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Neurol Sci (2005) 26:s205s209

DOI 10.1007/s10072-005-0488-4

L. Fusco N. Specchio

Non-epileptic paroxysmal manifestations during sleep in infancy


and childhood

Abstract Sleep-related paroxysmal disorders in infancy


and childhood have recently been reviewed and classified
by the American Sleep Disorders Association. Although in
some patients diagnosis is easy to achieve, at times a
video/EEG recording of the episodes is required because
of the similarity to epileptic seizures. These disorders
often represent a challenge for paediatricians as an accurate diagnosis is required. We report a review of the literature and a characterisation of our series of patients.
Paroxysmal events during sleep are frequently misdiagnosed as psychogenic fits, epileptic seizures or physiologic events with evident disadvantages for children and families. The widespread use of neurophysiological techniques and better knowledge of the semeiology of epileptic seizures have led us to correctly define these events,
which have sometimes been erroneously treated in the
past.
Key words Childhood parasomnias Childhood epilepsy
Sleep terrors Pavor nocturnus Frontal lobe seizures

L. Fusco () N. Specchio


Division of Neurology
Bambin Ges Childrens Hospital
Scientific Institute
Piazza S. Onofrio 4, I-00165 Rome, Italy
e-mail: fusco@opbg.net

Introduction
Epileptic seizures and parasomnias represent the two
major groups of sleep-related paroxysmal disorders in
childhood. Usually epileptic seizures can be easily identified by random occurrence, stereotyped behaviour, brief
duration and, when present, motor stiffness and clonic
rhythmic movements. Movement disorders and paroxysmal behaviour belonging to parasomnias are at times more
difficult to recognise.
Parasomnias have been defined as a group of clinical
disorders associated with undesirable physical phenomena
that occur predominantly during sleep [1]. They represent
partial activation of the central nervous system producing
skeletal muscle activity. Some are associated with violent
motor and autonomic activity, whereas others have minimal muscle disturbance. Some parasomnias, such as nightmares and hypnic jerks, are so common that most individuals have experienced them.
A great number of parasomnias are more common in
childhood than in adult people: Laberge et al. [2] found,
among 1353 subjects aged between 3 and 13 years, that
78% experienced at least one episode of parasomnias.
The International Classification of Sleep Disorders [1]
divides parasomnias into four categories, which are
defined by the phase of the sleep in which they occur:
arousal disorders, sleep-wake transition disorders, parasomnias usually associated with REM sleep and other
parasomnias which could occur either in the REM or
NREM sleep.
In clinical practice, paediatricians are, not infrequently,
asked to diagnose such nocturnal paroxysmal events,
which could be misdiagnosed as epileptic seizures, psychogenic seizures or physiologic events.
The need to classify them implicates that they have to
be recorded by a Video-EEG and polysomnographic technique [3], either if they are suspected to be epileptic or to
be parasomnias. On the other hand hyperkinetic seizures in

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L. Fusco, N. Specchio: Non-epileptic paroxysmal manifestations during sleep

Fig. 1 Neonate aged 17 days. During


quiet sleep repetitive myoclonic jerks
have been recorded without epileptiform EEG counterpart (modified
from [6])

children can be difficult to recognise because of the similarity of the clinical manifestations with non-epileptic
events such as parasomnias and pseudoseizures: also in
such cases Video-EEG appears as the most effective procedure for the correct diagnosis of this type of seizure [4].
We describe a series of patients referred to us because
of the presence of paroxysmal non-epileptic events occurring during sleep.

Benign neonatal sleep myoclonus


Benign neonatal sleep myoclonus (BNSM) is a phenomenon characterised by the occurrence of repetitive
myoclonic jerks during NREM sleep in the early life of
healthy newborns [5]. It is recognised as a non-epileptic
paroxysmal event and video-polygraphic recording of ictal
phenomena helps the diagnosis [6]. BNSM is included in
the fourth category of the International Classification of
Sleep Disorders [1] defined as other parasomnias, in
which disorders entirely benign such as BNSM are associated to other dramatic events such as unexplained nocturnal death, REM-related sinus arrest and homicidal somnambulism [7].
Because myoclonic manifestations in a newborn are
usually warning symptoms and may indicate brain damage
or progressive encephalopathy, the recognition of this
benign form of myoclonus will avoid unnecessary diagnostic tests and possibly harmful treatment. In a previous
paper [6] we reported about 12 newborns with BNSM who
were referred to us for investigations of convulsion and
since then many children been diagnosed with BNSM.
Usually the neonates are born after normal pregnancy and
delivery and none has a family history of convulsions,

