416 lines
31 KiB
Markdown
416 lines
31 KiB
Markdown
---
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title: "Childhood Stroke"
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docid: "dc608435-4c6c-4b53-985a-4630cd24d5ce"
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authors:
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- key: "47381de4-c9fd-4999-8dd0-1808cd72db6b"
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value: "Luke L. Linscott, MD"
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breadcrumbs:
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-
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name: "Pediatrics"
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slug: "pediatrics"
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treeNodeId: "a915965c-d436-44cf-ae65-2f22e7246ea4"
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-
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "2b5cea64-a083-489e-ac0c-ec14ba059026"
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-
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name: "Brain"
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slug: "brain"
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treeNodeId: "95caa0da-bc4f-4103-8551-f58d6e415781"
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-
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name: "Traumatic and Vascular Lesions"
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slug: "traumatic-and-vascular-lesions"
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treeNodeId: "1b07bd39-2fac-4687-8460-9ea81fa3f9c9"
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-
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name: "Childhood Stroke"
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slug: "childhood-stroke"
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treeNodeId: null
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category: "Pediatrics"
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cmeTopicId: "8bec57b6-3f2c-4787-8f1b-04c07c1848c5"
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documentVersionId: "3b5f228c-631c-4212-a6aa-4fdbe7fd5d76"
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imageCount: 24
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lastUpdated: "11/01/21"
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pageDescription: "Childhood Stroke"
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pageKeywords: "Pediatrics, Diagnosis, Brain, Traumatic and Vascular Lesions, Childhood Stroke"
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pageTitle: "Childhood Stroke | STATdx"
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enhancedTitle: "Childhood Stroke"
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type: "DX"
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references: true
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breadcrumbs:
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- "Pediatrics"
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- "Diagnosis"
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- "Brain"
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- "Traumatic and Vascular Lesions"
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- "Childhood Stroke"
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---
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# KEY FACTS
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- ## Terminology
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- Acute alteration of neurologic function due to loss of vascular integrity
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- ## Imaging
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- NECT: ↓ attenuation of affected gray matter
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- Insular ribbon sign → loss of distinct insular cortex
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- Hyperdense middle cerebral artery (MCA) sign → thrombosed MCA
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- MR: ↓ diffusion within ~ 30 minutes of arterial occlusion
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- Cytotoxic edema is evident in affected territory on FLAIR/T2 by 4-6 hours after arterial occlusion
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- Enhancement of infarct typically occurs after 5-7 days
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- CTA/MRA: Critical for early evaluation & identification of possible etiology (e.g., dissection, arteriopathy)
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- MR perfusion imaging can provide valuable information regarding region at risk in setting of acute stroke
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- Arterial spin labeling can provide useful perfusion information without contrast administration
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- MR vessel wall imaging is helpful to identify inflammatory arteriopathy
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- ## Top Differential Diagnoses
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- Complex migraine
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- Seizure-related injury
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- Acute encephalitis
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- Mitochondrial encephalopathies
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- Posterior reversible encephalopathy syndrome
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- ## Pathology
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- Major causes: Cardiac disease (~ 25%), moyamoya-type arteriopathy, dissection, vasculitis, hematologic/metabolic
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- No underlying cause discovered in ~ 25% of cases
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- ## Clinical Issues
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- Incidence: 2-3/100,000 per year in USA
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- Mortality: 0.6/100,000
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- Children typically present later than adults (> 24 hours)
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- Focal deficit may be masked by lethargy, coma, irritability
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- Treatment in pediatric acute stroke is often conservative
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- Thrombolysis/thrombectomy not well studied in children
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- Capacity for recovery in children much better than adults
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# TERMINOLOGY
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- ## Synonyms
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- Cerebrovascular accident, cerebral infarct, cerebral ischemia
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- ## Definitions
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- Acute alteration of neurologic function due to loss of vascular integrity
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Cytotoxic edema & restricted diffusion (acutely) in affected vascular territory
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- ### Location
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- Proximal & distal middle cerebral artery (MCA) territories are most commonly affected
