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Pediatric Neurology 49 (2013) 458e464

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Pediatric Neurology

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Original Article

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

Nancy Rollins MD a,*, Glen Lee Pride MDa, Patricia A. Plumb MSNb,Michael M. Dowling MD, PhD, MSCS c

aDepartment of Radiology, University of Texas Southwestern Medical Center, Dallas, TexasbChildren’s Medical Center, Dallas, TexascDepartment of Pediatrics and Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, Texas

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abstract

METHODS: Potential clinical barriers to making a time

ly diagnosis of pediatric brainstem stroke and pitfalls ofnoninvasive vascular imaging are presented. METHODS: An institutional review boardeapproved institutionaldatabase query from 2001-2012 yielded 15 patients with brainstem strokes. Medical records were reviewed forsymptoms, stroke severity using the Pediatric National Institutes of Health Stroke Scale, and outcomes using thePediatric Stroke Outcome Measure. Magnetic resonance angiography was compared with digital subtractionangiography. RESULTS: There were 10 boys and five girls; 9 months to 17 years of age (mean 7.83 years). Symptomswere headaches (eight); visual problems (eight), seizure-like activity (seven), motor deficits (six), and decreasedlevel of consciousness in four. Time since last seen well was 12 hours to 5 days. Pediatric National Institutes ofHealth 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 andinvolved >50% of the pons in six and <50% in seven; 2/15 had medullary strokes. Magnetic resonance angiographyshowed 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 confirmed the basilar arteryocclusion 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 StrokeOutcome Measures at 2-36 months is 0-5.0/10 (mean 1.25). CONCLUSIONS: Vague symptoms contributed to delaysin diagnosis. Magnetic resonance angiography was equivalent to digital subtraction angiography for basilar arteryocclusion but not for vertebral artery dissection. Evenwith basilar artery occlusion and high stroke scales, outcomewas good when systemic anticoagulation was started promptly.

Keywords: pediatric, brainstem stroke, magnetic resonance angiography (MRA), outcome, arterial dissection, digital subtractionangiography

Pediatr Neurol 2013; 49: 458-464� 2013 Elsevier Inc. All rights reserved.

Introduction

Compared with adults, pediatric ischemic strokes areuncommon, with a frequency of 1.8-3.3/100,000.1-4 Amongpediatric strokes, strokes involving the brainstem are rare,accounting for <8% of childhood strokes.1-3 There are nowidely accepted guidelines for vascular imaging in acute

ory:5 June 2013; Accepted in final form 9 July 2013nications should be addressed to: Dr. Rollins; Department ofChildren’s Medical Center; 1935 Medical District Dr; Mail-

; Dallas, TX 75235.ddress: Nancy.rollins@childrens.com

- see front matter � 2013 Elsevier Inc. All rights reserved.i.org/10.1016/j.pediatrneurol.2013.07.007

pediatric strokes althoughmagnetic resonance angiography(MRA) is widely used.4,5 The optimal medical managementfor pediatric stroke resulting from large artery disease is notclear.1,2,6-8 We reviewed a decade-long experience withpediatric brainstem strokes examining issues impacting thetimely diagnosis, practice patterns with respect to imagingand 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 strokesseen at a single tertiary referral hospital over 11 years endingMarch 2013

N. Rollins et al. / Pediatric Neurology 49 (2013) 458e464 459

identified by an institutional review boardeapproved analysis of aninstitutional database. Hemorrhagic strokes and brainstem strokesassociated with anterior circulation infarcts were excluded.

A board-certified pediatric neurologist (M.M.D.) with special exper-tise in pediatric stroke had either evaluated the patients at presentationor 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 theexamination items and scoring ranges of the NIHSS and can be retro-spectively scored from medical records with a high degree of reliabilityand 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 whichprovides objective assessment of right sensorimotor, left sensorimotor,language expression, language reception, and cognitive/behavioral inchildren taking into account expected age-related abilities. The PSOMtotal score (0-10) is the sum of these five subscale scores. A PSOM totalscore <0.5 indicates normal or insignificant deficit, 1-1.5 indicates amoderate deficit, and �2 indicates a severe deficit in at least one sub-scale.10 PSOM score of 2 within a domain is equivalent to an adultmodified Rankin score of 2 (e.g., unable to perform all previousactivities).

