European Child & Adolescent Psychiatry
8:149±153 (1999) Ó Steinkop€ Verlag 1999

H. Klasen
J. Britton
M. Newman

Accepted: 23 December 1998
H. Klasen á M. Newman (&)
Department of Child and
Adolescent Psychiatry
St. George's Hospital
GB-London SW17 0QT
J. Britton
Atkinson Morley's Hospital
Copse Hill
GB-London SW20 0NE

CASE REPORT

Moyamoya disease in a 12-year-old
Caucasian boy presenting with acute
transient psychosis

Abstract This paper reports the case
of a 12-year-old boy who presented
with an acute transient psychosis.
Investigations revealed Moyamoya
disease with occlusion of the left
middle cerebral artery. There was no
previous history of transient ischaemic attacks, no history of psychiatric disease, no family history of
psychosis and no history of illicit
drug use. Although no previous

Introduction
Moyamoya (Japanese for `cloud of smoke') is a cerebrovascular disease caused by segmental stenosis or
occlusion of one or both internal carotid arteries. This
leads to a network of small anastomotic collaterals
visible on carotid angiograms. The aetiology is uncertain
and it is assumed to be related to an acquired lesion of
the blood vessels at the base of the brain (8). The main
presentations of the disease, which has been described
predominantly in Japan, are hemiparesis, localised
muscular weaknesses, headaches, or seizures, ®rst appearing during childhood and adolescence. The nature
of the disorder is uncertain and there are probably
several causes for the characteristic radiological appearance. Complications include mental deterioration,
recurrent strokes, or intracranial haemorrhage (7).
Transient ischaemic attacks are commonly seen
and seem closely related to hypoxia after hyperventilation (5).
Psychotic symptoms as the sole manifestation of
Moyamoya disease have, to our knowledge, not been
described to date. However, McDade (6) described a 19year-old Asian man su€ering from Moyamoya who
presented with a schizophrenia-like illness. This man
had a strong family history of Moyamoya, was attend-

cases of Moyamoya disease with
psychosis as the sole presenting
feature have been described, we
suggest the possibility of a causal
link.

Key words Moyamoya disease ±
psychosis ± adolescent ± temporal
lobe pathology

ing a school for those with moderate learning disability
and had, at the ages of 6 and 7 years, su€ered episodes
of right hemiplegia caused by his illness.
In the present paper we report the clinical case of a
12-year-old boy with no family history of schizophrenia
and no previous psychiatric or neurological history who
presented with an acute psychosis. He was subsequently
diagnosed as su€ering from Moyamoya disease. The
possibilities for a causal link will be discussed.

Case
Family History
The boy lived at home with both parents and his
16-year-old brother. Both parents were middle class
professionals and were of European origin. There were
no extraordinary stresses in the family or social situation
prior to the onset of the symptoms and family relationships were described as good. The only family history
of psychiatric illness reported was that the paternal
grandfather had had a ``nervous breakdown'' at some
point. Details of that illness were not known, except
that the problems had not led to discharge from the
Army.

