sleep walks. This PSG tracing over about 30 seconds shows
SWS activity throughout a time when she sat up in bed, looked around with her
eyes open, and then lay down again (apparently awake). Awakened shortly
after (with difficulty) and had no memory of the event.
Persistent
slow wave
activity –
cortex
asleep
Sleep walking
This patient walked through a plate
glass door while sleep walking
Best managed by simply limiting ability to walk into dangerous
situations – chain lock on bedroom door, window locks, for example.
Wear pyjamas!
Night terrors
•  Sleep, or night, terrors consist of sitting up
during sleep, emitting a piercing cry, and
showing behavioural features of acute fear, as if
dreaming: e.g. tachycardia, dilated pupils,
tachypnoea, sweating.
•  However the child cannot be consoled, and
makes no sense. Will struggle if contained.
•  There is no dream imagery.
•  Similar aetiology to sleep walking – partial arousal disorder – cortex
still asleep. Usually aged 5 – 7 years.
•  Genetic component and no suggestion of psychiatric problem
•  They usually occur in the first batch of SWS and last up to 5 minutes
or so. May evolve into sleep walking.
Night terrors
•  The following morning the child has no memory of the event.
•  The main problem is that neighbours think the child is being hurt or abused.
•  The main approach is to reassure the parents that the child is not in any real
distress, is likely to grow out of it, and provide information that can be shown
to neighbours etc.
•  If going away for a short time, the terrors can be temporarily aborted by
waking the child 15 minutes or so before the usual time of the terror.
Diazepam works, but should be used intermittently and with extreme caution.
•  Often will not occur during hospital sleep study – lighter sleep.
•  Complex partial seizures (frontal/temporal) can be confused with night
terrors and sleep walking.
Periodic limb movements during sleep
and restless legs syndrome
Restless legs
•  Weird sensations coming from the legs, making the patient constantly
want to move their legs: itching , crawling , burning , relieved by
moving the legs – sometimes every few seconds. Worst in evening, or
on going to bed, or when relaxed/sleepy.
•  May fall asleep and wake with similar sensations that force them to
get up and walk around.
•  Severity varies greatly. Minor nuisance to major reduction in quality
of life.
•  Symptoms may fluctuate with time, affects about 5% of the
population.
•  During sleep these patients usually have periodic limb (usually leg)
movements every 40 seconds or so during non-REM sleep.
Periodic limb movements during sleep
and restless legs syndrome
Periodic movements of the legs during sleep is the sleep equivalent of restless legs.
Periodic limb movements during sleep
•  Thought to be due to increased nerve traffic from the legs causing
repeated withdrawal reflex, as if pinching the toe.
•  Can occur in paraplegics indicating it can be a spinal reflex.
•  Every 40 seconds, because level of sleep fluctuates with this
periodicity (so call cyclical alternating pattern), occurs at
lightest sleep level.
•  May or may not lead to cortical arousals.
•  May or may not lead to daytime sleepiness (usually symptomless,
especially in the elderly).
•  Often noticed by partner (may be kicked).
•  Cause unknown, related to many other disorders, genetic component.
•  Can arise after sleep apnoea treated (mechanism unknown).
Periodic limb movements during sleep
Periodic limb movements during sleep, management
History usually very helpful
•  Consider:- peripheral neuropathy, uraemia, haemodialysis, previous leg
neurology (e.g. sciatica).
•  Look for iron deficiency (low ferritin levels reported but rarely seen).
•  Sleep study: measured with leg EMG electrodes, leg actigraphy, or on video.
Treatment is nowadays with:•  Dopamine agonists such as Pramipexole (usually used for Parkinsons
disease and suppression of prolactin production).
•  Earlier drugs such as mild opiates (e.g. codeine), benzodiazepines
(clonazepam) may still have a role.
•  Others unproven, (carbamezepine, magnesium, gabapentin)
Sleep Automatism/automatic
behaviour/confusional arousals
Poorly characterised. Similar to sleep
walking but subject apparently able to do
much more complex activities e.g.
