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title, docid, authors, breadcrumbs, category, cmeTopicId, documentVersionId, imageCount, lastUpdated, pageDescription, pageKeywords, pageTitle, enhancedTitle, type, references, breadcrumbs
title docid authors breadcrumbs category cmeTopicId documentVersionId imageCount lastUpdated pageDescription pageKeywords pageTitle enhancedTitle type references breadcrumbs
Childhood Stroke dc608435-4c6c-4b53-985a-4630cd24d5ce
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47381de4-c9fd-4999-8dd0-1808cd72db6b Luke L. Linscott, MD
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Pediatrics pediatrics a915965c-d436-44cf-ae65-2f22e7246ea4
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Brain brain 95caa0da-bc4f-4103-8551-f58d6e415781
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Traumatic and Vascular Lesions traumatic-and-vascular-lesions 1b07bd39-2fac-4687-8460-9ea81fa3f9c9
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Childhood Stroke childhood-stroke null
Pediatrics 8bec57b6-3f2c-4787-8f1b-04c07c1848c5 3b5f228c-631c-4212-a6aa-4fdbe7fd5d76 24 11/01/21 Childhood Stroke Pediatrics, Diagnosis, Brain, Traumatic and Vascular Lesions, Childhood Stroke Childhood Stroke | STATdx Childhood Stroke DX true
Pediatrics
Diagnosis
Brain
Traumatic and Vascular Lesions
Childhood Stroke

title: "Childhood Stroke" docid: "dc608435-4c6c-4b53-985a-4630cd24d5ce" authors:

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  • name: "Childhood Stroke" slug: "childhood-stroke" treeNodeId: null category: "Pediatrics" cmeTopicId: "8bec57b6-3f2c-4787-8f1b-04c07c1848c5" documentVersionId: "3b5f228c-631c-4212-a6aa-4fdbe7fd5d76" imageCount: 24 lastUpdated: "11/01/21" pageDescription: "Childhood Stroke" pageKeywords: "Pediatrics, Diagnosis, Brain, Traumatic and Vascular Lesions, Childhood Stroke" pageTitle: "Childhood Stroke | STATdx" enhancedTitle: "Childhood Stroke" type: "DX" references: true breadcrumbs:
  • "Pediatrics"
  • "Diagnosis"
  • "Brain"
  • "Traumatic and Vascular Lesions"
  • "Childhood Stroke"

KEY FACTS

  • Terminology

    • Acute alteration of neurologic function due to loss of vascular integrity

  • Imaging

    • NECT: ↓ attenuation of affected gray matter - Insular ribbon sign → loss of distinct insular cortex - Hyperdense middle cerebral artery (MCA) sign → thrombosed MCA
    • MR: ↓ diffusion within ~ 30 minutes of arterial occlusion - Cytotoxic edema is 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 can provide useful perfusion information without contrast administration
    • MR vessel wall imaging is helpful to identify inflammatory arteriopathy

  • Top Differential Diagnoses

    • Complex migraine
    • 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 is often conservative - Thrombolysis/thrombectomy not well studied in children
    • Capacity for recovery in children much better than adults

TERMINOLOGY

  • Synonyms

    • Cerebrovascular accident, cerebral infarct, cerebral ischemia

  • Definitions

    • Acute alteration of neurologic function due to loss of vascular integrity

IMAGING

  • General Features

    • Best diagnostic clue

      - Cytotoxic edema & restricted diffusion (acutely) in affected vascular territory

    • Location

      - Proximal & distal middle cerebral artery (MCA) territories are most commonly affected

    • Morphology

      - Stroke caused by arterial occlusion typically conforms to 1 arterial territory

  • CT Findings

    • NECT

      - ↓ attenuation of affected gray matter (GM) with loss of normal GM-white matter (WM) differentiation
        - ↓ in WM attenuation is less pronounced
        - Often wedge-shaped & localized to 1 arterial territory
        - Diffuse ischemic injury can lead to reversal sign with GM diffusely ↓ in attenuation relative to WM
- Insular ribbon sign → loss of distinct insular cortex
- Hyperdense middle cerebral artery (MCA) sign → ↑ density of acutely thrombosed MCA
- Hemorrhagic transformation (HT)
        - Symptomatic HT in 3%; asymptomatic HT in 30%
                  - Asymptomatic HT is usually parenchymal
        - WM or deep nuclear hemorrhage is 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

