Original Article

Brainstem Strokes in Children: An 11-Year Series From a Tertiary Pediatric

Center
Nancy Rollins MD
a,
*
, Glen Lee Pride MD
a
, Patricia A. Plumb MSN
b
,
Michael M. Dowling MD, PhD, MSCS
c
a
Department of Radiology, University of Texas Southwestern Medical Center, Dallas, Texas
b
Childrens Medical Center, Dallas, Texas
c
Department of Pediatrics and Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas
abstract
METHODS: Potential clinical barriers to making a timely diagnosis of pediatric brainstem stroke and pitfalls of
noninvasive vascular imaging are presented. METHODS: An institutional review boardeapproved institutional
database query from 2001-2012 yielded 15 patients with brainstem strokes. Medical records were reviewed for
symptoms, stroke severity using the Pediatric National Institutes of Health Stroke Scale, and outcomes using the
Pediatric Stroke Outcome Measure. Magnetic resonance angiography was compared with digital subtraction
angiography. RESULTS: There were 10 boys and ve girls; 9 months to 17 years of age (mean 7.83 years). Symptoms
were headaches (eight); visual problems (eight), seizure-like activity (seven), motor decits (six), and decreased
level of consciousness in four. Time since last seen well was 12 hours to 5 days. Pediatric National Institutes of
Health Stroke Scale was 1-34; <10 in eight; 3 in 1, 10-20 in two, and >20 in four. Strokes were pontine in 13/15 and
involved >50% of the pons in six and <50% in seven; 2/15 had medullary strokes. Magnetic resonance angiography
showed basilar artery occlusion in 8/13 patients and vertebral artery dissection in two. Digital subtraction angi-
ography done within 9-36 hours of magnetic resonance angiography in 10/15 patients conrmed the basilar artery
occlusion seen by magnetic resonance angiography and showed vertebral artery dissection in four patients. Pa-
tients were systemically anticoagulated without hemorrhagic complications. One patient died. Pediatric Stroke
Outcome Measures at 2-36 months is 0-5.0/10 (mean 1.25). CONCLUSIONS: Vague symptoms contributed to delays
in diagnosis. Magnetic resonance angiography was equivalent to digital subtraction angiography for basilar artery
occlusion but not for vertebral artery dissection. Even with basilar artery occlusion and high stroke scales, outcome
was good when systemic anticoagulation was started promptly.
Keywords: pediatric, brainstem stroke, magnetic resonance angiography (MRA), outcome, arterial dissection, digital subtraction
angiography
Pediatr Neurol 2013; 49: 458-464
2013 Elsevier Inc. All rights reserved.
Introduction
Compared with adults, pediatric ischemic strokes are
uncommon, with a frequency of 1.8-3.3/100,000.
1-4
Among
pediatric strokes, strokes involving the brainstem are rare,
accounting for <8% of childhood strokes.
1-3
There are no
widely accepted guidelines for vascular imaging in acute
pediatric strokes although magnetic resonance angiography
(MRA) is widely used.
4,5
The optimal medical management
for pediatric stroke resulting fromlarge artery disease is not
clear.
1,2,6-8
We reviewed a decade-long experience with
pediatric brainstem strokes examining issues impacting the
timely diagnosis, practice patterns with respect to imaging
and accuracy of noninvasive imaging at detecting verte-
brobasilar pathology in children, and outcomes in a popu-
lation treated with systemic anticoagulation.
Materials and Methods
This was retrospective review of patients with brainstem strokes
seen at a single tertiary referral hospital over 11 years ending March 2013
Article History:
Received 15 June 2013; Accepted in nal form 9 July 2013
* Communications should be addressed to: Dr. Rollins; Department of
Radiology; Childrens Medical Center; 1935 Medical District Dr; Mail-
stop F1.06; Dallas, TX 75235.
E-mail address: Nancy.rollins@childrens.com
Contents lists available at ScienceDirect
Pediatric Neurology
j ournal homepage: www. el sevi er. com/ l ocat e/ pnu
0887-8994/$ - see front matter 2013 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.pediatrneurol.2013.07.007
Pediatric Neurology 49 (2013) 458e464
identied by an institutional review boardeapproved analysis of an
institutional database. Hemorrhagic strokes and brainstem strokes
associated with anterior circulation infarcts were excluded.
