Child's Nerv Syst (1994) 10:131-134
9 Springer-Verlag 1994

P. M a n c u s o
I. C h i a r a m o n t e
M. Carpinteri
S. Cicero
A. Zingale
L. Basile
R. Tropea

Received: 27 November 1991
P. Mancuso 1 (:2~) - I. Chiaramonte
M. Carpinteri 9 S. Cicero 9 A. Zingale
L. Basile 9 R. Tropea
Istituto di Neurochirurgia,
Universit/t degli studi di Catania,
Piazza S. Maria di Gesfi, 2,
1-95100 Catania, Italy
Fax: (095) 317107
1 Mailing address:

Via Necropoli Grotticelle, 16/A,
1-96100 Siracusa, Italy

Idiopathic middle cerebral artery occlusion
in children: report on three cases

Abstract Three cases o f middle
cerebral artery occlusion in children
are reported. The origin o f M1 was
the site o f occlusion in two cases,
while occlusion 1 cm after the origin o f M1 was f o u n d in the third.
The possible etiological diagnosis
was infectious arteritis in the first
two cases, cranial t r a u m a in the
third. In no case were serious contralateral m o t o r deficits detected in
follow-up examinations. F o l l o w - u p

Introduction

Case reports

Cerebrovascular occlusive disease is relatively frequent in
adults, in w h o m atherosclerosis is implicated as the m o s t
c o m m o n cause. Cerebral infarction is rare in children,
however, where the incidence according to Schoenberg
et al. [12] is 2.52 cases per 100000 per year, 55% o f which
are ischemic. A m o n g the m a n y causes o f pediatric stroke
are heart or systemic diseases [3, 10] (leukemia, sickle cell
anemia, h o m o c y s t i n u r i a , collagen diseases), arteritis o f
infectious or idiopathic origin, and t r a u m a ; atherosclerosis has been reported as a rare cause [4].
W i t h cerebral a n g i o g r a p h y and, m o r e recently, computed t o m o g r a p h y (CT) and magnetic resonance imaging
( M R I ) , the individual ischemic lesions can be accurately
diagnosed and well evaluated in terms o f their anatomical
location and extension. Radiologically, pediatric stroke
differs very little f r o m adult ischemic stroke, but the histological response o f the y o u n g brain and various etiological and physiopathological factors m a y p r o d u c e expected clinical courses in children, requiring different m a n agement options.
This report deals with three cases o f idiopathic middle
cerebral artery occlusion with a benign clinical course in
pediatric patients.

Case 1

digital subtraction a n g i o g r a p h y
showed complete regression o f arterial lesions in the second case. The
a u t h o r s conclude that middle cerebral artery occlusion m a y have a
benign clinical course in children;
surgical revascularization is indicated only in highly selected cases.
Key words Cerebral ischemia
Cerebral arteritis
Middle cerebral artery

A 12-year-old boy was referred to our institute 1 week after suffering a right hemiparesis and mixed aphasia of gradual onset, The
clinical picture had been heralded by transient ischemic attacks in
the left carotid territory. Cerebral CT and MRI performed in the
referring hospital revealed two ischemic lesions, the first in the
anterior limb of the left internal capsule, the second located in the
left posterior temporal lobe (Fig. 1). Laboratory tests, electro- and
echocardiogram and medical evaluation all had normal findings.
Cerebral angiography showed total occlusion of the origin of the
left middle cerebral artery with late filling of insular branches via
collateral circulation from the pericallosal and posterior cerebral
arteries. Severe stenosis of the supraclinoidal tract of the internal
carotid artery was also detected (Fig. 2).
The patient's neurologic status improved with physiotherapy,
and 1 month after onset he was able to walk.
An embolic etiology of the stroke was excluded due to the
normal cardiologic findings; the angiographic picture with concurrent carotid siphon stenosis suggested a diagnosis of infectious arteritis, presumably from an undiagnosed nasopharyngeal infection.
Case 2
This 9-year-old boy was admitted to our institute with the neurologic signs and symptoms of left hemiparesis and left lateral homony-

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mous hemianopia. Cerebral CT showed diffuse swelling of the right
hemisphere with a midline shift. MRI showed a large right temporoparietal infarction (Fig. 3). Cerebral angiography showed total
occlusion of the origin of the right middle cerebral artery and
marked carotid siphon stenosis (Fig. 4). Laboratory tests and cardiologic and medical evaluations all gave normal findings.
The clinical course was favorable, and 2 years after onset the
patient is able to walk and has virtually no neurologic complaints
apart from a slight left-side spasticity. A diagnosis of arteritis was
made on the basis of multiple arterial lesions. Digital subtraction
angiography 2 months later disclosed recanalization of the middle
cerebral artery; carotid stenosis was no longer visible (Fig. 5).
Case 3
This 8-year-old boy was referred to our institute from the Pediatric
Department with a past clinical history of cranial trauma. Neurologic examination disclosed no focal deficits apart from moderate
mental retardation and slight choreoathetosis. CT showed an
ischemic lesion in the head of the right caudate nucleus. MRI confirmed the diagnosis and in addition showed a hypointensive lesion
in the fight sylvian cistern (Fig. 6); a possible diagnosis of sylvian
arteriovenous malformation was made. Laboratory tests and cardiologic and medical evaluations had normal results. Cerebral angiography showed total right middle cerebral arterial occlusion
1 cm after the origin; collateral circulation was via the lenticulostriate (the possible arteriovenous malformation seen on MRI), pericallosal, and posterior cerebral arteries. Concurrent occlusion of the
terminal tract of the homolateral posterior temporal artery together
with an accessory middle cerebral artery were also shown (Fig. 7).
Possible etiological diagnosis was post-traumatic occlusion of
the right middle cerebral artery, although a diagnosis of arteritis
could not be excluded.