    • T1: Acute: ↓ signal with gyral swelling - Chronic: ± ↑ signal in cortical laminar necrosis
    • T1 FS: Allows identification of mural hematoma (↑ signal) in dissected vessel
    • T2: 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
    • T1 C+: Cortical & leptomeningeal enhancement is 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 that is salvageable with acute stroke therapy - Arterial spin labeling can provide useful perfusion information without contrast administration
    • MRS: ↑ lactate is hallmark of ischemia/infarct - Not specific
    • Vessel wall imaging: Vessel wall enhancement suggests inflammatory arteriopathy - Vessel wall enhancement patterns improve discrimination of underlying stroke etiology

  • Ultrasonographic Findings

    • Grayscale ultrasound

      - Affected territory is hyperechoic in acute/subacute stage

    • Color Doppler

      - Direct Doppler evaluation is 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 is rarely necessary in acute evaluation of childhood stroke - Only 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 is 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

DIFFERENTIAL DIAGNOSIS

  • Complex Migraine

    • ↓ (early) or ↑ (late) perfusion with normal DWI
    • Engorgement of vessels on SWI

  • Seizure-Related Injury

    • Swelling & restricted diffusion secondary to persistent seizure activity
    • Differentiation by clinical presentation & EEG

  • Acute Encephalitis

    • Acute parenchymal inflammation secondary to infectious agents, typically viral
    • Slower onset with encephalopathy

  • Mitochondrial Encephalopathies

    • Symmetric basal ganglia involvement is common
    • Usually have manifestations beyond CNS

  • Posterior Reversible Encephalopathy Syndrome

    • Patchy cortical/subcortical edema is most common in parietal & occipital lobes, typically in setting of hypertension
    • Diffusion restriction is uncommon

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 I - Idiopathic - 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

      - Depends on patient age, etiology, & involved artery
        - < 1 year: Seizures, encephalopathy > focal neurologic
        - > 1 year: Usually focal neurologic (e.g., hemiplegia)
        - Speech difficulties, gait abnormality, seizure
        - Embolic cause: Sudden onset of symptoms
        - Stenoocclusive cause: Gradual/intermittent (e.g., TIA)
- Focal deficit may be masked by lethargy, coma, irritability
- Preceding transient events occur in 25%

    • Children typically present later than adults (> 24 hours) - Poor recognition/understanding of symptoms by child, caregiver, physician

  • Demographics

    • Age

      - Incidence/mortality greatest < 1 year

    • 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 is better than in adults, due to - Better compensatory mechanisms, collateral recruitment, neuronal plasticity - Fewer concomitant risk factors

  • Treatment

    • Clinical window of opportunity/benefit is 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 disease

DIAGNOSTIC CHECKLIST

  • Image Interpretation Pearls

    • Use same imaging signs as adults
    • Have low threshold for use of CTA

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References

Selected References

  1. van Es ACGM et al: Endovascular treatment for acute ischemic stroke in children: experience from the MR CLEAN Registry. Stroke. 52(3):781-8, 2021
  2. Visser MJ et al: Automated perfusion-diffusion magnetic resonance imaging in childhood arterial ischemic stroke. Stroke. 52(10):3296-304, 2021
  3. Donahue MJ et al: Neuroimaging advances in pediatric stroke. Stroke. 50(2):240-8, 2019
  4. Dlamini N et al: Arterial wall imaging in pediatric stroke. Stroke. 49(4):891-98, 2018
  5. Khalaf A et al: Pediatric stroke imaging. Pediatr Neurol. 86:5-18, 2018
  6. Beslow LA: Stroke diagnosis in the pediatric emergency department: an ongoing challenge. Stroke. 48(5):1132-3, 2017
  7. Satti S et al: Mechanical thrombectomy for pediatric acute ischemic stroke: review of the literature. J Neurointerv Surg. 9(8):732-7, 2017
  8. Wilson JL et al: Endovascular therapy in pediatric stroke: utilization, patient characteristics, and outcomes. Pediatr Neurol. 69:87-92.e2, 2017
  9. Madaelil TP et al: Mechanical thrombectomy in pediatric acute ischemic stroke: clinical outcomes and literature review. Interv Neuroradiol. 22(4):426-31, 2016
  10. 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
  11. 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
  12. Gemmete JJ et al: Arterial ischemic stroke in children. Neuroimaging Clin N Am. 23(4):781-98, 2013
  13. Kitchen L et al: The pediatric stroke outcome measure: a validation and reliability study. Stroke. 43(6):1602-8, 2012
  14. Beslow LA et al: Hemorrhagic transformation of childhood arterial ischemic stroke. Stroke. 42(4):941-6, 2011
  15. Cárdenas JF et al: Pediatric stroke. Childs Nerv Syst. 27(9):1375-90, 2011
  16. Dowling MM et al: Intracardiac shunting and stroke in children: a systematic review. J Child Neurol. 26(1):72-82, 2011
  17. Lanni G et al: Pediatric stroke: clinical findings and radiological approach. Stroke Res Treat. 2011:172168, 2011
  18. Larrue V et al: Etiologic investigation of ischemic stroke in young adults. Neurology. 76(23):1983-8, 2011
  19. Munot P et al: Characteristics of childhood arterial ischemic stroke with normal MR angiography. Stroke. 42(2):504-6, 2011
  20. Sedney CL et al: Cervical abnormalities causing vertebral artery dissection in children. J Neurosurg Pediatr. 7(3):272-5, 2011
  21. Lopez-Vicente M et al: Diagnosis and management of pediatric arterial ischemic stroke. J Stroke Cerebrovasc Dis. 19(3):175-83, 2010
  22. Shellhaas RA et al: Mimics of childhood stroke: characteristics of a prospective cohort. Pediatrics. 118(2):704-9, 2006

