---
title: "Childhood Stroke"
docid: "12f14b63-8dd0-4523-afe1-6fda2331e6bf"
authors:
- key: "47381de4-c9fd-4999-8dd0-1808cd72db6b"
value: "Luke L. Linscott, MD"
- key: "b2e6dabb-ee1c-42a4-a332-9f0814c1c607"
value: "Surjith Vattoth, MD, FRCR"
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name: "Childhood Stroke"
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lastUpdated: "08/06/20"
pageDescription: "Childhood Stroke"
pageKeywords: "Brain, Diagnosis, Pathology-Based Diagnoses, Stroke, Cerebral Ischemia and Infarction, Childhood Stroke"
pageTitle: "Childhood Stroke | STATdx"
enhancedTitle: "Childhood Stroke"
type: "DX"
references: true
breadcrumbs:
- "Brain"
- "Diagnosis"
- "Pathology-Based Diagnoses"
- "Stroke"
- "Cerebral Ischemia and Infarction"
- "Childhood Stroke"
---
# KEY FACTS
- ## Terminology
- Acute neurologic dysfunction due to loss of vascular integrity
- ## Imaging
- NECT: ↓ attenuation of affected gray matter
- Insular ribbon sign → loss of distinct insular cortex
- Hyperdense MCA sign → thrombosed MCA
- MR: ↓ diffusion within ~ 30 minutes of arterial occlusion
- Cytotoxic edema evident in affected territory on FLAIR/T2 by 4-6 hours after arterial occlusion
- Enhancement of infarct typically occurs after 5-7 days
- CTA/MRA: Critical for early evaluation & identification of possible etiology (e.g., dissection, arteriopathy)
- MR perfusion imaging can provide valuable information regarding region at risk in setting of acute stroke
- Arterial spin labeling (ASL) can provide useful perfusion information without contrast administration
- ## Top Differential Diagnoses
- Seizure-related injury
- Acute encephalitis
- Mitochondrial encephalopathies
- Posterior reversible encephalopathy syndrome
- ## Pathology
- Major causes: Cardiac disease (~ 25%), moyamoya-type arteriopathy, dissection, vasculitis, hematologic/metabolic
- No underlying cause discovered in ~ 25% of cases
- ## Clinical Issues
- Incidence: 2-3/100,000 per year in USA
- Mortality: 0.6/100,000
- Children typically present later than adults (> 24 hours)
- Focal deficit may be masked by lethargy, coma, irritability
- Treatment in pediatric acute stroke usually conservative
- Thrombolysis/thrombectomy not well studied in children
- Capacity for recovery in children much > adults
- ## Diagnostic Checklist
- When stroke is suspected clinically or by imaging, do not hesitate to perform vessel imaging
# TERMINOLOGY
- ## Synonyms
- Cerebrovascular accident, cerebral infarct, cerebral ischemia
- ## Definitions
- Acute alteration of neurologic function due to loss of vascular integrity
- This chapter specifically addresses arterial ischemia beyond perinatal period
# IMAGING
- ## General Features
- ### Best diagnostic clue
- Cytotoxic edema & restricted diffusion (acutely) in affected vascular territory
- ### Location
- Proximal & distal middle cerebral artery (MCA) territory most commonly affected
- ### Morphology
- Stroke caused by arterial occlusion typically conforms to 1 arterial territory
- ## CT Findings
- ### NECT
- ↓ attenuation of affected gray matter with loss of normal gray matter-white matter differentiation
- ↓ in white matter attenuation less pronounced
- Often wedge-shaped & localized to 1 arterial territory
- Diffuse ischemic injury can lead to reversal sign with gray matter diffusely ↓ in attenuation relative to white matter
- Insular ribbon sign → loss of distinct of insular cortex
- Hyperdense MCA sign → ↑ density of acutely thrombosed MCA
- Hemorrhagic transformation (HT)
- Symptomatic HT in 3%; asymptomatic HT in 30%
- Asymptomatic HT usually parenchymal
- White matter or deep nuclear hemorrhage often mass-like → hematoma within infarcted tissue
- ### CECT
- Enhancement of infarcted territory typically occurs after 5-7 days
- ### CTA
- Invaluable for demonstrating focal vascular abnormalities in acute setting