KENNETH PARKS CASE. A 23-year-old married Toronto man, was suffering from
severe insomnia caused by joblessness and gambling debts. Early morning, May 23
1987 he arose, got in his car and drove 23 kilometers to his in-laws' home. He
stabbed to death his mother-in-law with a carving knife. Parks also assaulted his
father in law with a tyre lever. He then drove to the police and said "I think I have
killed some people . . . my hands," only then realizing he had severely cut his own
hands requiring repair of several flexor tendons of both hands.
Because he could not remember anything about the murder and assault, had no
motive for the crime whatsoever, and did have a history of sleepwalking, his team of
defense experts (psychiatrists, a psychologist, a neurologist and a sleep specialist)
concluded Ken Parks was 'asleep' when he committed the crime, and therefore
unaware of his actions.
Homicidal somnambulism: a case report (Sleep 1994;17:253-64)
Common disorders of Sleep
Non-REM -
Sleep Walking
Night terrors
Periodic leg movements
Sleep automatism
REM -
Nightmares
REM sleep behaviour disorder
Cataphrenia (REM sleep groaning)
Narcolepsy
Both -
Obstructive sleep apnoea, central sleep apnoea
Unrelated -
Circadian rhythm disorders
Drug effects
Sleep in other medical disorders (e.g. heart failure, depression)
Nightmares
•  Nightmares are simply vivid and unpleasant dreams that distress the
sufferer. However upsetting dreams will not be remembered unless they
provoke awakening.
•  All of us suffer from them occasionally. Much more rarely these are
recurrent. More common in children (25%, defined as for >3 months).
•  This can lead to not wanting to go to sleep, or sleep refusal in children.
•  A particular theme is presumably repeatedly activated by the PGO
spikes of REM sleep. The more preoccupied the patient is with the
nightmare content, perhaps the more likely it is to be activated each
night.
•  Triggered by some drugs, e.g. catecholamines, β blockers (propranolol),
bupropion, barbiturate, benzodiazepine and alcohol withdrawal.
•  Associated with post traumatic stress disorder.
•  Dreams in Narcolepsy are often more disturbing than usual.
•  Treatment difficult. Relaxation techniques, cognitive behavioural
therapy, few days of tricyclic antidepressants before bed (REM
suppression, but beware rebound).
"The Nightmare" by
the Swiss-English
artist Henry Fuseli
(1741-1825). Notice
the demon seated on
the dreamer's chest
in this famous
painting. Two
similar paintings
exist.
REM behaviour disorder
The first series of cases of REM behaviour disorder was described in 1985
by Mark Mahowald, MD, and Carlos Schenck, MD, of the University of
Minnesota.
Due to damage of Jouvet s centre – leading to loss of REM atonia during
REM sleep – hence these patients act out their dreams (usually aggressive).
Cell
potential
Muscle
Cord
REM behaviour disorder
If woken – immediately aware of dreaming and can relate this – quite different
from night terrors.
A 77-year old minister had been behaving violently in his sleep for 20 years,
sometimes even injuring his wife.
A 60-year old surgeon would jump out of bed during nightmares of being attacked
by "criminals, terrorists and monsters."
A 62-year old industrial plant manager who was a war veteran dreamt of being
attacked by enemy soldiers and fights back in his sleep, sometimes
injuring himself.
REM sleep
with phasic
EMG activity
Gross
movements in
both arms and
legs
REM behaviour disorder
•  Diagnosis. History very typical. May occur infrequently, PSG or
video taping often negative. Prodrome of prominent limb movements
and talking (sometimes in response to others talking to them!). Often
spontaneously aggressive (compare with night terrors)
•  Strongly correlated with Parkinsons disease and multi-system
atrophy (Shy-Drager syndrome, a general degenerative disorder of
the brain, with autonomic failure + Parkinsons). May predate
evidence of these by years.
•  Treatment. Responds well to clonazepam, two hours before bed,
thought to encourage the fall in muscle tone during REM sleep.
Occasionally, tricyclic antidepressants help (desipramine). L-Dopa
may work.
•  Try and prevent harm to patient and others. Sleep alone, locked
windows, safety glass, minimal furniture.
