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Episodic nocturnal wanderings responsive to anticonvulsant drug therapy.

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Episodic Nocturnal Wanderings
Responsive to Anticonvulsant Drug Therapy
Timothy A. Pedley, MD, and Christian Guilleminault, MD
~~~~
Six patients between the ages of 17 and 32 years presented with unusual sleep-walking episodes characterized by
screaming or unintelligible vocalizations; complex, often violent automatisms; and -ambulation. Two or more attacks
could occur in a single night and were most common in the early morning hours. Family and personal histories did not
show epilepsy, psychiatric disorders, or (with 1 exception) previous somnambulism. Electrographic investigation
revealed that all patients had normal waking-sleep cycles but that 4 of them had epileptiform abnormalities on their
electroencephalograms. All patients were treated with either phenytoin or carbamazepine, with cessation of the
abnormal nocturnal behavior during follow-up periods ranging from 9 to 48 months. This syndrome appears to be
distinct from more typical non-rapid eye movement dyssomnias and suggests an atypical form of epilepsy.
Pedley TA, Guillerninault C. Episodic nocturnal wanderings responsive to anticonvulsant drug
therapy. Ann Neurol 2:30-35, 1977
Sleepwalking in children has been investigated extensively during the past decade. A number of studies
have demonstrated that somnambulism before puberty is not a manifestation of epilepsy [l-41. O n the
other hand, little information is available about
complex, abnormal behavior occurring during sleep
in adolescents and adults. I t has frequently been
assumed that somnambulism in this older population is related to a disorder of non-rapid eye
movement (NREM) sleep, analogous to clinically
similar dyssomnias in children. Since the somnambulistic wanderings in some adults are accompanied
by screaming and yelling o r other bizarre behavioral
manifestations, these patients are usually referred for
psychotherapy with little consideration given to a
possible organic neurological disorder.
This report describes a small group of patients with
unusual but similar episodes of nocturnal wanderings.
None of the patients had evidence of abnormal sleep
cycles, but several had epileptiform abnormalities on
their electroencephalograms. All have responded well
to anticonvulsant drug therapy.
Patient Population
more than two years before the clinical symptoms began. A
single patient reported sleep-walking episodes between 7
and 10 years of age. None of the others had any history of
sleep disorders, and there were no family histories suggestive of sleep disturbance or epilepsy.
Recording Procedures
All patients underwent at least one all-night polygraphic
monitoring procedure and had a separate complete electroencephalographic investigation. EEG activity, the chin
muscle electromyogram, and the electrooculogram were
systematically recorded for scoring of sleep and wakefulness
[ 5 ] and the different sleep stages [6]. An infrared camera
linked to a television monitor and videotape recorder permitted continuous observation of the sleeping patient during the all-night recording. A two-way intercom attached to
a cassette tape deck recorded a n y sounds the patient made
while asleep. In this way observed behavior could b e correlated with the polygraphic tracing. The electroencephalographic investigation was carried out after 24 hours of sleep
deprivation [7] and always included a prolonged recording
with nasopharyngeal leads in place. Both waking and sleep
EEGs were obtained.
Results
Clinical Behavior
Six patients ranging in age from 17 to 32 years were studied
because of repetitive abnormal and sometimes lifethreatening behavior during sleep for which they were completely amnesic. All patients had been full-term infants with
no history of gestational, delivery, or neonatal difficulties.
Two reported head trauma during childhood not associated
with loss of consciousness and which did not require hospitalization. Three patients had experimented with street
drugs, primarily marijuana and hashish, but had stopped
Patient 1
From the Department of Neurology and the Sleep Disorders
Clinic, Stanford University Medical Center, Stanford, CA.
Address reprint requests to Dr Guillerninault, Sleep Disorders
Clinic, Stanford University Medical Center, Stanford, CA 94305.
Accepted for publication Jan 11, 1977.
30
The abnormal nocturnal wanderings were strikingly
similar. The following history is representative.
A previously healthy 18-year-old man began having
cpisodes of bizarre behavior lasting approximately 2 to 10
minutes occurring between 2 and 6 AM. H e would suddenly
jump from his bed, climb onto a chair, and begin leaping
from one piece of furniture to another. As he frequently
missed his intended destination, he suffered many bruises
and abrasions. The movements were violent and occasionally were punctuated by screaming or hitting his head against
the wall. Each episode was characteristically terminated by a
dive from a dresser onto the bed, where he would grab
semipurposefully at his bed partner and then fall deeply
asleep. During the episode he was unresponsive to questions, admonitions, or stimuli. If awakened a few minutes
later, he was amnesic for the episode. O n some occasions
one or more additional attacks followed the first, but these
were typically less violent. He might sit up, yell, and then
stare for 30 to 60 seconds before falling back asleep. At
other times he would mumble incoherently and appear
frightened. Initially the episodes occurred once every six
months. Two years later, when h e was first seen at Stanford,
they were occurring five nights out of seven.