- Intimal flap in acutely dissected vessel
- Major arterial occlusion may prompt thrombolysis or mechanical thrombectomy in appropriate setting
- ## MR Findings
- **T1WI**:**** Acute: ↓ signal with gyral swelling
- Chronic: ± ↑ signal in cortical laminar necrosis
- **T1WI FS**: Allows identification of mural hematoma (↑ signal) in dissected vessel
- **T2WI**: Loss of flow void in thrombosed vessel
- **FLAIR**: ↑ signal with gyral swelling (within 4-6 hours)
- Abnormal sulcal ↑ signal (climbing ivy sign) of chronic, slow-flow collaterals in setting of longstanding proximal vascular occlusion
- **DWI**: Most sensitive for early detection of ischemia
- Acute: Restricted diffusion (↑ DWI, ↓ ADC signal) ≤ 30 minutes after ischemic insult
- Subacute (7-14 days): Pseudonormalization of signal
- ↑ DWI, ADC ~ brain parenchyma
- Chronic: Facilitated diffusion in gliotic brain
- ↑/~ DWI, ↑ ADC
- **SWI/T2* GRE**: May see ↑ size & number of cortical vessels****
- Suggests ↑ extraction fraction & possibly recoverable brain
- **T1WI C+**: Cortical & leptomeningeal enhancement seen after 5-7 days following acute infarct
- Enhancing climbing ivy sign
- **MRA**: Can detect arterial occlusion & stenosis in large- & medium-sized cerebral vessels
- Important to identify underlying dissection or arteriopathy
- **PWI**: Provides valuable information about affected brain
- Ischemic penumbra: ↓ perfusion, no DWI change (PWI-DWI mismatch)
- May define brain salvageable with acute stroke therapy
- Arterial spin labeling can provide useful perfusion information without contrast administration
- **MRS**: ↑ lactate hallmark of ischemia/infarct
- Not specific
- **Vessel wall imaging**: Vessel wall enhancement patterns improve discrimination of underlying stroke etiology
- ## Ultrasonographic Findings
- ### Grayscale ultrasound
- Affected territory hyperechoic in acute/subacute stage
- ### Color Doppler
- Direct Doppler evaluation ideal for surveillance of vascular occlusion in neonate with open sutures
- Transcranial Doppler evaluation of circle of Willis through temporal squamosa
- ↑ velocities can predict stenoses detectable by MRA
- Used as screening tool in children with sickle cell anemia
- ## Angiographic Findings
- Catheter angiography rarely necessary in acute evaluation of childhood stroke
- Justified if contemplating endovascular therapy
- Best modality for detailed evaluation of primary arteriopathies
- ## Nuclear Medicine Findings
- PET & SPECT techniques can be used to
- Identify salvageable regions at risk (ischemic penumbra)
- Demonstrate effects of synangiosis surgery in moyamoya-type vasculopathies
- ## Imaging Recommendations
- ### Best imaging tool
- CT initial imaging test for signs/symptoms of stroke; excellent for excluding hemorrhagic stroke (more common in children vs. adults)
- MR with DWI, MRA, PWI
- ### Protocol advice
- Contrast can help in assessing timing of injury & performing perfusion imaging
- Consider dedicated vessel wall imaging
# DIFFERENTIAL DIAGNOSIS
- ## Seizure-Related Injury
- Swelling & restricted diffusion secondary to persistent seizure activity
- Differentiation by clinical presentation & EEG
- [Acute Encephalitis](/document/acute-encephalitis/a45f63bb-c25b-481d-a001-9c520c58060b)
- Acute parenchymal inflammation secondary to infectious agents, typically viral
- Slower onset with encephalopathy
- [Mitochondrial Encephalopathies](/document/mitochondrial-encephalopathies/40004435-b768-4baf-a31e-651f8a174fe2)
- Symmetric basal ganglia involvement common
- Usually have manifestations beyond CNS
- [Posterior Reversible Encephalopathy Syndrome](/document/acute-hypertensive-encephalopathy--/efc6f9c2-dad9-4eb8-bad2-421bfaf1ec57)
- Patchy cortical/subcortical edema most common in parietal & occipital lobes, typically in setting of hypertension
- Diffusion restriction uncommon