Discussion
Unlike in adults, atherosclerosis plays a m i n o r role in
stroke in children, m a n y other etiological or predisposing
factors such as sickle cell anemia, homocystinuria, congenital or acquired cardiac m a l f o r m a t i o n s , leukemias,
disseminated intravascular coagulation, or familial lipid
or lipoprotein abnormalities being m o r e c o m m o n
[3, 5, 10].
The patients described here are n o t amenable to
surgery, and medical treatment o f the underlying disease
represents the only therapeutic option. However, several
other cases o f pediatric stroke have been reported which
were amenable to complete neuroradiologic study and,
eventually, to surgical treatment. Shillito [14] reported 25
cases o f ischemic stroke in children m a n a g e d f r o m 1948
to 1962. Review o f angiographic studies resulted in 6
diagnoses o f internal carotid artery occlusion in the neck
and 21 o f intracranial vascular occlusive lesions. Two
patients u n d e r w e n t middle cerebral artery embolectomy.
Eighteen h a d h a d a respiratory infectious disease, high
fever, or c h i l d h o o d e x a n t h e m a prior to the onset o f neurologic deficits; no predisposing factors were f o u n d in
the remaining seven patients. Shillito concluded that

n a s o p h a r y n g e a l or paranasal sinus infections m a y result
in intracranial arterial occlusion due to either extension
o f arteritis to carotid artery branches or t h r o m b o e m bolism. Egg-Oloffson etal. [2] f o u n d a preceding
n a s o p h a r y n g e a l infection in 6 o f 11 cases o f pediatric
stroke. T h e y concluded that bacterial or viral respiratory
infections m a y lead to arteritis with consequent t h r o m boembolic occlusions.
A controversial issue is the role played in pediatric
stroke by anatomical variations o f the carotid such as
kinking or coiling. Sarkari et al. [11] reported 9 cases o f
pediatric stroke or transient ischemic attacks where cerebral a n g i o g r a p h y showed such variations on the symptomatic side. Parrish and Byrne [9] reported 9 cases o f
pediatric stroke where angiographic studies showed an
internal carotid coiling on the s y m p t o m a t i c side. Hilal
et al. [6], by contrast, reviewed 87 cases o f pediatric
stroke and f o u n d intracranial arterial occlusions in 17
cases but no cases o f c o n c u r r e n t carotid kinking or coiling.
M o y a - m o y a disease is a chronic occlusive cerebrovascular disorder that can cause severe p e r m a n e n t neurologic deficits in children. The results o f surgical treatment
are encouraging [1, 7]; surgery m u s t be performed before
p e r m a n e n t deficits have developed [8].
C a r o t i d fibromuscolar hyperplasia was recognized as
a possible cause o f pediatric stroke only in 1977 when
Shields [13] reported two cases where the characteristic
"string o f beads", which usually involves the extracranial
carotid artery, involved the intracranial carotid and middle cerebral arteries. The rarity o f the disease in children,
however, makes it impossible to determine precise indications for surgical treatment.

Fig. 1 Case 1. CT shows a hypodense lesion involving the left
internal capsule and the posterior part of the temporal lobe
Fig. 2 Case 1. Cerebral angiography shows complete occlusion at
the origin of the left middle cerebral artery; severe stenosis in the
terminal tract of the carotid siphon is also visible
Fig. 3 Case 2. T2-weighted MRI shows a left insulotemporal hyperintense lesion
Fig. 4 Case 2. Cerebral angiography shows complete occlusion of
the origin of the right middle cerebral artery; severe stenosis of the
carotid siphon is also visible
Fig. 5 Case 2. Follow-up digital subtraction angiography shows
right middle cerebral artery recanalization; the carotid stenosis is no
longer visible
Fig. 6 Case 3. MRI shows a hyperintense lesion in the head of the
right caudate nucleus
Fig. 7 Case 3. Cerebral angiography shows occlusion of the right
middle cerebral artery 1 cm after the origin; collateral circulation
via the lenticulostriate and pericallosal arteries is also visible

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Conclusions
The clinical and etiological diagnosis of pediatric stroke
is difficult due to the variety of causes and mechanisms underlying the condition. N o etiological factors
were clearly identified in the cases reported by us, since
medical records did not show concurrent metabolic
or cardiac diseases and neuroradiologic examinations
did not suggest any chronic arteritis such as moyamoya.
In cases I and 2, cerebral angiography showed total
occlusion of the origin of the middle cerebral artery associated with severe stenosis of the homolateral carotid
siphon; the multiplicity of anatomical locations and total
reversibility of the lesions, demonstrated at follow-up angiography in case 2, might be suggestive of a diagnosis of
arteritis even though no symptoms or signs of infection

were recorded in either patient. Case 3 was different since
the middle cerebral artery occlusion was detected 1 cm
after the origin and cerebral infarction was confined to
the head of the caudate nucleus; a presumptive diagnosis
of traumatic occlusion was made even though no certain
etiological factors could be determined.
While the potential benefit o f surgery has been established in patients affected by m o y a - m o y a disease [1, 7, 8],
little data is available on the role of surgical revascularization in children with idiopathic intracranial arterial
occlusions.
In our opinion, the anatomical potential of sufficient
collateral circulation in the immature brain [10] with the
possibility of a good neurologic recovery and the reversibility of arterial occlusions, as demonstrated in case
2 by serial angiography, make surgery indicated only in
highly selected cases.

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