(Stroke. 1998;29:1320-1321.)
© 1998 American Heart Association, Inc.
Cerebral Hypoperfusion During Acute Kawasaki Disease
Takashi Ichiyama, MD;
Miki Nishikawa, MD;
Takashi Hayashi, MD;
Mayumi Koga, MD;
Norimichi Tashiro, MD;
Susumu Furukawa, MD
From the Department of Pediatrics, Yamaguchi University School of
Medicine, Yamaguchi 755-8505, Japan.
Correspondence to Takashi Ichiyama, MD, Department of Pediatrics, Yamaguchi University School of Medicine, 1144 Kogushi, Ube, Yamaguchi 755-8505, Japan.
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Abstract
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Background and Purpose—Kawasaki
disease is a febrile disease of children notable for systemic
vasculitis. There have been many previous reports of various
complications, including disorders of the central nervous system. We
evaluated cerebral perfusion during the acute stage in patients with
Kawasaki disease.
Methods—Single-photon emission-computed tomography (SPECT) with
99mTc-hexamethylpropyleneamine
oxime was performed in 21 children with acute stage Kawasaki disease.
Follow-up SPECT and MRI were performed about 1 month after the first
SPECT in patients who exhibited abnormal SPECT findings during the
acute stage.
Results—In 6 of 21 children SPECT imaging demonstrated localized
cerebral hypoperfusion without abnormal neurological findings or
clinical symptoms, and the follow-up SPECT and MRI approximately 1
month after the first SPECT revealed no abnormalities.
Conclusions—Some patients with Kawasaki disease have transient
localized cerebral hypoperfusion at the acute stage.
Key Words: cerebral circulation • hypoperfusion • Kawasaki disease • tomography, emission computed
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Introduction
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Kawasaki disease (KD)
is an acute illness of early childhood characterized by prolonged
fever, diffuse mucosal inflammation, indurative edema of the hands and
feet, a polymorphous skin rash, and nonsuppurative
lymphadenopathy.1 KD is also called mucocutaneous
lymph node syndrome in the early literature.2 The
histopathologic findings in KD comprise pan-vasculitis with
endothelial necrosis and the infiltration of
mononuclear cells into small- and medium-sized blood
vessels.3 4 KD has many complications, including
involvement of the nervous system, with the most important being
cardiac involvement. Neurological complications, including cerebral
infarction, aseptic meningitis, facial palsy, sensorineural hearing
loss, hemiparesis, ataxia, and encephalopathy, have been previously
reported.5 6 7 8 9 10 11 We performed single-photon
emission computed tomography (SPECT) in children with KD to investigate
brain perfusion in the acute stage.
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Subjects and Methods
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Twenty-one children (10 girls and 11 boys; aged 3 months to 4
years; mean age, 1.7 years) with acute KD on admission to our hospital
between February 1996 and December 1997 were included in this study
(see the Table
). Their parents gave informed consent for
their participation in the study. The patients met the specific
diagnostic criteria for KD.1 The day
of onset of fever was considered the first day of illness. All patients
were treated with high-dose intravenous immunoglobulin (400
mg · kg1 ·
d1 for 5 days) and oral aspirin
administration.
[99mTc]hexamethylpropyleneamine
oxime (99mTc-HMPAO) SPECT scanning was performed
from 10 to 15 minutes after an intravenous injection of 110
to 300 MBq of 99mTc-HMPAO in children with KD on
days 4 to 16 (mean±SD, 8.1±3.7 days) of illness. The
99mTc-HMPAO was prepared from a freeze-dried kit
(Ceretec; Amersham International) by adding 1110 MBq of freshly eluted
99mTc perchnetate to 5 mL of a saline solution.
The injection was performed within 30 minutes after preparation. The
scanning equipment consisted of a rotating, large field-of-view gamma
camera fitted with a low-energy and high-resolution collimator.
Follow-up SPECT and MRI were performed approximately 1 month after the
first SPECT in patients who exhibited abnormal SPECT findings during
the acute stage. MRI was performed using a 1.5-T scanner with a
spin-echo T1-weighted sequence of 560/14/2 ms (repetition time/echo
time/excitations) and a T2-weighted sequence of 3000/90/1 ms.
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Results
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Six (29%) of the 21 patients exhibited localized cerebral
hypoperfusion on SPECT during the acute stage (see the
Figure). The SPECT abnormalities were not related to the
maximum serum C-reactive protein level or the duration of the fever
(Table
). All 6 patients with abnormal first SPECT findings exhibited
normal follow-up SPECT and MRI findings at about 1 month after the
first SPECT, and no definite abnormal neurological findings or clinical
symptoms were seen in the acute and convalescent stages. Patient 11,
who had no abnormalities on SPECT in the present study, exhibited
transient dilatation of the coronary arteries, and patient 21
had transient mitral regurgitation on 2-dimensional
echocardiography.
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Figure 1. [99mTc]hexamethylpropyleneamine
oxime single-photon emission-computed tomography (SPECT) in the acute
and convalescent stages. A, In patient 1, a 5-month-old boy, the first
SPECT revealed decreased regional cerebral blood flow in the left
temporal area (arrowheads) on day 8 of illness. The second SPECT (B)
revealed no abnormalities on day 50 of illness. C, In patient 2, a
3-year-old boy, the first SPECT revealed decreased regional cerebral
blood flow in the right frontal area (arrowheads) on day 9 of illness.
The second SPECT (D) revealed no abnormalities on day 37 of illness. E,
In patient 3, an 18-month-old girl, the first SPECT revealed decreased
regional cerebral blood flow in the left frontotemporal area
(arrowheads) on day 5 of illness. The second SPECT (F) revealed no
abnormalities on day 47 of illness.
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Discussion
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We suggest that the transient localized cerebral hypoperfusion
observed on SPECT in the present 5 patients might have been caused
by cerebral vasculitis. KD is characterized by systemic vasculitis, so
there may be cerebral vasculitis in KD. Cerebral infarction, 1 of the
neurological complications of KD, may be caused by cerebral arteritis.
It has been reported that SPECT reveals hypoperfusion areas in cerebral
vasculitis, including, for example, systemic lupus
erythematosus, microscopic polyangiitis, and
Sneddon's syndrome (livedo racemosa and cerebral
infarction).12 13 14 The case of a boy with KD who
developed asymptomatic cerebral infarction has been
reported,10 and it has also been reported that
patients with asymptomatic cerebral vasculitis with
systemic lupus erythematosus, microscopic
polyangiitis, and Sneddon's syndrome showed cerebral hypoperfusion on
SPECT.12 13 14
This is the first report of localized cerebral hypoperfusion observed
on SPECT in the acute stage of KD. Our study emphasizes that patients
with KD may have localized cerebral hypoperfusion during the acute
stage. The abnormalities observed on SPECT in all 5 patients were
transient, and neurological examination revealed no abnormalities in
the acute and convalescent stages. However, long-term follow-up
evaluation and the study of many patients with appropriate case
controls are necessary to conclude whether patients with transient
cerebral hypoperfusion have sequelae, including cognitive or behavioral
problems.
Received March 9, 1998;
revision received April 13, 1998;
accepted April 13, 1998.
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Abstract
Introduction
