This post is about a very unusual experience that occurs to a lot of kids. I thought I would write about it from that perspective. Consider the young boy in the photo at the top of this post from the mid-1950s. He would be the oldest of 5 children born to a working-class couple and this was their first home. For 2 years around the time of this photo very unusual events would occur frequently at night.
On a regular basis – he would wake his parents up - screaming.
What he could not articulate very well
at the time was that he would see an old woman entering his room at night. She walked through a narrow door from an
adjacent room rather than a large door connecting the main rooms. She had
either very long hair or headwear that accentuated her pale complexion. She had a threatening facial expression. She did not say anything or make any noise –
but walked silently closer to the boy.
With each step he got more anxious and scared. At some point he started screaming and did
not stop until his mother or father came into the room to see what was the
matter. Even then he was unconsolable. He
could only piece together that sequence of events after it had happened many (tens)
of times.
His parents typically found him flushed, sweating, with
his heart pounding. It took him about 10
minutes to recover and no matter what they did – they could not speed the
recovery process along. They also could
never figure out what was bothering him.
He could never make a clear description even after the recovery period.
Over repeated incidents, and being asked the same
questions the apparition became known as the Death-a-lo. It was a name that
came naturally to him, but his parents did not know what to make of it. They did not understand what he was
seeing. They knew there was nothing in
the room. They were also concerned that he was making so much noise at night
that he may be waking the neighbors. One
day they were walking with him in a public area and 3 Catholic nuns walked by
and he pointed to them and shouted: “Death-a-los”. It was not clear if he was communicating a
fear of nuns or a resemblance to the hallucination. When the boy was older – he
noticed a chalk drawing of a woman at his grandmother’s house that also resembled
the hallucination. In the small town
where the family lived – problems like this never really came to the attention
of physicians. There were no
pediatricians or psychiatrists. All
medical care was done by primary care physicians.
Eventually those nocturnal hallucinations and
hyperarousal resolved. This boy went on
to become a neurotic child preoccupied with somatic concerns and premature
death. For several years, he was concerned that he had a fatal illness –
typically cancer or a lethal infection – usually rabies. He was anxious about
doing something wrong and making sure that he did things right. Even though the hallucinations and night
terrors were gone – he had lifelong insomnia and would lay awake late into the
night. He constantly thought about things – thoughts of the past, things that
had happened, what the future might be like, important things he learned in
school, potential catastrophic events, and illnesses. He was eventually able to put the insomnia to
good use. He could study all night long
and was able to think creatively rather than just worry.
This vignette is a description of sleep terrors or night
terrors. In the current psychiatric and
sleep nomenclature (1,2), it is classified under the Non-Rapid Eye Movement (NREM)
Sleep Arousal Disorders of the Parasomnias.
The characteristic feature of NREM Sleep Arousal Disorders is abnormal
events occurring during incomplete arousals during the first third of the sleep
cycle (slow wave sleep). The main
disorders are sleep walking and sleep terrors. About 1/3 of children have had
at least 1 episode of sleep walking and roughly the same for sleep terror
episodes at 18 months of age. Sleep
terror episodes diminish with age to only about 2.2% of adults. The prevalence
of sleep terror disorder (recurrent episodes leading to distress and
impairment) in adults and children (1-14%) varies widely depending on the methodology
used for estimates (3).
There are potentially many etiologies of NREM sleep arousal
disorders in prepubertal children, but it is probably best conceptualized as a
developmental phase that will resolve with little to no intervention. Some research papers will list algorithms to
capture rare etiologies, but expert guidelines (4) suggest a detailed history
to determine the features of the episode with a medical and full neurological
examination. More extensive testing is
indicated only if there are abnormal findings or further differentiation is
needed from sleep associated epilepsy or other parasomnias. Differentiation from nightmares is not difficult
based on timing, more clear recall of the nightmare event, and the lack of
marked autonomic arousal.
Like most disorders of consciousness, there is no clear mechanism. At the level of clinical neurophysiology – sleep EEGs will clearly show a transition from stage 3 and 4 slow wave sleep (N3) to a period of hyperarousal with increased muscle tone/movements, tachycardia, and hyperventilation. I currently have permissions to display 2 EEGs of sleep terrors pending and will post them if I get those permissions.
At the pathophysiological level, these arousals are like
sleep inertia or incomplete arousals from slow wave sleep. They can be induced by forced awakening of
people in slow wave sleep. Sleep
disorders, sleep deprivation, circadian rhythm disturbances, physical
illnesses, physical stimuli (excessive environmental noise, bladder distention,
sleep disordered breathing), and prescription or non-prescription drugs that
affect sleep states can all precipitate these episodes. The developmental form is more likely to
persist and no specific etiology is typically determined.