Findings on neurological examination were within normal
limits, as were routine blood studies, skull roentgenograms,
and a computerized tomographic scan. The all-night polygraphic monitoring revealed a normal sleep pattern. A typical
episode was not recorded. Electroencephalographic evaluation demonstrated an active epileptiform focus in the right
anterior temporal region associated with focal slowing (Fig
1).The patient was started on phenytoin, and within two
weeks the episodes stopped and had not recurred more than
twelve months later.
F i g 1 . Wuking (A!aizd sleep (6)EEGs i n Putient I ,
demonstrating right anterior temporal spikes and .harp
zuai'es with associated focal slowing. A prominent pulre
urt;fact is present in the right na.topharyngeal electrode
(PG,) in B .
A
Five other patients had similar histories and a number
of features in common. In each instance the abnormal
behavior had first occurred between 15 and 20 years
of age. Each person had normal onset of sleep, and
families or sleeping partners reported that the first 2
to 4 hours of sleep were uneventful, after which the
abnormal behavior might occur at any time. Often,
when one episode was observed on a given night, a
second and occasionally a third episode ofless severity
would follow. The episodes frequently clustered, occurring several nights in succession, followed by
symptom-free intervals of variable duration. The attacks began with abrupt behavioral "arousal" with no
prodromal symptoms. Violent ambulation, often with
repetitive complex m o t o r activity, followed, associated with screaming, yelling, unintelligible
mumbling, o r comprehensible though irrelevant
phrases. These utterances were accompanied by tears
o r a fearful expression in 4 patients. Observers uniformly commented on physical effort and displays of
unusual strength if the patients were restrained. During the wanderings the patients' eyes were open but
appeared fixed. No patient appeared flushed or experienced piloerection or incontinence. In many instances the patients were able to return to bed and
would promptly fall asleep. O n other occasions they
would suddenly awaken some distance away, confused and unaware of what had transpired. Amnesia
for the episodes was complete both immediately and
subsequently.
B
Pedley and Guilleminault: Anticonvulsants for Adult Somnambulism 31
In each patient, thorough neurological examination
showed no abnormalities. Four of the 6 patients seriously injured themselves during attacks. For example, an 18-year-old man jumped through a glass door,
severing his right axillary artery and a portion of the
brachial plexus. During wakefulness the patients' behavior was in no way noteworthy. Specifically, no
patient manifested significant psychiatric disability or
had other symptoms suggesting partial complex seizures or absence attacks. In the sole woman in the
group there was no relationship between attacks and
her menstrual cycle.
An additional patient with closely related nocturnal
behavior was separated from the main group because
of his age and more complex medical history.
Patient 2
A 70-year-old man had been well until 1960, when he
suffered a myocardial infarction. Three months later he
began having recurrent nightmares and episodes of abnormal behavior during sleep, which persisted for fifteen
years. A typical attack began with a sudden change in respiratory pattern during which his breathing became deeper
and slower. He would then sit u p in bed, start mumbling,
and scream or yell incoherently. Automatic ambulation occurred frequently, and he would stumble or fall over obstacles in his path. During one episode he kicked the wall,
fracturing his great toe.
All-night polygraphic investigation revealed a normal
sleep pattern. EEG recording demonstrated continuous
polymorphic theta and delta activity over the posterior scalp
regions during wakefulness and frequent small sharp spikes
(SSS) in the right anteromesial temporal regon during
Stages 1 and 2 NREM sleep. He was treated with carbamazepine and has had n o further episodes of altered
sleep.
Elect rogruphic Da tu
I n all patients, all-night recordings documented normal sleep cycles. Two patients had abortive attacks
while being monitored. These occurred during Stage
2 NREM sleep and were not associated with any discernible abnormalities or unusual features on the
polygraphic tracing. Unfortunately, muscle and
movement artifact obscured some details of the background activity.