A board-certied pediatric neurologist (M.M.D.) with special exper-
tise in pediatric stroke had either evaluated the patients at presentation
or reviewed medical records for patient demographics, clinical presen-
tation, time since last seen well, risk factors, and Pediatric National In-
stitutes of Health Stroke Scale (PedNIHSS).
9
The PedNIHSS retains the
examination items and scoring ranges of the NIHSS and can be retro-
spectively scored from medical records with a high degree of reliability
and validity.
1,9
Laboratory investigations were per our institutional pe-
diatric stroke guidelines. Neurological status at follow-up was charac-
terized using the Pediatric Stroke Outcome measures (PSOM),
10
which
provides objective assessment of right sensorimotor, left sensorimotor,
language expression, language reception, and cognitive/behavioral in
children taking into account expected age-related abilities. The PSOM
total score (0-10) is the sum of these ve subscale scores. A PSOM total
score <0.5 indicates normal or insignicant decit, 1-1.5 indicates a
moderate decit, and 2 indicates a severe decit in at least one sub-
scale.
10
PSOM score of 2 within a domain is equivalent to an adult
modied Rankin score of 2 (e.g., unable to perform all previous
activities).
Imaging
Magnetic resonance imaging was done at 1.5T and included three-
dimensional time-of-ight MRA through the head without contrast; 11
patients also had MRA of the neck. Digital subtraction angiography (DSA)
was done 9-36 hours after MRA and included selective bilateral vertebral
artery injections with frontal and lateral projections from the origin of
the vertebral artery to the vertebral-basilar conuence.
Experienced neuroradiologists reviewed magnetic resonance imag-
ing and MRA for location(s) of the strokes and arterial abnormalities and
DSA and reached consensus blinded to the clinical status of the patients.
MRAs at presentation were graded as being of diagnostic quality or
inadequate with respect to visualization of the vertebrobasilar system
and analyzed for patency and absence of structural abnormalities of the
vertebral-basilar system. Findings on MRA were compared with those
seen by DSA.
Results
Clinical
Sixteen children were identied during this interval. One
patient was excluded: a 5 year old with clival osteomyelitis
and septic cavernous sinus thrombophlebitis complicated
by a pontine infarct. The remaining 15 patients were 9
months to 17 years of age (mean 7.83 years); 10 boys and
ve girls (Table 1). Risk factors included recent trauma in
seven; three were football related. One patient had neck
cellulitis, one was on oral contraceptives, and one patient
had known congestive cardiomyopathy and was poorly
compliant with anticoagulation. The other six patients had
no known risk factors although one was subsequently found
to have a cardiomyopathy and one a patent foramen ovale.
No patient had a cervical spine fracture, connective tissue
disorder, or had undergone chiropractic manipulation; risk
factors are indicated in Table 1.
The most common presenting symptomwas headache in
eight patients with onset 12-48 hours before diagnosis.
Visual problems including gaze preference, nystagmus, and
diplopia were seen in eight patients; ve also had unilateral
hemiparesis and patient was densely quadriparetic. Seven
patients had intermittent rhythmic movements at presen-
tation initially attributed to seizures. Depressed level of
consciousness seen in four patients ranged from somnolent
but arousable to comatose and on ventilatory support in
one. Time elapsed from last seen well to diagnosis was 12
hours to 5 days.
Maximal PedNIHSS was 1-34 (mean 17.38) at presenta-
tion and had worsened from8 to 15 in one patient and 22 to
34 in another. All patients were loaded with 75-80 units
heparin per kilogram of body weight at diagnosis of brain-
stem stroke and maintained on heparin until conversion to
low-molecular-weight heparin and/or low-dose aspirin
without hemorrhagic complications. No patient received
intravenous tissue plasminogen activator.