Sleep problems are very common in populations with
psychiatric disorders. That insomnia and
some of the medications prescribed for primary psychiatric disorders can
trigger NREM arousals and that should be part of the ongoing dialogue with any
patients who are being treated. Some
studies indicate that these sleep problems are more likely in family members
who have first degree relatives with psychiatric disorders, sleep disorders,
and sleep disordered breathing. In the
care of the patient discussed above – his father probably died from sleep apnea
and he was eventually diagnosed with severe sleep apnea at age 55 and has been
using CPAP ever since.
There has not been a lot of extensive work done looking at
subsequent psychopathology and NREM arousal disorders. A study from 1980 (8) is
still quoted suggesting that the sleep terror group is more likely to
experience anxiety, depression, obsessive-compulsive tendencies and inhibit
outward expressions of aggressions. There has been very little work done on the
phenomenology of sleep terror episodes – most likely since episodes are
associated with amnesia. The Death-a-lo descriptions was possible only after
many episodes over a period of at least one year.
Like most disorders, there is a wide range of severity with
both sleep walking and sleep terrors.
Consultants will typically ask about the safety of the sleep environment
and make suggestion where necessary. All
the previously mentioned factors that can lead to the arousal can be modified. There
are environmental, behavioral, and pharmacological interventions
(benzodiazepines, antidepressants, melatonergic agents). The impression I get from reading the current
literature is that the need for pharmacological intervention is rare. That is probably expected when the disorder
is time limited, environmentally sensitive, and can be treated with anticipatory
or scheduled awakenings. After the
usual time of the disturbance has been determined – the parents wake the child
up about 30 minutes before the event. That is described as being as effective as
medications – but whether a non-treatment comparison was done is unknown.
In the case of the boy in the vignette, he is now in his 70s. He has had no parasomnias since this
pre-pubertal episode. A few years later
he did have an episode of severe visual hallucinations that occurred due to a
febrile illness, but he has not been diagnosed or treated for psychiatric
problems – apart from the lifelong insomnia and obstructive sleep apnea.
I basically did OK!
George Dawson, MD, DFAPA
References:
1: Diagnostic and
Statistical Manual of Mental Disorders: DSM-5. 5th ed., American Psychiatric
Association, 2013. DSM-V, doi-org.db29.linccweb.org/10.1176/ appi.
2: American Academy
of Sleep Medicine. International classification of sleep disorders, revised:
Diagnostic and coding manual. Chicago, Illinois: American Academy of Sleep
Medicine, 2001.
3: Leung AKC, Leung
AAM, Wong AHC, Hon KL. Sleep Terrors: An Updated Review. Curr Pediatr Rev.
2020;16(3):176-182. doi: 10.2174/1573396315666191014152136. PMID: 31612833;
PMCID: PMC8193803.
“Sleep terrors typically occur in children between 4 and
12 years of age, with a peak between 5 and 7 years of age. It is estimated that
sleep terrors occur in 1 to 6.5% of children 1 to 12 years of age, although a
prevalence of 14% or higher has also been reported. The wide variation in
prevalence can be attributed to differences in definitions of a sleep terror,
methodology, and studied population. The lifetime prevalence of sleep terrors
has been estimated to be approximately 10%. The condition is uncommon after
puberty. In the pediatric aged group, the condition is more common in boys than
in girls. In the adult population, both sexes are equally affected.”
4: Avidan AY. Disorders of arousal. In: Kryger
M, Roth T, Goldstein CA, Dement WC.
Principles and Practice of Sleep Medicine. 7th ed. Philadelphia,
PA: Elsevier, Inc, 2017: 1071-1086.
5: Guilleminault C,
Palombini L, Pelayo R, Chervin RD. Sleepwalking and sleep terrors in
prepubertal children: what triggers them? Pediatrics. 2003 Jan;111(1):e17-25.
doi: 10.1542/peds.111.1.e17. PMID: 12509590.
6: Loddo, G., Lopez,
R., Cilea, R. et al. Disorders of Arousal in adults: new diagnostic tools for
clinical practice. Sleep Science Practice 3, 5 (2019). https://doi.org/10.1186/s41606-019-0037-3
7: DiMario FJ Jr,
Emery ES 3rd. The natural history of night terrors. Clin Pediatr (Phila). 1987
Oct;26(10):505-11. doi: 10.1177/000992288702601002. PMID: 3652596.
8: Kales JD, Kales A,
Soldatos CR, Caldwell AB, Charney DS, Martin ED. Night terrors. Clinical
characteristics and personality patterns. Arch Gen Psychiatry. 1980
Dec;37(12):1413-7. doi: 10.1001/archpsyc.1980.01780250099012. PMID: 7447622.
“Both groups had high levels of psychopathology, with
higher values for the night terror group. These sleepwalkers showed active,
outwardly directed behavioral patterns, whereas the night terror patients
showed an inhibition of outward expressions of aggression and a predominance of
anxiety, depression, tendencies obsessive-compulsive/, and phobicness. Although
night terrors and sleepwalking in childhood seem to be related primarily to
genetic and developmental factors, their persistence and especially their onset
in adulthood are found to be related more to psychological factors.”