Repetitive jerks

Quiet sleep

epilepsy or sleep disturbances. The age at onset of


myoclonic jerks during sleep ranges from 1 to 15 days.
The main feature of the myoclonus is that the jerks end
with awakening. All neonates are neurologically normal.
Video-polygraphic EEG recording shows that myoclonus
occurs only during NREM sleep and is characterised by
synchronous jerks at a frequency of four to five per second
(Fig. 1). Bouts recur irregularly in series lasting 2030
min. If the infant is woken or the sleep phase is changed,
myoclonus stops. The distribution of myoclonus is usually generalised, although the arms are involved more than
the legs. At times the jerking spreads progressively to
other muscle groups. At times myoclonus is segmentary,
involving only one limb or one side of the body. Facial
myoclonias are rare. Myoclonus is rhythmic and sometimes so intense and frequent as to provoke shaking
throughout the body. EEG is always normal, as is psychomotor development of the infants during the follow-up.

Repetitive sleep starts


Sleep starts, also known as hypnagogic or hypnic jerks,
are bilateral, brief body jerks that coincide with sleep
onset. Usually they occur during the transition from wakefulness to sleep and may be considered a physiological
accompaniment of falling asleep. They are included in the
category of sleep-wake transition parasomnias by the
International Classification of Sleep Disorders [1]. At
times sleep starts may be excessive, and configure a true
sleep disorder, characterised by frequent awakenings and
sleep onset difficulty. Excessive sleep starts have been
reported in patients with Parkinsonism [8] and in children
with migraine [9]. Excessive sleep starts occurring in

L. Fusco, N. Specchio: Non-epileptic paroxysmal manifestations during sleep

clusters at the onset of sleep have already been reported by


us [10] in neurologically impaired children. We observed
and described the repetitive occurrence of sleep starts in
epileptic children with spastic-dystonic diplegia and cognitive deficits. Usually the children who present with
repetitive sleep starts have tetraparesis, from light to very
severe, both with pyramidal or extrapyramidal features.
Often they previously had a diagnosis of epilepsy in the
first year of life, sometimes West Syndrome or more often
focal motor epilepsy. The onset of repetitive sleep starts is
in the second year of life. The parents came to the physician because of the appearance of multiple spasms during the initial phase of sleep, which have usually been
believed to be a new type of epileptic seizure. Video/EEG
recordings of the events during sleep show the presence of
clusters of massive myoclonic/tonic contractions, resembling sleep starts, in the transition phase between wakefulness and sleep stage I, with the EEG counterpart being
unremarkable. The clusters last from a few to 15 minutes.
The duration of each muscle contraction is from 500 ms to
5 s, depending on the rapidity of the contraction, which
can be more or less tonic. No epileptiform pattern is
observed corresponding to the jerks and often an arousal
response can follow the jerks (Fig. 2). The interictal EEG
is abnormal in most of the children, usually with poorly
organised background activity with a lack of regional differentiation, and the possibility of multiple spike activity,
as they have static encephalopathy and epilepsy. Although
physiological sleep starts are usually a non-periodic event,
in these cases the clinical and EEG characteristics of every
single event permits their definitive classification as sleep
starts. Indeed, muscle contraction is massive, involving
more trunk than limbs, and onset is sudden and usually
spontaneous, but can be induced by acoustic and tactile
stimulation. Clinical characteristics are similar to a startle,
although duration exceeds that usually accepted for physi-

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ological sleep starts. Even in normal subjects sleep starts


can occur in a brief cluster, due to the physiological oscillation between sleep and wakefulness during the period of
falling asleep. In neurologically impaired subjects, this
oscillation can be enhanced by the lack of physiological
inhibition of the pyramidal tract, due to the pyramidal
lesion. Parents suggested that the phenomenon can be limited by the prone position, and this position is now recommended to parents, more than drug therapy. The prone
position can limit movement itself, thus limiting arousal
due to movement and improving the quality of falling
asleep. If drugs are necessary, in cases of severe sleep disturbance and daily drowsiness, benzodiazepine appears to
be the drug of choice.