Imaging
Magnetic resonance imaging showed the brainstem
stroke involved the pons in 13 patients and the medulla in
two. Pontine infarcts affected <50% of the brainstem in six
and 50% in seven. The medullary strokes were <50% of the
width of the medulla in both patients and involved the
inferior cerebellar peduncle and cerebellar occulus in one.
Of the 13 pontine strokes, four were limited to the pons, six
were associated with other posterior circulation strokes,
and three involved the pons and pontomesencephalic
junction. Of the six patients with multifocal posterior cir-
culation strokes, four had infarcts of different ages.
The pontine strokes were associated with basilar artery
occlusion by MRA in 8/13 patients (Table 2) and a normal
basilar artery in ve. Pontine strokes associated with basilar
artery occlusion were 50% of the transverse diameter of
the pons in six patients and <50% in two. MRA of the neck
was acquired in 11 of 15 patients; there was adequate
visualization of the extra dural segments of the vertebral
arteries in seven and inadequate visualization in four (Figs 1
and 2). The V3 segment was most problematic by MRA, as
were hypoplastic vertebral arteries (Figs 2 and 3). MRA
missed two of four vertebral artery dissections seen by DSA.
Medullary strokes were associated with a hypoplastic
vertebral artery in one and thrombus within the ipsilateral
distal vertebral artery in one.
DSA was not done in ve patients because of withdrawal
of support in one, cardiomyopathy in two, and unequivo-
cally normal MRA in two. DSA conrmed basilar artery oc-
clusion seen by MRA in seven of eight patients and was not
done in one of eight. The vertebral arteries were normal by
DSA in three patients with basilar artery occlusion. DSA
showed vertebral artery dissection in four patients with
basilar artery occlusion. Two patients each had posterior
circulation emboli and congenital unilateral vertebral artery
hypoplasia by DSA. One patient, a 17-year-old boy, under-
went endovascular intervention about 16 hours after the
rst onset of symptoms after rapid neurological deteriora-
tion to locked-in state over several hours despite
adequate systemic anticoagulation. Clot extraction from the
basilar artery was done with the 0.032-inch Penumbra
thromboaspiration system (Penumbra Inc., Alameda, Cali-
fornia) and a total of 4 mg of intra-arterial tissue plasmin-
ogen activator. There were no procedural or hemorrhagic
complications.
Follow-up magnetic resonance imaging ranging from 2
to 22 months is available in nine patients and showed
encephalomalacia and gliosis corresponding to regions of
restricted diffusion in the brainstem (Fig 3). Seven patients
N. Rollins et al. / Pediatric Neurology 49 (2013) 458e464 459
had follow-up MRA done at 3T, which showed recanaliza-
tion of previous thrombosed basilar artery in two, incom-
pletely healed vertebral artery dissection in three,
persistent basilar artery occlusion in two, and an ectatic
irregular basilar artery in one patient. Four patients expe-
rienced recurrent stroke or strokelike symptoms 2-13
months after initial diagnosis, including one patient who
was noncompliant with anticoagulation.
Clinical outcome
One patient died after withdrawal of care in the
intensive care unit. Of the 14 patients for whom >2
months has elapsed since brainstem stroke, one has not
returned for follow-up. This latter patient had a 5-day
delay in diagnosis and massive pontine stroke; esti-
mated PSOM at discharge was 5/10, which is quite poor.
PSOM for the 13 patients seen in clinical follow-up of
2-36 months is 0-4 (mean 1.25). One patient had cerebral
palsy and developmental delay before the brainstem
stroke. The patient who had endovascular intervention
had gradual improvement in neurological status; PSOM at
3 years is 2.5/10. Motor function is good with mild
asymmetric hemiparesis and mild behavioral issues. The
patient attends college with no special services, devices,
or assistance. Of note is a 7-year-old boy with a PedNIHSS
of 34 in whom the locked-in state resolved without
endovascular intervention; PSOM at 36 months is 2.5/10.
Eleven patients have emotional and/or behavioral prob-
lems not present before the brainstem stroke, which were
TABLE 1.