- [Neonatal Herpes Encephalitis](/document/herpes-encephalitis-type-1/556f5f76-c20b-44ca-a913-c53b11c93341)
- Infant with seizures 2-5 weeks after birth
- DWI most sensitive for detection in early disease
- Often bilateral with temporal predominance, but can occur anywhere
- ## MELAS
- **M**itochondrial **e**ncephomyopathy, **l**actic acidosis, **s**troke-like episodes
- Areas of ischemia crossing arterial territories, often parietal
- MRS: ↑ lactate in normal-appearing brain
- [Group B Strep Meningitis](/document/group-b-streptococcal-meningitis/bafa10c7-e65b-4432-9959-b8e5e4af708c)
- Associated vasculitis causes ischemia in small perforating arteries
- Unilateral or bilateral deep gray nuclei ischemia
# PATHOLOGY
- ## General Features
- 6 major causes of arterial stroke in children
- Cardiac disease (~ 25%)
- Congenital heart disease, valvular heart disease, arrhythmias, & cardiomyopathies
- Moyamoya-type arteriopathy
- Sickle cell disease
- Neurofibromatosis type 1
- Radiation therapy
- Trisomy 21
- Alagille syndrome
- Arterial dissection (e.g., trauma)
- CNS vasculitis
- Hematologic/metabolic (e.g., coagulopathy)
- Idiopathic (~ 25%)
- No underlying cause discovered
# CLINICAL ISSUES
- ## Presentation
- ### Most common signs/symptoms
- Depend on patient age, etiology, & involved artery
- < 1 year: Seizures, encephalopathy > focal neurologic
- > 1 year: Usually focal neurologic (e.g., hemiplegia, early hand preference)
- Speech difficulties, gait abnormality, seizure
- Seizure → deficit often attributed to postictal state (Jacksonian paralysis)
- Embolic cause: Sudden onset of symptoms
- Stenoocclusive cause: Gradual/intermittent (e.g., TIA)
- Focal deficit may be masked by lethargy, coma, irritability
- Children typically present later than adults (> 24 hours)
- Poor recognition/understanding of symptoms by child, caregiver, physician
- Uncommon diagnosis in children requires high degree of suspicion
- ## Demographics
- ### Age
- Incidence/mortality greatest < 1 year
- Large percentage occur in perinatal period
- Perinatal arterial ischemic stroke (PAIS)
- ### Epidemiology
- Incidence: 2-3/100,000 per year in USA
- Mortality: 0.6/100,000
- Underrecognized as significant source of morbidity in pediatric population
- ## Natural History & Prognosis
- Capacity for recovery better than in adults, due to
- Better compensatory mechanisms, collateral recruitment, neuronal plasticity
- Fewer concomitant risk factors
- ## Treatment
- Clinical window of opportunity/benefit not as well understood in children as compared to adults
- Mainstay of chronic therapy for fixed vascular lesions & vasculopathies: Aspirin
- Transfusion therapy for at-risk children with sickle cell
- Mechanical thrombectomy may be considered in certain patient presentations
# DIAGNOSTIC CHECKLIST
- ## Image Interpretation Pearls
- Use same imaging signs as adults
- Have low threshold for use of CTA
994b2dbd-754f-4f23-9516-6f515e4a2678
## References
# Selected References
1. [Felling RJ et al: Predicting recovery and outcome after pediatric stroke: results from the International Pediatric Stroke Study. Ann Neurol. ePub, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32215969%5Bpmid%5D)
1. [Ibrahim AY et al: Fractional flow on TOF-MRA as a measure of stroke risk in children with intracranial arterial stenosis. AJNR Am J Neuroradiol. 41(3):535-41, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32115418%5Bpmid%5D)
1. [Morotti A et al: Pediatric ischemic stroke. J Neurol. 267(4):1221-2, 2020](http://www.ncbi.nlm.nih.gov/pubmed/?term=32157384%5Bpmid%5D)
1. [Donahue MJ et al: Neuroimaging advances in pediatric stroke. Stroke. 50(2):240-8, 2019](http://www.ncbi.nlm.nih.gov/pubmed/?term=30661496%5Bpmid%5D)
1. [Dlamini N et al: Arterial wall imaging in pediatric stroke. Stroke. 49(4):891-8, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29581340%5Bpmid%5D)