Rhythmic movement disorders


Rhythmic movement disorders are classified as a sleepwake transition disorder in the International Classification
of Sleep Disorders [1] and comprise head banging, head
rolling and body rocking.
All of these are rhythmic movements occurring on
falling asleep, and their maximum peak of expression is
during early development in normal children, although
they are usually seen more frequently in patients affected
by mental retardation [11]. This phenomenon usually
begins during the first year of life with an average age of 9
months and ends spontaneously by 4 years of age [12, 13].
Head banging and body rocking usually involve large
groups of muscles, usually the head, the neck and the trunk
[14]. The head is sometimes forcedly and repetitively banged
into the pillow. The movements typically occur just prior to
sleep onset, but continue into light sleep, and can recur during the whole night, always into light sleep. Movements are

Fig. 2 EEG polygraphic recording of four


spontaneous sleep starts during drowsiness.
The EEG counterpart is a diffuse movement
artefact

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L. Fusco, N. Specchio: Non-epileptic paroxysmal manifestations during sleep

Fig. 3 Child aged 5 years. Just before the


onset of movements, the child awakes.
Then, during the episode, the EEG shows
active wakefulness intermingled with muscle and movement artefacts

stereotyped and repetitive and last from several minutes to


hours, then end spontaneously and sleep is restored.
Irritability and daytime somnolence may occur. Children are
usually non-responsive during the attack and do not recall
the events on awakening. The video/EEG monitoring shows
that, just before the onset of movements, the child awakes.
Then, during the episode, the EEG shows active wakefulness
intermingled with muscle and movement artefacts (Fig. 3).

Arousal disorders
The arousal disorders include sleep-related paroxysmal
phenomena such as sleepwalking, sleep terrors and confusional arousals [1]. They have been considered as arousal
disorders based on the hypothesis that during the events,

an incomplete arousal occurs, due to an immature or disturbed arousal mechanism [15]. Sleep terrors are characterised by a confusional partial arousal, sudden screams, at
times in series, followed by incomprehensive language and
behavioural manifestations of intense fear; usually occurring at the end of the first slow-wave phase of NREM
sleep. The episode lasts several minutes, not unusually
more than ten. The child appears to not recognise his parents, and at times is afraid of them. Successive episodes in
the same night are not common and usually the interval
between the events is of months. The video/EEG recording
of one episode of sleep terror show on the EEG the persistence of delta slow activity at the onset of the event (Fig.
4a) and throughout (Fig. 4b), suggesting a persistent
asleep brain in an awakened body.
Sleep terrors, but also confusional arousal and sleepwalking, may be confused with epileptic seizures, especial-

Fig. 4 EEG recording of one episode of sleep terror in a 4-year-old child. The EEG shows the persistence of delta slow activity at the
onset of the event (a) and throughout (b)

L. Fusco, N. Specchio: Non-epileptic paroxysmal manifestations during sleep

ly with focal seizures involving the frontal lobes. Frontal


lobe epilepsy, both sporadic and familial forms, is characterised by mainly nocturnal seizures, with a bilateral hyperkinetic motor component. Seizures are at times so confused
that they can be misinterpreted as parasomnia phenomena.
The differential diagnosis is made on the basis of an accurate anamnesis, as epilepsy is characterised by stereotyped
seizures, more than one seizure per night and recurrence
nightly. At times epileptic children may have had nonepileptic phenomena in early childhood, such as sleep terror or confusional arousal. Definite criteria in differential
diagnosis between parasomnias and epileptic seizures have
been widely reported in the last few years [16].

Conclusions
Paroxysmal phenomena during sleep in childhood are a
common occurrence and usually depend on sleep disturbance, such as parasomnias. Otherwise, epileptic seizures,
especially with frontal lobe onset, have their maximum
expression during sleep, often with behavioural ictal manifestation, which could be confused with parasomnias.
Carefully collected anamnestic data and, in selected cases,
the video/EEG recording of the event, can be useful in differential diagnosis.

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