Clinical presentation and risk factors
Patient Age/
Gender
Clinical Presentation Etiology Risk Factors PedNIHSS PSOM
1 9 mo/M HP Unknown Elevated lipoprotein A 7 0.5/10 PSOM at 6 mo;
behavior problems
2 13 yr/M Seizure-like episodes,
decreased LOC
Unknown Protein C borderline low 23 3/10 at 7 mo
Hemiataxia, dysarthria,
visual, cognitive
3 17 yr/M HA, slurred speech,
seizure-like activity
Trauma, vertebral artery
dissection
none 27 2.5/10 at 26 mo; mild
HP, mild cognitive decits
4 15 yr/F Confusion, dizziness,
visual eld cuts, nystagmus
OCP Factor V heterozygous;
ANA
15 2.5/10 at 4 mo; motor
and cognitive
5 12 yr/M HA, vomiting, neck pain, HP Trauma None 7 0.5/10 PSOM at 2.5 yr;
right HP
6 7 yr/M HA, vertigo, ataxia, seizure-like
episodes
Trauma, vertebral artery
dissection
None 34 1/10 at 3 yr; motor and
cognitive
7 16 yr/M HA, dysarthria, HP Trauma Patent foramen ovale 5 1.5/10 at 2 mo cognitive,
behavior, Right HP
8 17 yr/M HA, gaze preference, depressed
LOC
Cardiomyopathy
Thrombus vertebral
artery
MTHFR heterozygous 3 0/10 at 2 yr
9 3 yr/F HP, anisocoria, dysconjugate
gaze
Soft-tissue infection Arteritis 26 Estimated 4/10;
preexisting global
developmental delay
10 7 yr/F HA, dysconjugate gaze,
nystagmus
Trauma, vertebral artery
dissection
None 8 1/10 at 2 yr; dysmetria,
behavior issues
11 8 yr/M HA, nausea, vomiting, HP,
visual problems seizure-like
movements
Trauma Elevated lipoprotein A 31 Support withdrawn in ICU
12 14 mo/M HP, gaze preference
Nystagmus
Hypoplastic right
vertebral artery
PAI-1 mutation 4 0/10 at 18 mo
13 5 yr/F Dizzy, blurred vision;
progressed to hemiparesis
Spontaneous vertebral
artery dissection
Family history of strokes;
no conrmatory tests
9 0.5/10 at 2 mo; behavior
14 16 yr/F Near comatose; quadriparesis
on ventilatory support
Unknown none 24 5/10; estimated at discharge
bilateral motor, behavior
15 17 yr/M HA, diplopia, dizziness Trauma Cardiomyopathy,
noncompliant
on anticoagulation
1 N/A
Abbreviations:
ANA Antinuclear antibody
F Female
M Male
HA Headache
HP Hemiparesis
ICU Intensive care unit
LOC Level of consciousness
MTHFR Methylenetetrahydrofolate reductase
OCP Oral contraceptive pills
PAI-1 plasmingen activator inhibitor-1
PedNIHSS Pediatric National Institutes of Health Stroke Scale
PSOM Pediatric Stroke Outcome Measure
N. Rollins et al. / Pediatric Neurology 49 (2013) 458e464 460
seen only with strokes that involved the pons. PSOM in
the two medullary strokes is 0.
Discussion
We describe a decade-long experience with brainstem
strokes in a tertiary pediatric hospital and identied a lack
of familiarity with this clinical entity among health care
providers, which resulted in signicant delays in diagnosis.
Symptoms were often vague and nonspecic and most
often included headaches and visual problems with motor
decits. Six of 13 patients with pontine strokes had rhyth-
mic posturing or dystonic movements incorrectly attributed
to seizures, which delayed the correct diagnosis. Such
movements have been described in adults with brainstem
strokes, more prevalent with pontine infarction, and
attributed to damage to the pontine pyramidal tracts.
11
Clinical manifestations in our cohort differ somewhat
from the Toronto Stroke Registry in which motor decits
were most common.
1
In the Swiss Neuropediatric Stroke
Registry, presenting signs of basilar artery occlusion
were impaired consciousness, motor decits, brainstem
dysfunction, and headaches.