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- ### Morphology
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- Stroke caused by arterial occlusion typically conforms to 1 arterial territory
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- ## CT Findings
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- ### NECT
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- ↓ attenuation of affected gray matter (GM) with loss of normal GM-white matter (WM) differentiation
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- ↓ in WM attenuation is less pronounced
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- Often wedge-shaped & localized to 1 arterial territory
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- Diffuse ischemic injury can lead to reversal sign with GM diffusely ↓ in attenuation relative to WM
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- Insular ribbon sign → loss of distinct insular cortex
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- Hyperdense middle cerebral artery (MCA) sign → ↑ density of acutely thrombosed MCA
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- Hemorrhagic transformation (HT)
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- Symptomatic HT in 3%; asymptomatic HT in 30%
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- Asymptomatic HT is usually parenchymal
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- WM or deep nuclear hemorrhage is often mass-like → hematoma within infarcted tissue
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- ### CECT
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- Enhancement of infarcted territory typically occurs after 5-7 days
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- ### CTA
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- Invaluable for demonstrating focal vascular abnormalities in acute setting
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- Intimal flap in acutely dissected vessel
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- Major arterial occlusion may prompt thrombolysis or mechanical thrombectomy in appropriate setting
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- ## MR Findings
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- **T1**: Acute: ↓ signal with gyral swelling
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- Chronic: ± ↑ signal in cortical laminar necrosis
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- **T1 FS**: Allows identification of mural hematoma (↑ signal) in dissected vessel
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- **T2**: Loss of flow void in thrombosed vessel
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- **FLAIR**: ↑ signal with gyral swelling (within 4-6 hours)
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- Abnormal sulcal ↑ signal (climbing ivy sign) of chronic slow flow collaterals in setting of longstanding proximal vascular occlusion
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- **DWI**: Most sensitive for early detection of ischemia
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- Acute: Restricted diffusion (↑ DWI, ↓ ADC signal) ≤ 30 minutes after ischemic insult
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- Subacute (7-14 days): Pseudonormalization of signal
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- ↑ DWI, ADC ≈ brain parenchyma
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- Chronic: Facilitated diffusion in gliotic brain
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- ↑/≈ DWI, ↑ ADC
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- **SWI/T2* GRE**: May see ↑ size & number of cortical vessels****
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- Suggests ↑ extraction fraction & possibly recoverable brain
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- **T1 C+**: Cortical & leptomeningeal enhancement is seen after 5-7 days following acute infarct
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- Enhancing climbing ivy sign
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- **MRA**: Can detect arterial occlusion & stenosis in large- & medium-sized cerebral vessels
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- Important to identify underlying dissection or arteriopathy
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- **PWI**: Provides valuable information about affected brain
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- Ischemic penumbra: ↓ perfusion, no DWI change (PWI-DWI mismatch)
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- May define brain that is salvageable with acute stroke therapy
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- Arterial spin labeling can provide useful perfusion information without contrast administration
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- **MRS**: ↑ lactate is hallmark of ischemia/infarct
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- Not specific
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- **Vessel wall imaging**: Vessel wall enhancement suggests inflammatory arteriopathy
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- Vessel wall enhancement patterns improve discrimination of underlying stroke etiology
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- ## Ultrasonographic Findings
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- ### Grayscale ultrasound
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- Affected territory is hyperechoic in acute/subacute stage
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- ### Color Doppler
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- Direct Doppler evaluation is ideal for surveillance of vascular occlusion in neonate with open sutures
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- Transcranial Doppler evaluation of circle of Willis through temporal squamosa
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- ↑ velocities can predict stenoses detectable by MRA
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- Used as screening tool in children with sickle cell anemia
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- ## Angiographic Findings
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- Catheter angiography is rarely necessary in acute evaluation of childhood stroke
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- Only justified if contemplating endovascular therapy
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- Best modality for detailed evaluation of primary arteriopathies
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- ## Nuclear Medicine Findings
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- PET & SPECT techniques can be used to
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- Identify salvageable regions at risk (ischemic penumbra)
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- Demonstrate effects of synangiosis surgery in moyamoya-type vasculopathies
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- ## Imaging Recommendations
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- ### Best imaging tool
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- CT is initial imaging test for signs/symptoms of stroke; excellent for excluding hemorrhagic stroke (more common in children vs. adults)
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- MR with DWI, MRA, PWI