Four of the 6 patients in the main study group had
abnormal EEGs (Table 1). Three had focal epileptiform activity in the right anterior temporal region
(see Fig 1). O n e patient had short, generalized bursts
of 5 to 6 Hz spike-wave activity which occurred rarely
during wakefulness but became more frequent and
higher in amplitude during light sleep (Fig 2). Qualifications regarding the interpretation of this particular abnormality are discussed later. One of the patients
with a right anterior temporal focus demonstrated, in
addition, unusual high-amplitude diphasic or
32 Annals of Neurology Vol 2
No 1 July 1977
Table 1 . Ekctroencephalogru~hicFinding5 i n 6 Adult
Patients with Conzplex Somnamhdism
Patient's Sex
and Age (yr) EEG Interpretation
M, 19
My
31
M, 32
M,
M, 18
F, 27
Right anterior temporal epileptiform focus
Right anterior temporal epileptiform focus
Right anterior temporal epilepaform focus;
bifrontal sharp-slow waves during
drowsiness and light sleep
Generalized bursts of 5 to 6 Hz spike-wave
discharges
"
Normal
Normal
monophasic sharp-slow waves over the frontal and
frontopolar regions during Stage 2 NREM sleep (Fig
3). These occurred either as isolated transients o r as
short runs lasting 2 to 3 seconds. Two of the patients
demonstrated SSS in the anterior and anteromesial
temporal regions. In our opinion as well as that of
others [8,9], SSS constitute a normal finding in many
asymptomatic individuals during the initial stages of
sleep. These discharges are often best demonstrated
in the nasopharyngeal leads, and though they are
epileptiform in appearance they have no clinical significance. They should be distinguished from discharges such as are illustrated in Figure 1, which may
have pathological implications.
Treatment
Two of the patients were initially given flurazepam
without effect. Phenobarbital was prescribed for a
19-year-old man but exacerbated his nocturnal symptoms. All patients are currently being treated with
either phenytoin (2 patients) o r carbamazepine ( 5
patients). Dosage has been adjusted to maintain
therapeutic serum concentrations. The follow-up
period varies from 6 t o 48 months. To the present
time, no patient has had a recurrence of abnormal
nocturnal behavior, and all remain on medication.
Discussion
Sleep-walking is common in childhood, with up to 6%
of children aged 5 to 12 years reported as having at
least one episode [lo]. Polygraphic studies have revealed that typical somnambulism occurs during the
transition from slow-wave sleep (Stages 3 to 4 NREM)
to the first REMperiod [2, 31. Other manifestations of
NREM dyssomnias such as enuresis, night terrors,
and somniloquy often coexist [l, 111. While the
nocturnal wanderings reported here mimic childhood
somnambulism in some respects, they differ in several
important ways. The differential points are summarized in Table 2.
A
Fig 2. Generalized bnrstJ of 5 to 6 H z spike-wave activitj
(underlined). orcurring bilaterallj~,of highest amplitude
over the frontal areas. These usere o f lous d a g e with the
patient au,ake (A)but became more prominent during
drowsiness (B) and light .deep. (See text f o r qnalifications
regarding the significance o f thiJ pattern.)
B
Table 2.Diffeepentiul Chaructenstzcs o f Chzldhood Somnanzbulzsm and That
in
Patient Group o f Adolescents and Young Adults
Variable
NREM Dvssomnias
Patient Population
Behavior
Sleep-walking occurs at slow pace; patients
look sleepy; often associated with
enuresis, pavor nocturnus, o r
somniloqu y
Patients usually unarousable but docile
Sleep-walking occurs at fast pace with
associated complex, violent behavior;
patients look awake and wary; associated
screaming and incoherent speech
Completely unresponsive to environment;
patients resist any restraints
Probably stage 2, usually 4 hr or more after
sleep onset; frequent in early morning
hours
Usually 2 or more times nightly
Present
Older adolescents and young adults
Males > females
Negative
Not known with certainty-possibly
normal
2/3 epileptiforrn
Phenytoin or carbamazepine
Responsiveness
Occurrence
Frequency
Retrograde amnesia
Age
Sex
Family history
Sleep recordings
EEG
Treatment
90-120 min after sleep onset during
transition to hrst REM period; not seen
in early morning
Once nightly; rarely twice
Present
Predominantly 6- I 2 yr
Males > females
Frequently positive
Paroxysmal monorhythmic delta activity
with somnambulism
Normal
Benzodiazepines
Pedley and Guilleminault: Anticonvulsants for Adult Somnambulism
33
Though conclusive proof is lacking, several considerations make it likely that our patients have unusual
complex partial seizures. Epileptiform activity was recorded from the right anterior temporal region in 3
patients, but 2 others had normal tracings. In the sixth
patient generalized bursts of 5 to 6 Hz spike-wave
discharges were seen; this represents a controversial
pattern that some investigators believe provides "no
proof for the presence of epilepsy, either on the basis
of statistical probability or in direct relationship with
the symptoms of the individual patient" [12]. In the
absence of definitive data, however, we currently use
amplitude and field ofdistribution as major criteriafor
distinguishing between the benign wave-spike phantom (< 25 p v ) described by Marshall [I31 and other
spike-wave discharges occurring at the same frequency but perhaps having greater epileptogenic importance. W e therefore consider the discharge shown
in Figure 2 to be epileptogenic and to correlate with
clinical seizures.