2
Four of the patients had
posterior circulation infarcts of different ages by magnetic
resonance imaging attesting to difculties in making the
clinical diagnosis.
Basilar artery occlusion was seen in eight patients with
pontine strokes and was due tovertebral artery dissection in
four. Of the vertebral artery dissection, three wereassociated
with trauma; most often football-related, whereas in one
patient, the vertebral artery dissection was spontaneous.
Trauma is the most common cause of extracranial vertebral
artery dissection in children in whom there is often a lucid
interval followed by headaches, neck pain, dizziness, and
neurological decits.
12,13
The known association of minor
head and neck trauma with brainstemstrokes highlights the
FIGURE 1.
A 16-year-old girl whose basilar artery occlusion was diagnosed 5 days after symptom onset. Pediatric National Institutes of Health Stroke Scale was 24;
systemic anticoagulation was begun at diagnosis. (A) Diffusion-weighted imaging at 5 days shows stroke involving >50% of the pons. (B) Magnetic reso-
nance angiography shows basilar artery occlusion and suboptimal visualization of the distal vertebral artery from slow ow; a dissection could not be
excluded by magnetic resonance angiography. Digital substraction angiography showed normal vertebral artery. (C) Diffusion-weighted imaging at 10 days
shows more extensive restricted diffusion is due to Wallerian degeneration within the middle cerebellar peduncles. Pediatric Stroke Outcome Measure at
discharge to rehabilitation was 5/10.
TABLE 2.
Pontine strokes associated with BAO
Patient Age/Gender PedNIHSS Size of Pontine Infarct VAD by DSA PSOM Comments
1 9 mo/M 7 <50% NA 0.5/10 at 6 mo
3 17 yr/M 27 50% V4 2.5/10 at 26 mo Clot extraction
4 15 yr/F 15 50% NA 2.5/10 at 4 mo
6 7 yr/M 34 50% V3 2.5/10 at 3 yr
9 3 yr/F 26 >50% V3 4/10 (estimated) Ex-premature
11 8 yr/M 31 50% n/a NA Care withdrawn
13 5 yr/F 9 <50% V3 0.5/10 at 2 mo
14 16 yr/F 24 50% NA 5/10 at discharge
Abbreviations:
BAO basilar artery occlusion
DSA Digital subtraction angiography
PedNIHSS Pediatric National Institutes of Health Stroke Scale
PSOM Pediatric Stroke Outcome Measure
VAD Vertebral artery dissection
NA not available
N. Rollins et al. / Pediatric Neurology 49 (2013) 458e464 461
need for careful follow-up of children with neurological
complaints including headaches during or after participa-
tion in athletic activities. However, headaches also occurred
in our patients with brainstem strokes without vertebral
arterydissection. Other etiologies for basilar arteryocclusion
in children include cardioembolism, trauma, and hyperco-
agulable disorders, although the etiology may remain
elusive even after extensive investigation.
14
Risk factors in
our cohort included cervical soft-tissue cellulitis with
arteritis and oral contraceptive use in one patient each. The
extensive coagulation proles routinely acquired in our pe-
diatric stroke patients showed a plasmingen activator in-
hibitor-1 mutation in one patient and increased lipoprotein
A in two, but were otherwise noncontributory.
Computed tomography angiography is a sensitive and
accurate technique for diagnosis of basilar artery occlusion
and vertebral artery dissections in adults,
15
although there
are no reports attesting to the diagnostic accuracy of
computed tomography angiography in children and no
patients in our cohort had computed tomography angiog-
raphy. In our cohort, MRA was equivalent to DSA in the
depiction of the basilar artery, but not for cervical vertebral
artery dissection because these dissections were missed by
MRA in two patients and MRA failed to dene hypoplastic
vertebral artery in two. In acute basilar artery occlusion, the
distal vertebral arteries were often poorly visualized mak-
ing it difcult to exclude vertebral arterial pathology.
Depiction of the third segment of the vertebral artery is
clinically important because dissection most often involves
this segment as the vertebral artery exits the C2 transverse
foramen.