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- ### Protocol advice
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- Contrast can help in assessing timing of injury & performing perfusion imaging
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# DIFFERENTIAL DIAGNOSIS
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- ## Complex Migraine
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- ↓ (early) or ↑ (late) perfusion with normal DWI
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- Engorgement of vessels on SWI
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- ## Seizure-Related Injury
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- Swelling & restricted diffusion secondary to persistent seizure activity
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- Differentiation by clinical presentation & EEG
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- [Acute Encephalitis](/document/acute-encephalitis/a45f63bb-c25b-481d-a001-9c520c58060b)
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- Acute parenchymal inflammation secondary to infectious agents, typically viral
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- Slower onset with encephalopathy
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- [Mitochondrial Encephalopathies](/document/mitochondrial-encephalopathies/40004435-b768-4baf-a31e-651f8a174fe2)
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- Symmetric basal ganglia involvement is common
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- Usually have manifestations beyond CNS
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- [Posterior Reversible Encephalopathy Syndrome](/document/acute-hypertensive-encephalopathy--/efc6f9c2-dad9-4eb8-bad2-421bfaf1ec57)
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- Patchy cortical/subcortical edema is most common in parietal & occipital lobes, typically in setting of hypertension
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- Diffusion restriction is uncommon
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# PATHOLOGY
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- ## General Features
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- 6 major causes of arterial stroke in children
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- Cardiac disease (~ 25%)
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- Congenital heart disease, valvular heart disease, arrhythmias, & cardiomyopathies
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- Moyamoya-type arteriopathy
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- Sickle cell disease
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- Neurofibromatosis type I
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- Idiopathic
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- Arterial dissection (e.g., trauma)
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- CNS vasculitis
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- Hematologic/metabolic (e.g., coagulopathy)
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- Idiopathic (~ 25%)
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- No underlying cause discovered
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Depends on patient age, etiology, & involved artery
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- < 1 year: Seizures, encephalopathy > focal neurologic
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- > 1 year: Usually focal neurologic (e.g., hemiplegia)
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- Speech difficulties, gait abnormality, seizure
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- Embolic cause: Sudden onset of symptoms
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- Stenoocclusive cause: Gradual/intermittent (e.g., TIA)
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- Focal deficit may be masked by lethargy, coma, irritability
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- Preceding transient events occur in 25%
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- Children typically present later than adults (> 24 hours)
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- Poor recognition/understanding of symptoms by child, caregiver, physician
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- ## Demographics
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- ### Age
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- Incidence/mortality greatest < 1 year
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- ### Epidemiology
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- Incidence: 2-3/100,000 per year in USA
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- Mortality: 0.6/100,000
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- Underrecognized as significant source of morbidity in pediatric population
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- ## Natural History & Prognosis
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- Capacity for recovery is better than in adults, due to
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- Better compensatory mechanisms, collateral recruitment, neuronal plasticity
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- Fewer concomitant risk factors
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- ## Treatment
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- Clinical window of opportunity/benefit is not as well understood in children as compared to adults
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- Mainstay of chronic therapy for fixed vascular lesions & vasculopathies: Aspirin
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- Transfusion therapy for at-risk children with sickle cell disease
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# DIAGNOSTIC CHECKLIST
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- ## Image Interpretation Pearls
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- Use same imaging signs as adults
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- Have low threshold for use of CTA
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4fee7a61-bc2c-4ce0-a2d5-84ed346ac7d5
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## References
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# Selected References
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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](http://www.ncbi.nlm.nih.gov/pubmed/?term=33617341%5Bpmid%5D)
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1. [Visser MJ et al: Automated perfusion-diffusion magnetic resonance imaging in childhood arterial ischemic stroke. Stroke. 52(10):3296-304, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34404238%5Bpmid%5D)
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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)
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1. [Dlamini N et al: Arterial wall imaging in pediatric stroke. Stroke. 49(4):891-98, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29581340%5Bpmid%5D)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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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)
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1. [Lopez-Vicente M et al: Diagnosis and management of pediatric arterial ischemic stroke. J Stroke Cerebrovasc Dis. 19(3):175-83, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20434043%5Bpmid%5D)