Imaging

Magnetic resonance imaging was done at 1.5T and included three-dimensional time-of-flight MRA through the head without contrast; 11patients also hadMRA of the neck. Digital subtraction angiography (DSA)was done 9-36 hours after MRA and included selective bilateral vertebralartery injections with frontal and lateral projections from the origin ofthe vertebral artery to the vertebral-basilar confluence.

Experienced neuroradiologists reviewed magnetic resonance imag-ing and MRA for location(s) of the strokes and arterial abnormalities andDSA and reached consensus blinded to the clinical status of the patients.MRAs at presentation were graded as being of diagnostic quality orinadequate with respect to visualization of the vertebrobasilar systemand analyzed for patency and absence of structural abnormalities of thevertebral-basilar system. Findings on MRA were compared with thoseseen by DSA.

Results

Clinical

Sixteen childrenwere identified during this interval. Onepatient was excluded: a 5 year old with clival osteomyelitisand septic cavernous sinus thrombophlebitis complicatedby a pontine infarct. The remaining 15 patients were 9months to 17 years of age (mean 7.83 years); 10 boys andfive girls (Table 1). Risk factors included recent trauma inseven; three were football related. One patient had neckcellulitis, one was on oral contraceptives, and one patienthad known congestive cardiomyopathy and was poorlycompliant with anticoagulation. The other six patients hadno known risk factors although onewas subsequently foundto have a cardiomyopathy and one a patent foramen ovale.No patient had a cervical spine fracture, connective tissuedisorder, or had undergone chiropractic manipulation; riskfactors are indicated in Table 1.

Themost common presenting symptomwas headache ineight patients with onset 12-48 hours before diagnosis.Visual problems including gaze preference, nystagmus, anddiplopia were seen in eight patients; five also had unilateralhemiparesis and patient was densely quadriparetic. Sevenpatients had intermittent rhythmic movements at presen-tation initially attributed to seizures. Depressed level ofconsciousness seen in four patients ranged from somnolent

but arousable to comatose and on ventilatory support inone. Time elapsed from last seen well to diagnosis was 12hours to 5 days.

Maximal PedNIHSS was 1-34 (mean 17.38) at presenta-tion and had worsened from 8 to 15 in one patient and 22 to34 in another. All patients were loaded with 75-80 unitsheparin per kilogram of body weight at diagnosis of brain-stem stroke and maintained on heparin until conversion tolow-molecular-weight heparin and/or low-dose aspirinwithout hemorrhagic complications. No patient receivedintravenous tissue plasminogen activator.

Imaging

Magnetic resonance imaging showed the brainstemstroke involved the pons in 13 patients and the medulla intwo. Pontine infarcts affected <50% of the brainstem in sixand�50% in seven. Themedullary strokes were<50% of thewidth of the medulla in both patients and involved theinferior cerebellar peduncle and cerebellar flocculus in one.Of the 13 pontine strokes, four were limited to the pons, sixwere associated with other posterior circulation strokes,and three involved the pons and pontomesencephalicjunction. Of the six patients with multifocal posterior cir-culation strokes, four had infarcts of different ages.

The pontine strokes were associated with basilar arteryocclusion by MRA in 8/13 patients (Table 2) and a normalbasilar artery in five. Pontine strokes associated with basilarartery occlusion were �50% of the transverse diameter ofthe pons in six patients and <50% in two. MRA of the neckwas acquired in 11 of 15 patients; there was adequatevisualization of the extra dural segments of the vertebralarteries in seven and inadequate visualization in four (Figs 1and 2). The V3 segment was most problematic by MRA, aswere hypoplastic vertebral arteries (Figs 2 and 3). MRAmissed two of four vertebral artery dissections seen by DSA.Medullary strokes were associated with a hypoplasticvertebral artery in one and thrombus within the ipsilateraldistal vertebral artery in one.