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Ó Steinkop€ Verlag 1999

Personal History
The boy was born after a normal pregnancy and
delivery. His neonatal period was uncomplicated and
developmental milestones were normal. He was raised
bilingual. When he had started speaking, he had
required a short period of speech therapy to help with
pronunciation. He had attended mainstream schools
throughout and his attainments were usually in the
average range, although he had been slow to learn
reading and spelling during his primary school years. He
enjoyed sports and art, did not feel overly pressured at
school and had good peer relationships. His pre-morbid
personality, as described by his mother, was ``very sweet,
very sensitive, and strong-willed.'' He had no history of
serious illnesses, allergies, or operations. In particular,
he had not experienced any episodes of dizziness, of
transient hemiplegia, or seizures. He denied any illicit
drug use, was not smoking cigarettes, and was not
receiving any medication.
Presentation
His parents reported that, nine days before his
admission to hospital, their son had taken part in a
1500-metre run at school, which had considerably
exerted him. From then on, his behaviour had become
increasingly disturbed. He had experienced diculty
sleeping and had been staying up all night, watching
television. He had become very talkative and been
worrying about philosophical matters, the brain, brain
chemicals, and his sexual orientation. His speech and
actions had not always made sense to his family. On
one occasion, the boy had defaecated in his bed, but
he had been unconcerned about this, saying that he
was experimenting. His mood had become increasingly
changeable, ranging from periods of sadness and
tearfulness to periods of elation, agitation, and
anxiety.
On mental state examination, the boy appeared
frightened and, at times, confused. He was preoccupied
by persecutory beliefs although it was dicult, at initial
presentation, to clarify these. He referred repeatedly to
his sexuality and to his physical well-being. He had
visual and auditory hallucinations, stating that he was
hearing laughter and babies crying and seeing dolls
everywhere. Later on, he said that he believed that his
mother had been replaced by another person, that
people were secretly observing and camcording him, that
he was being hypnotised, that he was being sent
messages through the television, and that he was the
subject of an experiment. He also described how his
senses, especially his sense of smell, had become more
acute. He knew his age and year of birth, but was unable
to state his full date of birth.

On physical examination, his temperature was
37.8 C, although this returned to normal within 24
hours of hospital admission. He was right-handed.
No physical or neurological abnormalities were detected
and, in particular, there were no signs of hemiplegia or
muscular weakness. Fundi were normal.
Medical investigations
Investigations, including full blood count, urea and
electrolytes, liver function tests and thyroid function
tests were normal. Blood culture and toxicology,
including tests for volatile substances, were negative.
Cerebrospinal ¯uid investigations excluded bacterial
infection, and polymerase chain reaction for Herpes
simplex and enterovirus were negative. A urine drug and
toxicology screen elicited no compounds except benzodiazepines, which had been given on admission. An
EEG examination showed no abnormality.
The neuroradiological investigations revealed the
diagnosis of Moyamoya disease. On an initial MRI scan
(Fig. 1), no abnormality was detected in the hemispheres. However, in the region of the left basal ganglia,
multiple irregular signal voids indicative of small
collateral vessels were seen, where the middle cerebral
artery should have been. A subsequently performed
MRA (Fig. 2) contrasted well the abnormal left-sided
vasculation, when compared to the normal right side.
The proximal middle cerebral artery is absent on the left.
The left internal carotid angiogram (Fig. 3) showed that
the missing vessel had been replaced by multiple small
collateral vessels, ®lling the distal middle cerebral artery
branches. These form the typical `cloud of smoke'.
Neuropsychological assessment
The following tests were performed after the psychotic
symptoms had resolved:
· Wechsler Intelligence Scale for Children (WISC-IIIUK): Verbal, Performance and Full Scale Scores were
all within the normal range, with no signi®cant
di€erences between scores;
· Wechsler Objective Reading Dimension (WORD):
Reading and comprehension were within the normal
range. Spelling was in the low average range;
· Wide Range Assessment of Memory (WRAML)
(verbal learning and story memory subtests): Most
subscales were in the normal range except for delayed
recall of a list of words which was weak, falling within
the borderline range;
· Parts IV and V of the Token Test for screening of
language function: Each score fell within the average
range;

H. Klasen et al.
Moyamoya disease in a 12-year-old Caucasian boy

151

Fig. 1 T2 weighted MRI showing
multiple irregular signal voids in
the region of the left basal ganglia
(arrow). These are indicative of
multiple collaterals in place of the
left middle cerebral artery

· Executive functioning tests: The verbal ¯uency tests
showed performance in the average range and the
Trail Making Test showed impairment in both
Part A and Part B. This suggests visual scanning
diculties.