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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)
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## Images
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### Selected Images
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*Axial DWI MR in a 4-day-old term neonate presenting with seizures shows diffusion restriction <img src='img/arrows/CS.png'/> throughout the left middle cerebral artery (MCA) territory, consistent with a perinatal arterial ischemic stroke (PAIS).*
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*Axial DWI MR in a 4-day-old term neonate presenting with seizures shows diffusion restriction <img src='img/arrows/CS.png'/> throughout the left middle cerebral artery (MCA) territory, consistent with a perinatal arterial ischemic stroke (PAIS).*
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*Axial T2 MR in the same patient 2 years later shows cystic encephalomalacia <img src='img/arrows/CS.png'/> throughout left MCA territory & passive enlargement of the left lateral ventricle <img src='img/arrows/CO.png'/>. Patients with PAIS who do not present near birth with seizures may later present with early hand preference or extremity weakness.*
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*Axial FLAIR MR in a 2-year-old girl shows multiple areas of cytotoxic edema <img src='img/arrows/CS.png'/> in both cerebral hemispheres in this patient with moyamoya-type vasculopathy.*
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*Axial DWI MR in the same patient with moyamoya-type vasculopathy shows diffusion restriction in the right frontoparietal foci of signal abnormality <img src='img/arrows/CS.png'/>, suggesting an acute/subacute infarct. However, there is no diffusion restriction in the left parietal region <img src='img/arrows/WO.png'/>, suggesting this infarct is of an older age. Acute stroke should prompt careful arterial evaluation.*
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### Additional Images
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*Axial NECT in a 15-year-old girl with dilated cardiomyopathy shows a large area of low attenuation in the right MCA territory <img src='img/arrows/CS.png'/>. Note the sulcal effacement & loss of the gray matter-white matter differentiation.*
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*Axial DWI MR in the same patient confirms restricted diffusion in the right MCA territory <img src='img/arrows/CS.png'/>. Also note the focus of restricted diffusion in the left periventricular region <img src='img/arrows/WO.png'/>. Multiple infarcts in multiple vascular territories should raise suspicion of a proximal embolic source.*
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*Axial DWI MR in a 16-year-old boy involved in a motor vehicle collision (MVC) shows multiple small foci of diffusion restriction <img src='img/arrows/CS.png'/>, consistent with small infarcts. Multiple infarcts should raise concern for dissection, especially when confined to a single arterial territory.*
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*Axial CTA in the same patient with multiple infarcts shows vessel wall irregularity & an intimal flap in the left internal carotid artery (ICA) <img src='img/arrows/WS.png'/>, consistent with dissection. The right ICA <img src='img/arrows/CS.png'/> is small & showed areas of irregularity on other images (not shown). The findings are consistent with bilateral ICA dissections.*
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*Axial T1 C+ MR in a 2-year-old girl shows cortical enhancement <img src='img/arrows/CS.png'/> in the region of a right frontoparietal infarct, suggesting that it is at least a week old.*
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*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 <img src='img/arrows/CS.png'/>. This appearance is consistent with lenticulostriate collaterals of moyamoya-type vasculopathy in the setting of bilateral carotid terminus occlusions.*
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*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 <img src='img/arrows/WS.png'/>, 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).*
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*Axial CTA of the cervical arteries in the same patient shows a subtle linear filling defect <img src='img/arrows/WS.png'/>, consistent with an intimal flap in the left vertebral artery.*
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*Axial NECT in a 2-da-old with congenital heart disease & seizures shows a well-defined, wedge-shaped region of ↓ attenuation <img src='img/arrows/WS.png'/> corresponding to the left MCA vascular territory, consistent with an acute/subacute arterial ischemic stroke.*
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*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) <img src='img/arrows/CS.png'/> due to arterial collaterals distal to a proximal occlusion.*
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*Axial NECT shows a segment <img src='img/arrows/WO.png'/> 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.*
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*Axial T1 C+ MR shows the typical climbing ivy pattern of arterial collateral enhancement <img src='img/arrows/CS.png'/> in distal territories caused by proximal occlusion from a moyamoya-type vasculopathy. Note the white matter infarct on the left <img src='img/arrows/WS.png'/>.*
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*Coronal T2 MR shows multiple areas of infarction <img src='img/arrows/WS.png'/> resulting from left hemisphere herniation. Secondary infarction from herniation can cause more morbidity than the initial insult.*
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*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.*
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*Axial NECT in a 14-year-old boy with acute right hemiparesis shows a hyperdense MCA sign <img src='img/arrows/WS.png'/>, indicating acute thrombus in a proximal middle cerebral artery branch.*
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*Coronal FLAIR MR in the same patient shows edema in the insular cortex & frontal operculum supplied by the affected MCA branch <img src='img/arrows/WS.png'/>. The patient had complete recovery without direct treatment, & no etiology was found.*
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*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 <img src='img/arrows/WS.png'/>.*
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*Sagittal oblique volume-rendered MRA in the same child shows multiple foci of arterial narrowing <img src='img/arrows/WS.png'/> & dilation <img src='img/arrows/WO.png'/> due to a primary arteritis of the CNS.*
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*Axial CECT shows a subtle linear filling defect <img src='img/arrows/WC.png'/> in the left ICA of a child presenting with a left hemisphere infarct after mandibular surgery. The defect represents an arterial dissection.*
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*Axial T2 MR shows predominately cortical/subcortical swelling & abnormal signal <img src='img/arrows/WS.png'/> 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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