14
The third segment is horizontally oriented
and subject to artifactual loss of signal when using
FIGURE 3.
A 17-year-old boy in whom rapid deterioration lead to endovascular intervention 16 hours after symptom onset. (A) Diffusion-weighted imaging shows
pontine stroke involves >50% of the brainstem. (B) Follow-up magnetic resonance imaging at 12 months shows cystic changes within the pons; Pediatric
Stroke Outcome Measure was 2.5 at 26 months.
FIGURE 2.
A 7-year-old boy imaged 18 hours after onset of prodromal symptoms; management was systemic anticoagulation. Pediatric National Institutes of Health
Stroke Scale was 34; Pediatric Stroke Outcome Measure was 2.5 at 36 months. (A) Magnetic resonance angiography shows basilar artery occlusion and poor
visualization of right vertebral artery. (B) Source image from magnetic resonance angiography shows irregularity (arrow) of the left vertebral artery. (C)
Oblique view of the magnetic resonance angiography scan shows the subtle left vertebral artery dissection (arrow). (D) Digital substraction angiography
shows a hypoplastic but otherwise normal right vertebral artery and a dissection of the left V3 segment (arrow).
N. Rollins et al. / Pediatric Neurology 49 (2013) 458e464 462
time-of-ight MRA. Even with diagnostic quality MRA,
identication of more subtle dissections on MRA was
difcult.
We are not the rst to identify problems with time-of-
ight MRA in the detection of subtle craniocervical arterial
dissection in children. Tan reviewed MRA acquired at 1.5T
using time-of-ight techniques and compared ndings with
catheter angiography in 13 children with craniocervical
arterial dissection; 8/13 involved the vertebral arteries.
4
MRA was equivalent to catheter angiography in 1/8 pa-
tients and missed or underestimated extent of arterial pa-
thology in 7/8 dissections.
4
Fat-suppressed T1 images and
gadoliniummay improve the diagnostic accuracy of MRA, as
does 3T. Over the decade in which these patients were
accrued, T1 fat-suppressed images of the neck that may have
identiedintramural hematomas were not acquired nor was
gadolinium given for the MRA because the opacication of
the regional epidural plexus may mimic or obscure a verte-
bral artery dissection.
12
We routinely perform DSA for
strokes associated with equivocal ndings by MRA as DSA is
considered the goldstandard. We acknowledge that DSAis
invasive, requires radiation exposure, carries risk especially
in smaller children, and is expensive. However, given the
subtlety of the vertebral artery dissections in our patients
and the implications of recurrent embolic disease from a
vertebral artery dissection, the benets of DSA appear to
outweigh the potential risks and DSA was performed
without complications in our cohort.
Systemic anticoagulation is not longer considered
appropriate for acute strokes in adults because of limited
efcacy and increased risk of bleeding, although car-
dioembolic strokes and strokes fromarterial dissection may
be exceptions.
13
There are guidelines for medical manage-
ment of pediatric stroke, albeit not specically for brain-
stem strokes, and the recommendations are limited with
low evidence levels and recommendations based on data
extrapolated fromadults with strokes may be inappropriate
for children.
7,8
At our institution and as with anterior cir-
culation infarcts, in the absence of contraindications, sys-
temic anticoagulation is begun immediately and modied
according to clinical data and risk factors. Anticoagulation
and/or antiplatelet therapy is continued until there is
documented healing of the dissection.
Acute brainstem stroke secondary to basilar artery oc-
clusion in adults typically has mortality rates of 75-90% and
survivors have signicant functional disabilities.
16,17
This
poor prognosis has fueled more aggressive revasculariza-
tion with expanded therapeutic windows for endovascular
intervention with scattered reports of near miraculous
outcomes even when endovascular intervention has been
delayed as long as 9 hours. This is despite the Basilar Artery
International Cooperation Study registry suggesting near
universally dismal outcomes for adults with basilar artery
occlusion who undergo endovascular revascularization >9
hours since onset of symptoms suggesting basilar artery
occlusion.
18
Whether delayed recanalization of basilar ar-
tery occlusion is indicated in children has not been studied.