1. [Khalaf A et al: Pediatric stroke imaging. Pediatr Neurol. 86:5-18, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=30122281%5Bpmid%5D)
1. [Beslow LA: Stroke Diagnosis in the pediatric emergency department: an ongoing challenge. Stroke. 48(5):1132-3, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28336680%5Bpmid%5D)
1. [Satti S et al: Mechanical thrombectomy for pediatric acute ischemic stroke: review of the literature. J Neurointerv Surg. 9(8):732-7, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27448827%5Bpmid%5D)
1. [Wilson JL et al: Endovascular therapy in pediatric stroke: utilization, patient characteristics, and outcomes. Pediatr Neurol. 69:87-92.e2, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=28233666%5Bpmid%5D)
1. [Madaelil TP et al: Mechanical thrombectomy in pediatric acute ischemic stroke: clinical outcomes and literature review. Interv Neuroradiol. 22(4):426-31, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=26945589%5Bpmid%5D)
1. [Polan RM et al: Susceptibility-weighted imaging in pediatric arterial ischemic stroke: a valuable alternative for the noninvasive evaluation of altered cerebral hemodynamics. AJNR Am J Neuroradiol. 36(4):783-8, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25477354%5Bpmid%5D)
1. [Bernard TJ et al: Emergence of the primary pediatric stroke center: impact of the thrombolysis in pediatric stroke trial. Stroke. 45(7):2018-23, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24916908%5Bpmid%5D)
1. [Gemmete JJ et al: Arterial ischemic stroke in children. Neuroimaging Clin N Am. 23(4):781-98, 2013](http://www.ncbi.nlm.nih.gov/pubmed/?term=24156865%5Bpmid%5D)
1. [Freundlich CL et al: Pediatric stroke. Emerg Med Clin North Am. 30(3):805-28, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22974650%5Bpmid%5D)
1. [Kitchen L et al: The pediatric stroke outcome measure: a validation and reliability study. Stroke. 43(6):1602-8, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22474056%5Bpmid%5D)
1. [Beslow LA et al: Hemorrhagic transformation of childhood arterial ischemic stroke. Stroke. 42(4):941-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21350202%5Bpmid%5D)
1. [Cárdenas JF et al: Pediatric stroke. Childs Nerv Syst. 27(9):1375-90, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21336993%5Bpmid%5D)
1. [Dowling MM et al: Intracardiac shunting and stroke in children: a systematic review. J Child Neurol. 26(1):72-82, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21212453%5Bpmid%5D)
1. [Lanni G et al: Pediatric stroke: clinical findings and radiological approach. Stroke Res Treat. 2011:172168, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21603166%5Bpmid%5D)
1. [Larrue V et al: Etiologic investigation of ischemic stroke in young adults. Neurology. 76(23):1983-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21646623%5Bpmid%5D)
1. [Munot P et al: Characteristics of childhood arterial ischemic stroke with normal MR angiography. Stroke. 42(2):504-6, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21193747%5Bpmid%5D)
1. [Sedney CL et al: Cervical abnormalities causing vertebral artery dissection in children. J Neurosurg Pediatr. 7(3):272-5, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21361766%5Bpmid%5D)
1. [Shellhaas RA et al: Mimics of childhood stroke: characteristics of a prospective cohort. Pediatrics. 118(2):704-9, 2006](http://www.ncbi.nlm.nih.gov/pubmed/?term=16882826%5Bpmid%5D)
## Images
### Selected Images
. Note the sulcal effacement & loss of the gray matter-white matter differentiation.](images/app.statdx.com_image_thumbnail_4aa0e379-57fe-407d-af6f-af58fde1c979_annotated_true_size_900_quality_90_3b3be24eb74b4794faea29ad8ec6f6c94ab0bb32.jpg)
*Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right middle cerebral artery (MCA) territory . Note the sulcal effacement & loss of the gray matter-white matter differentiation.*
*Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right middle cerebral artery (MCA) territory . Note the sulcal effacement & loss of the gray matter-white matter differentiation.*
. Multiple infarcts in multiple vascular territories should raise suspicion of a proximal embolic source.](images/app.statdx.com_image_thumbnail_c47fcfac-a966-4233-9ab7-d170e8ed5763_annotated_true_size_900_quality_90_8e1912bbc856b0097561b1b2df24336a84d8d027.jpg)
*Axial DWI MR in the same patient confirms restricted diffusion in the right MCA territory . Also note the focus of restricted diffusion in the left periventricular region . Multiple infarcts in multiple vascular territories should raise suspicion of a proximal embolic source.*
*Axial DWI MR in a 6 year old with imbalance and acute infarct of the left basal ganglia shows diffusion restriction (↓ADC not shown). Acute infarct in a child should prompt further evaluation with MRA or CTA to detect an underlying vessel abnormality.*
segments of the middle cerebral artery, consistent with vasculitis.](images/app.statdx.com_image_thumbnail_60361896-c4fb-4710-9770-98c052a5cb95_annotated_true_size_900_quality_90_fa3ba3cad53a57c9668cc8932c5ad255f0d361dc.jpg)
*3D MRA of the circle of Willis in the same patient shows irregular narrowing of the left proximal and distal segments of the middle cerebral artery, consistent with vasculitis.*
*Axial TOF MRA in a 2 year old with multiple infarcts of various ages shows multiple small areas of flow-related signal in the bilateral thalami, consistent with lenticulostriate collaterals of moyamoya.*
*Axial DWI MR in the same 2-year-old girl with moyamoya-type vasculopathy shows diffusion restriction in the right frontoparietal foci of signal abnormality , suggesting an acute/subacute infarct. However, there is no diffusion restriction in the left parietal region , suggesting this infarct is of an older age.*
*Axial T1 C+ MR in a 1 year old with arteriopathy & subacute infarction shows gyriform enhancement of the cortical ribbon. Enhancement is common in the subacute phase of infarction. Precontrast T1 is necessary to distinguish true enhancement from the intrinsic ↑ T1 seen in cortical laminar necrosis.*
*Axial ADC map in the same patient shows modestly ↓ ADC within the affected cortex but resolution of acute gyral swelling, as evidenced by prominent sulci , suggesting the infarct is in the subacute phase.*
*Axial DWI MR in a 16-year-old boy involved in a motor vehicle collision (MVC) shows multiple small foci of diffusion restriction , consistent with small infarcts. Multiple infarcts should raise concern for dissection, especially when confined to a single arterial territory.*
. The right ICA is small & showed areas of irregularity on other images (not shown). The findings are consistent with bilateral ICA dissections.](1962d738-4661-4faf-96cc-b9e443de25e3)
*Axial CTA in the same patient shows vessel wall irregularity & an intimal flap in the left internal carotid artery (ICA) . The right ICA is small & showed areas of irregularity on other images (not shown). The findings are consistent with bilateral ICA dissections.*
### Additional Images
*Axial T1 C+ MR in a 2-year-old girl shows cortical enhancement in the region of a right frontoparietal infarct, suggesting that it is at least a week old.*
*Axial TOF MRA in a 2 year old with multiple infarcts of various ages shows multiple tiny foci of flow-related signal in the bilateral thalami . This appearance is consistent with lenticulostriate collaterals of moyamoya-type vasculopathy in the setting of bilateral carotid terminus occlusions.*
*Axial T2 MR in a high school football player who developed vomiting, confusion, & vertigo during a game shows gyral swelling & hyperintense signal in the medial temporal lobe , which is in the vascular territory of the left posterior cerebral artery. Intracranial MRA acquired at the same time showed a small embolus in the left posterior cerebral artery (PCA).*