DSA was not done in five patients because of withdrawalof support in one, cardiomyopathy in two, and unequivo-cally normal MRA in two. DSA confirmed basilar artery oc-clusion seen by MRA in seven of eight patients and was notdone in one of eight. The vertebral arteries were normal byDSA in three patients with basilar artery occlusion. DSAshowed vertebral artery dissection in four patients withbasilar artery occlusion. Two patients each had posteriorcirculation emboli and congenital unilateral vertebral arteryhypoplasia by DSA. One patient, a 17-year-old boy, under-went endovascular intervention about 16 hours after thefirst onset of symptoms after rapid neurological deteriora-tion to “locked-in” state over several hours despiteadequate systemic anticoagulation. Clot extraction from thebasilar artery was done with the 0.032-inch Penumbrathromboaspiration system (Penumbra Inc., Alameda, Cali-fornia) and a total of 4 mg of intra-arterial tissue plasmin-ogen activator. There were no procedural or hemorrhagiccomplications.

Follow-up magnetic resonance imaging ranging from 2to 22 months is available in nine patients and showedencephalomalacia and gliosis corresponding to regions ofrestricted diffusion in the brainstem (Fig 3). Seven patients

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 moHemiataxia, dysarthria,visual, cognitive

3 17 yr/M HA, slurred speech,seizure-like activity

Trauma, vertebral arterydissection

none 27 2.5/10 at 26 mo; mildHP, mild cognitive deficits

4 15 yr/F Confusion, dizziness,visual field cuts, nystagmus

OCP Factor V heterozygous;ANAþ

15 2.5/10 at 4 mo; motorand 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-likeepisodes

Trauma, vertebral arterydissection

None 34 1/10 at 3 yr; motor andcognitive

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, depressedLOC

CardiomyopathyThrombus vertebralartery

MTHFR heterozygous 3 0/10 at 2 yr

9 3 yr/F HP, anisocoria, dysconjugategaze

Soft-tissue infection Arteritis 26 Estimated 4/10;preexisting globaldevelopmental delay

10 7 yr/F HA, dysconjugate gaze,nystagmus

Trauma, vertebral arterydissection

None 8 1/10 at 2 yr; dysmetria,behavior issues

11 8 yr/M HA, nausea, vomiting, HP,visual problems seizure-likemovements

Trauma Elevated lipoprotein A 31 Support withdrawn in ICU

12 14 mo/M HP, gaze preferenceNystagmus

Hypoplastic rightvertebral artery

PAI-1 mutation 4 0/10 at 18 mo

13 5 yr/F Dizzy, blurred vision;progressed to hemiparesis

Spontaneous vertebralartery dissection

Family history of strokes;no confirmatory tests

9 0.5/10 at 2 mo; behavior

14 16 yr/F Near comatose; quadriparesison ventilatory support

Unknown none 24 5/10; estimated at dischargebilateral motor, behavior

15 17 yr/M HA, diplopia, dizziness Trauma Cardiomyopathy,noncomplianton anticoagulation

1 N/A

Abbreviations:ANA ¼ Antinuclear antibodyF ¼ FemaleM ¼ MaleHA ¼ HeadacheHP ¼ HemiparesisICU ¼ Intensive care unitLOC ¼ Level of consciousnessMTHFR ¼ Methylenetetrahydrofolate reductaseOCP ¼ Oral contraceptive pillsPAI-1 ¼ plasmingen activator inhibitor-1PedNIHSS ¼ Pediatric National Institutes of Health Stroke ScalePSOM ¼ Pediatric Stroke Outcome Measure

N. Rollins et al. / Pediatric Neurology 49 (2013) 458e464460

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 ectaticirregular basilar artery in one patient. Four patients expe-rienced recurrent stroke or strokelike symptoms 2-13months after initial diagnosis, including one patient whowas noncompliant with anticoagulation.