REM behaviour disorder
Patient s wife complained of his violent behaviour at night.
Initial sleep study showed only OSA. This sleep study was
during his first night on nasal CPAP (hence the loud hissing).
Cataphrenia (REM groaning)
•  A loud and prolonged expiratory groan during REM sleep.
•  Each groan can last over a minute, followed by a short
inspiration, and the groan repeated.
•  Horrendous noise that is most distressing to the listener
but not the perpetrator!
•  Produced by tightly apposing the vocal cords, raising
intrathoracic pressure, and forcing air out with noisy
consequences
•  Occasionally confused with snoring, leading inappropriate
advice
Cataphrenia (REM groaning)
This man had been labelled a snorer and was listed for a UPPP
despite the wife s description of a noise different to snoring
Pause – questions so far
Narcolepsy
Excessive sleepiness due to a neurological condition caused by
failure of neurotransmission in a tiny subset of brain neurones
(orexin/hypocretin). In humans due to damage, probably from a
virus infection if the right genetic susceptibility - HLA
DQB1*0602
These neurones, 60,000 or
so, in the hypothalamus, are
responsible for some aspects
of brain alertness and also
involved with the control of
muscle tone
Tail end
Rat brain
Projections of the hypocretin (orexin) system (A), to cholinergic neurons,
reticular formation and spinal cord; (B), to thalamus and basal ganglia; (C), to
basal forebrain; (D), to amygdala and dopaminergic neurons including
suprachiasmatic nucleus; (E), to locus coeruleus. Thought to promote arousal
and limit the REM atonia reflex.
Narcolepsy
Post mortem analysis of numbers
of orexin neurones in patients
who had narcolepsy, and matched
control subjects
Cerebrospinal fluid hypocretin-1 levels (direct
assay) across various disease categories
Controls
Narcolepsy/
hypersomnia
Other sleep
disorders
Mignot Arch Neurol 2002;59:1553-1562
Group with clear cataplexy
one dot = one patient
Narcolepsy
The loss of these neurones leads to a variety of consequences:1) 
2) 
Excessive daytime sleepiness – loss of alerting neurones
Loss of control of REM sleep and its associated atonia (loss of muscle tone)
This causes:3) Random insertion of REM sleep into sleep, which fragments sleep
and leads to sleep onset dreaming
4) Fragmentation of Non-REM sleep and more daytime sleepiness
5) Sleep paralysis – continuation of REM atonia after waking up
(although can occur in normal subjects)
6) Cataplexy – sudden onset loss of muscle tone during wakefulness
in response to intense emotions, such as a good
laugh, great
anticipation, etc. Varies from a slight drooping of
the face
and neck to apparently passing out and
falling on the floor.
The histories from these patients are fascinating, such as confusions between
dreaming and reality, alien visitations, evil presences, seeing bizarre animals
whilst drowsy, being labelled hysterical due to passing out whenever
emotional etc.
Sleep patterns in Narcolepsy
Narcolepsy
Hypnagogic hallucinations,
another term for dreams that
occur at sleep onset during the
interface between wake and
sleep.
Sleep paralysis can occur as an
isolated and inherited
condition or when sleep is
greatly disrupted (shift workers
etc). It is not always a sign of
narcolepsy.
Peak age of onset is late
adolescence/early adulthood
Cataplexy in Dogs
Due to inherited abnormality of the orexin receptor, therefore not
like human narcolepsy, and present from birth
The commentator describes Rusty
falling asleep, this is incorrect – the
dog is awake during these episodes
of cataplexy
Cataplexy in Humans
Narcolepsy
Diagnosis (mean delay from 1st symptom to diagnosis is 12-15 yrs)
History – cataplexy virtually diagnostic.
HLA typing – DQB1*0602, more useful to exclude diagnosis.
MSLT – looking for excessive sleepiness (sleep onset < 8 minutes); and early onset REM
(within 15 minutes) on two or more occasions out of four/five opportunities to sleep
(significant false positive and false negative result).
Sleep study – largely unhelpful except to rule out other cause (OSA, PLMS).