Thus the scores generally fell within the average range.
The only impairment noted appeared due to visual
scanning diculties. Such a diculty might be expected
with frontal lobe lesions, but is not normally related to
left temporal diculties. It is possible that speech,
reading, and spelling problems encountered in early
childhood might have been part of a language problem
secondary to temporal lobe functioning abnormalities,
but the test ®ndings do not indicate any consistent
impairment in the area.
Treatment and outcome

Fig. 2 MRA showing absent left proximal middle cerebral artery
(arrow) compared to normal right-sided vasculation

The boy was initially sedated with 2 mg diazepam,
before treatment was commenced with low doses of
chlorpromazine. Due to dystonic reactions, chlorpromazine was discontinued and tri¯uoperazine 2 mg daily
commenced on the third day of admission. There was a
marked decrease of psychotic symptoms over the
following 48 hours. His condition improved further
over the following 10 days and medication was reduced
to 1 mg tri¯uoperazine daily. During this time the boy
and his family were seen regularly to discuss this
traumatic experience and to help them to cope with
associated anxieties. The boy was able to return to
school three weeks after admission. By then he showed
no psychotic symptoms and his parents reported that he
was ``100% recovered.'' Medication was discontinued
three months after admission and the boy was advised
not to exercise heavily. In view of the transient nature of
symptoms and the absence of lasting functional impairment, further neurological and neurosurgical interventions were not recommended. At one-year follow up, no
further neurological or psychiatric disturbances were
reported and the boy was functioning well, both socially
and academically.

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Ó Steinkop€ Verlag 1999

Fig. 3 Left internal carotid angiogram showing that the
missing proximal middle
cerebral artery has been replaced
by a network of small collaterals
(arrows) which ®ll the distal
middle cerebral artery branches

Discussion
Despite the fact that psychotic symptoms as the sole
manifestation of Moyamoya have not been previously
described we suggest the possibility of a causal link, on
the basis of the location of the cerebral lesion, the
physical exertion with associated hyperventilation that
preceded the psychosis, and the improbability of the two
events occurring independently.
There is a growing body of evidence in support of the
view of schizophrenia as a dysfunction of the left
temporal lobe (7). The association, ®rst proposed by
Flor-Henry (2), stemmed from the frequently observed
association of schizophrenia-like illness and temporal
lobe epilepsy. However, schizophrenia-like symptoms
have also been reported in patients with temporal lobe
dysfunctions without epilepsy. For example, Buckley et
al. (1) found that in three patients developing schizophrenia-like symptoms after cerebral trauma, the abnormalities were con®ned to or included the left temporal
lobe, while this was not the case in controls with schizoa€ective symptoms. In our patient, the site of the lesion
in the dominant left temporal lobe and the psychotic
symptoms observed, complement existing ®ndings.
The psychotic phenomena in this boy started in the
summer, shortly after he had exerted himself in a 1500metre run. A number of reasons may explain this
mechanism. It might be that, while blood supply
through the collaterals was sucient at rest or during
moderate exercise, this was not so during a period of
heavy exertion. The possibility of slight dehydration

should also be considered. In addition, several authors
have described how hyperventilation may induce a
decrease in the concentration of oxy-haemoglobin and
have found it closely related to ischaemic attacks in
children with Moyamoya disease (3, 4, 5).
Although pre-pubertal schizophrenia and bipolar
disorder are rare, by the teenage years, particularly with
males, these illnesses are seen with increasing frequency.
A drug-induced psychosis is also possible and, as the boy
only presented nine days after the beginning of the
symptoms, the negative blood and urine test cannot fully
exclude all illicit substances taken at symptom onset.
However, in this case, there was no previous psychiatric
history and no clear family history of psychosis.
Symptoms were very transient and did not reoccur at
one-year follow up, even after medication was stopped.
The patient's history and his own account, as well as
those of his parents and his school, give no indication
for any illicit drug use. Thus, whilst it is possible that
this boy had a disorder on the mood spectrum
associated with psychotic features or a disorder on the
psychotic spectrum, we suggest that, as both Moyamoya
and psychosis in pre-pubertal children are rare disorders, it would seem very unlikely that we are dealing
with two independent phenomena.
We thus conclude that, although Moyamoya disease
is a relatively rare disorder in Europe, it should perhaps
be considered as a possible cause for psychosis in
children, especially ± though, as this case shows, not
exclusively ± in those with a history of previously
unexplained transient ischaemic attacks or brief episodes
of hemiplegia.

H. Klasen et al.
Moyamoya disease in a 12-year-old Caucasian boy

153

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