The relatively large size of the devices used for clot
extraction precludes use in small children but the delivery
devices could potentially be downsized if a clear benet for
restoring patency of the basilar artery was established. The
risk of hemorrhagic complications from intra-arterial
intervention in pediatric basilar artery occlusion is un-
known, although there are scattered reports of uncompli-
cated endovascular recanalization of basilar artery
occlusion in children.
19e21
In our single patient who un-
derwent mechanical clot extraction of basilar artery occlu-
sion 16 hours after symptom onset and 9 hours after
diagnosis of basilar artery occlusion, there was a marked
clinical improvement over the next 72 hours with no
hemorrhagic complications.
Without intervention, children with basilar artery oc-
clusion have a better prognosis than adults.
1,2
The Toronto
Stroke Registry reported 27 pediatric brainstem strokes and
used the PSOM total score as the outcome measure.
1
No
patient had tissue plasminogen activator or endovascular
intervention; two of three received anticoagulation or
aspirin and one of three did not. Among patients with
strokes involving the pons, midbrain, or medulla and at
follow-up from 1 month to 11 years, 8 months, 12 had a
good outcome; seven were normal and ve had insigni-
cant decits. There were 12 patients with a poor outcome;
10 with moderate or severe decits and two acute deaths.
Neither the presence of basilar artery occlusion, altered
level of consciousness, or age predicted outcome.
1
Pre-
dictors of poor outcome in the Toronto study were pontine
infarct size 50% and coma at presentation.
1
The Swiss
Neuropediatric Stroke Registry analyzed seven patients
with basilar artery occlusion and 90 patients derived from
68 publications. Twenty patients received systemic anti-
coagulation, 16 had aspirin, and 12 underwent intra-arterial
thrombolysis. Time from symptom onset to treatment was
4-168 hours. Outcomes were assessed using the modied
Rankin Scale; a good outcome was dened by a modied
Rankin score of 0-2 and a bad outcome was 3-6.
2
The sur-
vival rate was 92%, with a good outcome in 50% of children
with basilar artery occlusion, compared with the 45-80%
survival rate and 20-30% good outcome reported in adults.
18
In a multivariate analysis of PedNIHSS score, length of
basilar artery occlusion, basilar artery occlusion recanali-
zation, antithrombotic, thrombolytic and mechanical
endovascular treatment, quadriplegia, and coma, the Ped-
NIHSS score was the only element signicantly associated
with outcome with a PedNIHSS <17 being an indicator of
good outcome.
2
In four of our seven surviving subjects with
basilar artery occlusion the PedNIHSS was >17, but three
had good functional outcomes, suggesting even a high
PedNIHSS is not necessarily indicative of a poor prognosis.
The poorest outcome was seen in the patient in whom the
diagnosis of basilar artery occlusion and initiation of sys-
temic anticoagulation was delayed for 5 days. For patients
with a PSOM >0, there were consistent behavioral and
cognitive problems that may be pseudo-bulbar and/or
related to the stress of stroke and subsequent rehabilitation.
These functional problems were not seen with medullary
strokes.
Conclusions
As in adults, vague prodromal symptoms often precede
actual basilar artery occlusion and delay diagnosis of
brainstem stroke. As such, ongoing education of health care
providers and the lay community about pediatric stroke
may be the most effective and cost-efcient way to improve
N. Rollins et al. / Pediatric Neurology 49 (2013) 458e464 463
outcomes. In our experience, early anticoagulation is asso-
ciated with better functional outcomes suggesting a need
for more in-depth study of the use of systemic anti-
coagulation in this clinical setting. Although vascular im-
aging is usually done with MRA rather than computed
tomography angiography, MRA may miss subtle vertebral
artery dissection. Evidence-based guidelines for vascular
imaging along with medical management are needed.
M.M.D. was supported by the Doris Duke Charitable Foundation and First American
Real Estate Services, Inc. and M.M.D. and P.A.P. were supported by the Perot Center
for Brain and Nerve Injury at Childrens Medical Center - Dallas.
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