CSF orexin levels – not yet widely available, false positive and false negative result rate
not known yet.
Treatment (often tricky)
Sleepiness –
non pharmacological – scheduled naps, regular sleep/wake cycle.
pharmacological – amphetamines, modafanil, trycyclics.
Cataplexy –
tricyclics (clomipramine, voloxazine, imipramine), fluoxetine.
New drug for cataplexy and probably sleepiness too - gamma hydroxybutyrate
Gamma Hydroxy Butyrate
Median % reduction in cataplexy
Gamma hydroxybutyrate.
(Sodium Oxybate, Xyrem)
Related to GABA, specific brain
receptors.
Short half life – taken before bed and 4
hours later during the night.
Absolute reduction in cataplectic attacks/wk
Simple to make but very
expensive!
GHB
Gamma hydroxybutyrate – rapidly becoming a
drug of abuse – liquid ecstasy , date rape
GHB overdose (50g+) can cause
unconsciousness, slowed heart rate, respiratory
depression, seizures, hypothermia, nausea,
vomiting, and coma (was used as a general
anaesthetic agent).
Number of patients treated for
GHB overdose or GHB-related
problems in hospital
emergency departments (US)
during 1992 to 1999 (usually
admitted unconscious,
disorientated, aggressive and
ataxic).
Common disorders of Sleep
Non-REM -
Sleep Walking
Night terrors
Periodic leg movements
Sleep automatism
REM -
Nightmares
REM sleep behaviour disorder
Cataphrenia (REM sleep groaning)
Narcolepsy
Both -
Obstructive sleep apnoea, central sleep apnoea - SECTION 3
Unrelated -
Circadian rhythm disorders
Drug effects
Sleep in other medical disorders (e.g. heart failure, depression)
Common disorders of Sleep
Non-REM -
Sleep Walking
Night terrors
Periodic leg movements
Sleep automatism
REM -
Nightmares
REM sleep behaviour disorder
Cataphrenia (REM sleep groaning)
Narcolepsy
Both -
Obstructive sleep apnoea, central sleep apnoea
Unrelated -
Circadian rhythm disorders
Drug effects
Sleep in other medical disorders (e.g. heart failure, depression)
Circadian rhythm problems
The body has an
intrinsic 25hr cycle
that is locked to the
outside world
through blue light
levels, bringing it
down to the correct
24hrs.
Neural and humoral (melatonin) signals
signal to the rest of the body where they
should be in the cycle. Body temperature is a
good marker of where in the cycle the body
thinks it should be .
Circadian rhythm problems
If the normal cycle is
disrupted, then the body
becomes out of sync
with the environment.
This means the
circadian cycles are in a
sleep phase when the
individual is trying to
function.
Westwood
flight – day
elongated
by 8 hours
Eastwood
flight – day
shortened
by 8 hours
This leads to sleepiness whilst trying to work and
insomnia when trying to sleep. This sleep
deprivation just makes things worse. It may take
many days to sync to the environment again.
Melatonin may help a bit
Reduced
performance
taking longer
to recover
after eastwood
flight
Phase advance and phase delay syndromes
Accentuation of morning larks (early birds) and night owls
Short (<24hr) sleep cycle
– tends to go to bed
relatively early, and to be
up very early. Poor
evening performance.
Long (>24hr) sleep cycle
– tends to go to bed
relatively late, and to get
up late. Poor morning
performance and get fired
for turning up late for
work.
Melatonin can help.
Shift work
•  No shift work schedule is ideal. The best is permanent nights
with no return to normal cycle during days off.
•  Shifts that come on 8 hours earlier each week, or every 5 days,
are highly disruptive (like flying back from the US each time).
•  Shifts that come on 8 hours later each week are better – more
easily accommodated (like flying to the US each time).
•  Many other shifts supposedly better – dipping into nights for 2 or
3 days so as not to become acclimatised .
•  Real source of sleepiness/accidents on the job.
•  Harder to cope with as get older.
•  Shift workers less healthy.
•  Melatonin/bright lights can help.
Shift work
Management if not coping.
•  May have to stop doing shift work – financial penalty.