Images

Selected Images

Axial DWI MR in a 4-day-old term neonate presenting with seizures shows diffusion restriction throughout the left middle cerebral artery (MCA) territory, consistent with a perinatal arterial ischemic stroke (PAIS). Axial DWI MR in a 4-day-old term neonate presenting with seizures shows diffusion restriction throughout the left middle cerebral artery (MCA) territory, consistent with a perinatal arterial ischemic stroke (PAIS).

Axial DWI MR in a 4-day-old term neonate presenting with seizures shows diffusion restriction throughout the left middle cerebral artery (MCA) territory, consistent with a perinatal arterial ischemic stroke (PAIS). Axial DWI MR in a 4-day-old term neonate presenting with seizures shows diffusion restriction throughout the left middle cerebral artery (MCA) territory, consistent with a perinatal arterial ischemic stroke (PAIS).

Axial T2 MR in the same patient 2 years later shows cystic encephalomalacia throughout left MCA territory & passive enlargement of the left lateral ventricle . Patients with PAIS who do not present near birth with seizures may later present with early hand preference or extremity weakness. Axial T2 MR in the same patient 2 years later shows cystic encephalomalacia throughout left MCA territory & passive enlargement of the left lateral ventricle . Patients with PAIS who do not present near birth with seizures may later present with early hand preference or extremity weakness.

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 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 DWI MR in the same patient 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. Acute stroke should prompt careful arterial evaluation. Axial DWI MR in the same patient 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. Acute stroke should prompt careful arterial evaluation.

Additional Images

Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right 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 MCA territory . Note the sulcal effacement & loss of the gray matter-white matter differentiation.

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 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 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. 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.

Axial CTA in the same patient with multiple infarcts shows vessel wall irregularity & an intimal flap in the left internal carotid artery (ICA) , consistent with dissection. The right ICA is small & showed areas of irregularity on other images (not shown). The findings are consistent with bilateral ICA dissections. Axial CTA in the same patient with multiple infarcts shows vessel wall irregularity & an intimal flap in the left internal carotid artery (ICA) , consistent with dissection. The right ICA is small & showed areas of irregularity on other images (not shown). The findings are consistent with bilateral ICA dissections.

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 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 3D 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 3D 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 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 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-da-old with congenital heart disease & seizures shows a well-defined, wedge-shaped region of ↓ attenuation corresponding to the left MCA vascular territory, consistent with an acute/subacute arterial ischemic stroke. Axial NECT in a 2-da-old with congenital heart disease & seizures shows a well-defined, wedge-shaped region of ↓ 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 I & known bilateral carotid terminus occlusions (resulting in a moyamoya-type vasculopathy pattern) shows abnormal sulcal enhancement (the climbing ivy sign) due to arterial collaterals distal to a proximal occlusion. Axial T1 C+ MR in an 8-year-old with a history of neurofibromatosis type I & known bilateral carotid terminus occlusions (resulting in a moyamoya-type vasculopathy pattern) shows abnormal sulcal enhancement (the climbing ivy sign) due to arterial collaterals distal to a proximal occlusion.

Axial NECT shows a segment of the left 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 first indicator of an acute stroke. Axial NECT shows a segment of the left 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 first 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 the left . 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 the left .

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. 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 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. 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. 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 ↑ cortical & subcortical white matter signal in the right PCA & left superior cerebellar artery distributions . Axial FLAIR MR in a 13-year-old girl with seizures after using ephedra shows foci of ↑ 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. Sagittal oblique volume-rendered MRA in the same child shows multiple foci of arterial narrowing & dilation due to a primary arteritis of the CNS.

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 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. Approximately 1/3 of childhood strokes will not have an underlying etiology diagnosed. Axial T2 MR shows predominately cortical/subcortical swelling & abnormal signal of the left parietal lobe, typical of a subacute left MCA territory infarct. Approximately 1/3 of childhood strokes will not have an underlying etiology diagnosed.

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