Polygraphic data were not helpful in understanding
the pathophysiology of the disorder. Only two abortive attacks were recorded, and these failed to correlate with any paroxysmal o r other abnormal electrical
activity. Both, however, occurred during Stage 2
NREM sleep, and the bursts of rhythmical delta activity which signify the onset of childhood sleep-walking
were conspicuously absent.
The favorable response to anticonvulsant drugs
provides additional support for these nocturnal wanderings having an ictal origin. Finally, the atypical
34 Annals of Neurology
Vol 2
No 1 July 1977
F i g 3 , Sharp-slou ii'ai'es uppeuring dnring light deep
oi'er the frontal and frotitopolar regions. with extensiije
spread more po.rterior{y. Other portions of this record
showed epikptijorm spikes in the right anterior
and anteronresial temporal dyeaj.
behavior of our patients resembles the symptoms of
certain unusual seizure disorders. Tharp [ 141 described several patients with orbitofrontal seizures
whose ictal behavior was characterized by ambulation,
repetitive automatic motor activity, and unintelligible
vocalizations. In addition to sharing some common
clinical manifestations, 1 of our patients had frequent
bifrontal sharp-slow waves similar to the interictal
abnormality in an individual in Tharp's series (compare our Fig 3 with Tharp's Fig 8 [ 141). None of our
patients, however, had signs of autonomic dysfunction such as flushing, mydriasis, or piloerection, frequent findings in the patients with orbitofrontal seizures. Walsh [ 151 reported a 29-year-old man who had
bizarre psychomotor seizures lasting 1 to 3 minutes,
manifested by incomprehensible howling, running,
and violent behavior during which he sometimes injured himself. The seizure discharge appeared to arise
from the anterior temporal region, but interpretation
of scalp EEG data was limited by muscle and movement artifact.
W e considered it unlikely that the abnormal behavior in our patients had apsychogenic basis, because
none of them had present or past symptoms of a
typical neurotic or thought disorder and mental status
tests in all patients were normal. In addition, our
psychiatric consultants thought that repetitive sleepwalking episodes of this type would be most unusual
as the sole manifestation of apsychological aberration.
It is possible, however, that environmental and
psychological factors play some role in the frequency
and duration of the episodes. For example, no patient
had a complete sleep-walking attack under the experimental conditions in the sleep laboratory.
Recognition of this syndrome as an atypical form of
epilepsy is important, as symptoms subside with anticonvulsant drug therapy.
Addendum
Since this paper was submitted, our attention has b e e n
drawn to t h e r e p o r t by Boller e t a1 (Neurology 25:1026,
1975) of a 65-year-old m a n with “paroxysmal nightmares”
following a right temporal lobe infarction. T h e episodic
abnormal behavior and favorable response ro phenytoin
suggest a relationship to our patient group.
Supported in part by US Public Health Service Grants NS 1072704 and NS 11075 from the NINCDS and by Grant RR-70 from the
General Clinical Research Center, Division of Research Resources.
References
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episodic phenomena during sleep, in Wortis J (ed): Recent
Advances in Biology and Psychiatry. New York, Plenum Press,
1965, vol 7, pp 197-222
2. Jacobson A, Kales A, Lehmann D, et al: Somnambulism: allnight electroencephalographic studies. Science 148:975-977,
1063
3. Kales A, Jacobson A, Paulson JJ, et al: Somnambulism:
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4. Broughton RJ: Sleep disorders: disorders of arousal! Science
159:1070-1078, 1068
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1968
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9. White JC, Langston JW, Pedley TA: Benign epileptiform
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10. Kleitman N (ed): Sleep and Wakefulness. University of
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12. Thomas JE, Mass DW: Six-per-second spike-and-wave pattern
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Pedley and Guilleminauit; Anticonvulsants for A d u l t Somnambulism
35
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