•  May be able to alter the pattern – usually impossible.
•  Encourage sleep during the day with quiet, dark environment.
•  Encourage alertness during the night with bright light and
moderate use of caffeine.
•  Melatonin may help if taken prior to the daytime sleep.
Sleep and drugs
An enormous number of prescription drugs can affect sleep.
Increase sleepiness – e.g. sedatives, antihistamines, antidepressants
Increase agitation/alertness – e.g. steroids, appetite suppressants
Increased dreaming/nightmares – β blockers (especially propanolol)
A full drug history, including
over the counter and herbal
remedies, needs to be taken in the
sleep clinic.
Pharmacists
labels
Recreational drugs and sleep
Alcohol, nicotine and caffeine can all alter sleep, both
separately and in combination
Nicotine – low doses cause mild sedation, higher doses cause
arousal and agitation. Half life of nicotine is 1 to 2 hours.
Alcohol – is a brain sedative.
Increases slow wave sleep and
reduces REM sleep.
Metabolised quickly, greatly
reduced blood levels after 3 to
4 hours. Rebound alertness.
Therefore alcohol helps to
promote sleep but fragments it
later on in the night, but with
slightly increased REM sleep.
Lighter, more
fragmented
sleep
Deeper SWS
Recreational drugs and sleep
Alcohol – also causes suppression of upper airway muscles –
increased snoring and OSA
Normal
overnight oxygen
levels with no
alcohol
Evidence of
obstructive sleep
apnoea early on
in the night with
alcohol
Issa and Sullivan 1982
Recreational drugs and sleep
Caffeine – causes increased alertness. Widely available in food/drinks,
and in over the counter medications (e.g. Pro-Plus, 50mg)
Long half life in the blood – 15hrs approximately
Therefore
even
afternoon
coffee will
give a
significant
blood level
during sleep
Recreational drugs and sleep
Caffeine – reduces sleep quality and the amount of SWS. Increases
alertness, probably acting as an adenosine antagonist (adenosine build
up thought to cause sleepiness) and thus slowing process S .
Less SWS
Rebound following night
Recreational drugs and sleep
Caffeine – present in many foods and drinks. 50mg has a significant
pharmacological effect, 100mg has a noticeable alerting effect and also
raises blood pressure acutely.
Caffeine in canned
Small serving
drinks (whole can)
Red Bull
80mg
Coca Cola
34mg
Diet Coke
45mg
Pepsi Cola
38mg
Dr Pepper
41mg
Jolt
71mg
7-Up
none
Recreational drugs and sleep
Caffeine and
alcohol together
Initially counter act –
then later on in the
night, alerting effects
are additive – don t
expect to sleep well
after a good dinner party
with plenty of wine and
freshly brewed coffee!
Sleep in other medical disorders
Many medical disorders impair sleep, e.g.
•  Pain from any cause, cancer, arthritis etc.
•  Renal failure – PLMS
•  Parkinson s disease
•  The drugs used
Particularly disturbed if causes central sleep apnoea
•  Heart (left ventricular) failure
•  Neuromuscular weakness involving the inspiratory
muscles
Sleep in heart failure
Heart (left ventricular) failure. Generates unstable
breathing with multiple arousals.
Ventilation gently waxes and wanes
Sleep in neuromuscular disease
•  When the inspiratory muscles fail, other muscles come in
to help – accessory muscles of respiration .
•  Unfortunately these drop out with sleep onset, and even
more so in REM sleep.
•  This leads to central apnoeas/hypopnoeas, hypoxic dips,
and recurrent arousal.
Worst during REM sleep
SaO2%
Transcutaneous
PCO2 (mmHg)
Duchenne
dystrophy
patient
Time of night
Conclusions
•  Sleep interacts with almost all aspects of human health
and disease. Sleep histories should be a part of
routine history taking – much can be learnt.
•  An understanding of sleep, and why disorders interact
with it, are essential to providing an all-round
sleep service for patients.
•  Furthermore, sleep and its disorders are fascinating,
every one is interested in sleep – you will never
want for dinner party chat topics ever again!