Clinical outcome

One patient died after withdrawal of care in theintensive care unit. Of the 14 patients for whom >2months has elapsed since brainstem stroke, one has notreturned 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 of2-36 months is 0-4 (mean 1.25). One patient had cerebralpalsy and developmental delay before the brainstemstroke. The patient who had endovascular interventionhad gradual improvement in neurological status; PSOM at3 years is 2.5/10. Motor function is good with mildasymmetric hemiparesis and mild behavioral issues. Thepatient attends college with no special services, devices,or assistance. Of note is a 7-year-old boy with a PedNIHSSof 34 in whom the locked-in state resolved withoutendovascular 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 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 mo3 17 yr/M 27 �50% V4 2.5/10 at 26 mo Clot extraction4 15 yr/F 15 �50% NA 2.5/10 at 4 mo6 7 yr/M 34 �50% V3 2.5/10 at 3 yr9 3 yr/F 26 >50% V3 4/10 (estimated) Ex-premature

11 8 yr/M 31 �50% n/a NA Care withdrawn13 5 yr/F 9 <50% V3 0.5/10 at 2 mo14 16 yr/F 24 �50% NA 5/10 at discharge

Abbreviations:BAO ¼ basilar artery occlusionDSA ¼ Digital subtraction angiographyPedNIHSS ¼ Pediatric National Institutes of Health Stroke ScalePSOM ¼ Pediatric Stroke Outcome MeasureVAD ¼ Vertebral artery dissectionNA ¼ not available

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seen only with strokes that involved the pons. PSOM inthe two medullary strokes is 0.

Discussion

We describe a decade-long experience with brainstemstrokes in a tertiary pediatric hospital and identified a lackof familiarity with this clinical entity among health careproviders, which resulted in significant delays in diagnosis.Symptoms were often vague and nonspecific and mostoften included headaches and visual problems with motordeficits. Six of 13 patients with pontine strokes had rhyth-mic posturing or dystonic movements incorrectly attributedto seizures, which delayed the correct diagnosis. Suchmovements have been described in adults with brainstemstrokes, more prevalent with pontine infarction, andattributed to damage to the pontine pyramidal tracts.11

Clinical manifestations in our cohort differ somewhat

FIGURE 1.A 16-year-old girl whose basilar artery occlusion was diagnosed 5 days after sysystemic anticoagulation was begun at diagnosis. (A) Diffusion-weighted imaginance angiography shows basilar artery occlusion and suboptimal visualizatioexcluded by magnetic resonance angiography. Digital substraction angiographyshows more extensive restricted diffusion is due to Wallerian degeneration witdischarge to rehabilitation was 5/10.

from the Toronto Stroke Registry in which motor deficitswere most common.1 In the Swiss Neuropediatric StrokeRegistry, presenting signs of basilar artery occlusionwere impaired consciousness, motor deficits, brainstemdysfunction, and headaches.2 Four of the patients hadposterior circulation infarcts of different ages by magneticresonance imaging attesting to difficulties in making theclinical diagnosis.

Basilar artery occlusion was seen in eight patients withpontine strokes andwas due to vertebral artery dissection infour. Of the vertebral artery dissection, threewere associatedwith trauma; most often football-related, whereas in onepatient, the vertebral artery dissection was spontaneous.Trauma is the most common cause of extracranial vertebralartery dissection in children in whom there is often a lucidinterval followed by headaches, neck pain, dizziness, andneurological deficits.12,13 The known association of minorhead and neck traumawith brainstem strokes highlights the

mptom onset. Pediatric National Institutes of Health Stroke Scale was 24;ng at 5 days shows stroke involving >50% of the pons. (B) Magnetic reso-n of the distal vertebral artery from slow flow; a dissection could not beshowed normal vertebral artery. (C) Diffusion-weighted imaging at 10 dayshin the middle cerebellar peduncles. Pediatric Stroke Outcome Measure at