*Axial CTA of the cervical arteries in the same patient shows a subtle linear filling defect consistent with an intimal flap in the left vertebral artery.*
*Axial NECT in a 2 day old with congenital heart disease & seizures shows a well-defined, wedge-shaped region of decreased attenuation corresponding to the left MCA vascular territory, consistent with an acute/subacute arterial ischemic stroke.*
*Axial T1 C+ MR in an 8 year old with a history of neurofibromatosis type 1 & known bilateral carotid terminus occlusions (resulting in a moyamoya-type vasculopathy pattern) shows abnormal sulcal enhancement (climbing ivy sign) due to arterial collaterals distal to a proximal occlusion.*
*Note the segment of the insular cortical ribbon that is no longer visible on this axial NECT in a 9 year old with acute right hemiparesis. This subtle finding may be the 1st indicator of an acute stroke.*
*Axial T1 C+ MR shows the typical climbing ivy pattern of arterial collateral enhancement in distal territories caused by proximal occlusion from a moyamoya-type vasculopathy. Note the white matter infarct on left .*
*Axial DWI MR in the same child shows an acute infarct on the right with T2 shine-through in an old left-sided stroke .*
*Coronal T2 MR shows multiple areas of infarction resulting from left hemisphere herniation. Secondary infarction from herniation can cause more morbidity than the initial insult.*
*Axial DWI MR shows a characteristic "watershed" distribution of infarction in the right cerebral hemisphere. This infarct was the result of a carotid terminus stenosis that developed from bacterial meningitis & vasculitis.*
*Axial NECT in a 14-year-old boy with acute right hemiparesis shows a hyperdense MCA sign , indicating acute thrombus in a proximal middle cerebral artery branch.*
*Coronal FLAIR MR in the same patient shows edema in the insular cortex & frontal operculum supplied by the affected MCA branch . The patient had complete recovery without direct treatment, & no etiology was found.*
*Axial FLAIR MR in a 13-year-old girl with seizures after using ephedra shows foci of increased cortical & subcortical white matter signal in the right PCA & left superior cerebellar artery distributions .*
*Sagittal oblique volume-rendered MRA in the same child shows multiple foci of arterial narrowing & dilation due to a primary arteritis of the CNS.*
in the left ICA of a child presenting with a left hemisphere infarct after mandibular surgery. The defect represents an arterial dissection.](27faf219-b885-4c52-9ed2-79a4471ff833)
*Axial CECT shows a subtle linear filling defect in the left ICA of a child presenting with a left hemisphere infarct after mandibular surgery. The defect represents an arterial dissection.*
*Axial T2 MR shows predominately cortical/subcortical swelling & abnormal signal of the left parietal lobe, typical of a subacute left MCA territory infarct. ~ 1/3 of childhood strokes will not have an underlying etiology diagnosed.*
*Axial DWI MR in a 17-year-old girl shows a geographic area of diffusion restriction in the right insular region, consistent with an infarct. Work-up revealed a hypercoagulable state (antiphospholipid antibody).*
*Axial ADC map in a 17-year-old girl shows a geographic area of diffusion restriction in the right insular region, consistent with an infarct. Work-up revealed a hypercoagulable state (antiphospholipid antibody).*
*Axial FLAIR MR in a 2-year-old girl shows multiple areas of cytotoxic edema in both cerebral hemispheres in this patient with moyamoya-type vasculopathy.*
*Axial T2WI MR shows a small, periventricular infarct in a 6 month old. MRA revealed left carotid aneurysm. Proximal arterial pathology should always be investigated at presentation.*