FIGURE 2.A 7-year-old boy imaged 18 hours after onset of prodromal symptoms; management was systemic anticoagulation. Pediatric National Institutes of HealthStroke Scale was 34; Pediatric Stroke Outcome Measure was 2.5 at 36 months. (A) Magnetic resonance angiography shows basilar artery occlusion and poorvisualization 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 angiographyshows 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) 458e464462

need for careful follow-up of children with neurologicalcomplaints including headaches during or after participa-tion in athletic activities. However, headaches also occurredin our patients with brainstem strokes without vertebralarterydissection.Otheretiologies for basilar arteryocclusionin children include cardioembolism, trauma, and hyperco-agulable disorders, although the etiology may remainelusive even after extensive investigation.14 Risk factors inour cohort included cervical soft-tissue cellulitis witharteritis and oral contraceptive use in one patient each. Theextensive coagulation profiles routinely acquired in our pe-diatric stroke patients showed a plasmingen activator in-hibitor-1 mutation in one patient and increased lipoproteinA in two, but were otherwise noncontributory.

Computed tomography angiography is a sensitive andaccurate technique for diagnosis of basilar artery occlusion

FIGURE 3.A 17-year-old boy in whom rapid deterioration lead to endovascular interventpontine stroke involves >50% of the brainstem. (B) Follow-up magnetic resonaStroke Outcome Measure was 2.5 at 26 months.

and vertebral artery dissections in adults,15 although thereare no reports attesting to the diagnostic accuracy ofcomputed tomography angiography in children and nopatients in our cohort had computed tomography angiog-raphy. In our cohort, MRA was equivalent to DSA in thedepiction of the basilar artery, but not for cervical vertebralartery dissection because these dissections were missed byMRA in two patients and MRA failed to define hypoplasticvertebral artery in two. In acute basilar artery occlusion, thedistal vertebral arteries were often poorly visualized mak-ing it difficult to exclude vertebral arterial pathology.Depiction of the third segment of the vertebral artery isclinically important because dissection most often involvesthis segment as the vertebral artery exits the C2 transverseforamen.14 The third segment is horizontally orientedand subject to artifactual loss of signal when using

ion 16 hours after symptom onset. (A) Diffusion-weighted imaging showsnce imaging at 12 months shows cystic changes within the pons; Pediatric

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time-of-flight MRA. Even with diagnostic quality MRA,identification of more subtle dissections on MRA wasdifficult.

We are not the first to identify problems with time-of-flight MRA in the detection of subtle craniocervical arterialdissection in children. Tan reviewed MRA acquired at 1.5Tusing time-of-flight techniques and compared findings withcatheter angiography in 13 children with craniocervicalarterial 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 andgadoliniummay improve the diagnostic accuracy of MRA, asdoes 3T. Over the decade in which these patients wereaccrued, T1 fat-suppressed images of the neck thatmayhaveidentified intramural hematomaswere not acquired norwasgadolinium given for the MRA because the opacification ofthe regional epidural plexus may mimic or obscure a verte-bral artery dissection.12 We routinely perform DSA forstrokes associated with equivocal findings by MRA as DSA isconsidered the “gold standard.”Weacknowledge that DSA isinvasive, requires radiation exposure, carries risk especiallyin smaller children, and is expensive. However, given thesubtlety of the vertebral artery dissections in our patientsand the implications of recurrent embolic disease from avertebral artery dissection, the benefits of DSA appear tooutweigh the potential risks and DSA was performedwithout complications in our cohort.

Systemic anticoagulation is not longer consideredappropriate for acute strokes in adults because of limitedefficacy and increased risk of bleeding, although car-dioembolic strokes and strokes from arterial dissection maybe exceptions.13 There are guidelines for medical manage-ment of pediatric stroke, albeit not specifically for brain-stem strokes, and the recommendations are limited withlow evidence levels and recommendations based on dataextrapolated from adults with strokes may be inappropriatefor 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 modifiedaccording to clinical data and risk factors. Anticoagulationand/or antiplatelet therapy is continued until there isdocumented healing of the dissection.

Acute brainstem stroke secondary to basilar artery oc-clusion in adults typically has mortality rates of 75-90% andsurvivors have significant functional disabilities.16,17 Thispoor prognosis has fueled more aggressive revasculariza-tion with expanded therapeutic windows for endovascularintervention with scattered reports of near miraculousoutcomes even when endovascular intervention has beendelayed as long as 9 hours. This is despite the Basilar ArteryInternational Cooperation Study registry suggesting nearuniversally dismal outcomes for adults with basilar arteryocclusion who undergo endovascular revascularization >9hours since onset of symptoms suggesting basilar arteryocclusion.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 clotextraction precludes use in small children but the deliverydevices could potentially be downsized if a clear benefit forrestoring patency of the basilar artery was established. Therisk 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 arteryocclusion 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 afterdiagnosis of basilar artery occlusion, there was a markedclinical improvement over the next 72 hours with nohemorrhagic complications.

Without intervention, children with basilar artery oc-clusion have a better prognosis than adults.1,2 The TorontoStroke Registry reported 27 pediatric brainstem strokes andused the PSOM total score as the outcome measure.1 Nopatient had tissue plasminogen activator or endovascularintervention; two of three received anticoagulation oraspirin and one of three did not. Among patients withstrokes involving the pons, midbrain, or medulla and atfollow-up from 1 month to 11 years, 8 months, 12 had agood outcome; seven were normal and five had insignifi-cant deficits. There were 12 patients with a poor outcome;10 with moderate or severe deficits and two acute deaths.Neither the presence of basilar artery occlusion, alteredlevel of consciousness, or age predicted outcome.1 Pre-dictors of poor outcome in the Toronto study were pontineinfarct size �50% and coma at presentation.1 The SwissNeuropediatric Stroke Registry analyzed seven patientswith basilar artery occlusion and 90 patients derived from68 publications. Twenty patients received systemic anti-coagulation, 16 had aspirin, and 12 underwent intra-arterialthrombolysis. Time from symptom onset to treatment was4-168 hours. Outcomes were assessed using the modifiedRankin Scale; a good outcome was defined by a modifiedRankin 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 childrenwith 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 ofbasilar artery occlusion, basilar artery occlusion recanali-zation, antithrombotic, thrombolytic and mechanicalendovascular treatment, quadriplegia, and coma, the Ped-NIHSS score was the only element significantly associatedwith outcome with a PedNIHSS <17 being an indicator ofgood outcome.2 In four of our seven surviving subjects withbasilar artery occlusion the PedNIHSS was >17, but threehad good functional outcomes, suggesting even a highPedNIHSS is not necessarily indicative of a poor prognosis.The poorest outcome was seen in the patient in whom thediagnosis of basilar artery occlusion and initiation of sys-temic anticoagulation was delayed for 5 days. For patientswith a PSOM >0, there were consistent behavioral andcognitive problems that may be pseudo-bulbar and/orrelated to the stress of stroke and subsequent rehabilitation.These functional problems were not seen with medullarystrokes.

Conclusions

As in adults, vague prodromal symptoms often precedeactual basilar artery occlusion and delay diagnosis ofbrainstem stroke. As such, ongoing education of health careproviders and the lay community about pediatric strokemay be the most effective and cost-efficient way to improve

N. Rollins et al. / Pediatric Neurology 49 (2013) 458e464464

outcomes. In our experience, early anticoagulation is asso-ciated with better functional outcomes suggesting a needfor 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 computedtomography angiography, MRA may miss subtle vertebralartery dissection. Evidence-based guidelines for vascularimaging along with medical management are needed.

M.M.D. was supported by the Doris Duke Charitable Foundation and First AmericanReal Estate Services, Inc. and M.M.D. and P.A.P. were supported by the Perot Centerfor Brain and Nerve Injury at Children’s Medical Center - Dallas.

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