What about Us!: Strokes in Infants, What about Us!: Strokes in Infants, Children and Young AdultsChildren and Young Adults

Pritish BawaPritish Bawa1 1 Deborah L. ReedeDeborah L. Reede1 1 Wendy RK SmokerWendy RK Smoker22 1 SUNY Downstate Medical Center Brooklyn NY 1 SUNY Downstate Medical Center Brooklyn NY

2 University of Iowa Hospitals and Clinics Iowa City IA2 University of Iowa Hospitals and Clinics Iowa City IA

Presentation # 1202

Disclosure Disclosure

The authors of this educational exhibit have The authors of this educational exhibit have no disclosures, conflicts of interest or no disclosures, conflicts of interest or financialfinancial relationships with commercial products.relationships with commercial products.

Objectives Objectives

The purpose of this exhibit is to:The purpose of this exhibit is to:

Heighten awareness that stroke occurs in infants, Heighten awareness that stroke occurs in infants, children and young adultschildren and young adults

Review the demographics, risk factors and etiologies of Review the demographics, risk factors and etiologies of stroke in various age groupsstroke in various age groups

Learn the imaging findings on various modalities Learn the imaging findings on various modalities (CT,CTA, MRA, MRV, spectroscopy and transcranial (CT,CTA, MRA, MRV, spectroscopy and transcranial ultrasound)ultrasound)

StrokeStroke DefinitionDefinition

WHO defines stroke as “a clinical syndrome of rapidly developing focal or global disturbance of brain function lasting >24 hours or leading to death with no obvious nonvascular cause”

Since this definition is far from ideal for children, researchers use other definitions for pediatric arterial ischemic stroke, such as “any neurological event including seizure associated with an acute infarction shown by MRI”

Stroke in Children and Young Adults

Among top 10 causes of death in children Results in death in 10%, neurologic deficit

or seizures in 70% and recurrent stroke in 20%

As common as brain tumors

Stroke Adults Children and Young Adults

Incidence of Cerebro-vascular events (annual) Ischemic

100-300/100,000

85%

2.5 - 13/100,000

55%Infants – Higher ischemic strokeOlder children (15-19 years) higher hemorrhagic stroke

Presentation

Etiologies (Common)

Abrupt onset of neurological deficit

Atherosclerotic diseaseCarotid stenosisAtrial FibrillationValvular diseaseDMSubstance abuseHBP SAHCoagulopathiesAnticoagulants

Neonates: seizures, hypotonia, lethargy

Infants: early hand preference (abnormal prior to age of 1 year)

Older infants, children and young adults: abrupt onset of neurological deficit

Hypoxic IschemicCongenital cardiac disease, CardioembolicVascular abnormalities FMD, Moyamoya,Hemoglobinopathies – Sickle cell diseaseProthrombotic states- Protein C/S deficiency, OCPs. Pregnancy, Antiphospholipid syndrome, Nephrotic SyndromeInfection CNS infection, Myocarditis, Sepsis/septic embolism, HIV, VaricellaTraumaSubstance abuseCerebral sinovenous thrombosis (CSVT)

Epidemiology 5% of all strokes occur in people younger than 45 years Childhood arterial ischemic stroke (AIS) 0.2-7.9/100,000 per

year Incidence of neonatal stroke less common approx.1 in 4,000

term live births Sinovenous thrombosis 0.67/100,000 in children and

neonates, over 40% in new born Hemorrhagic stroke 0.7-5.1/100,000 children (SAH

0.4/100,000 children) Peak age for both (ischemic and hemorrhagic – 1st yr. of life) More common in boys and African American children (not

explained by sickle cell disease in this population)

Imaging Ischemic StrokeCT vs MR

MR DWI most sensitive for cytotoxic DWI most sensitive for cytotoxic

edemaedema MRI and MRV preferred for CSVT MRI and MRV preferred for CSVT MRA especially useful for MRA especially useful for

craniocervical dissectioncraniocervical dissection

Disadvantage - Need cooperative patient or - Need cooperative patient or

sedationsedation

CT Sensitive for acute bleedingSensitive for acute bleeding Sensitivity decreases with timeSensitivity decreases with time Bland infarcts – low density within Bland infarcts – low density within

vascular territoryvascular territory CSVT – Linear densities in deep CSVT – Linear densities in deep

and cortical veinsand cortical veins CTA for vascular anatomy, rapid CTA for vascular anatomy, rapid

assessment of vascular lesions assessment of vascular lesions and arterial dissectionand arterial dissection

Disadvantage - AIS usually normal CT in first 12 - AIS usually normal CT in first 12

hrs.hrs.

- Radiation and IV contrast for CTA- Radiation and IV contrast for CTA

Overview

Neonates and Infants Children Young Adults

The etiologies of stroke vary in the pediatric and young adult population. Therefore the following discussion will focus on entities commonly encountered in the various age groups as listed below.

Classification

Ischemic Arterial Ischemic Stroke (AIS) Cerebral Sinovenous Thrombosis (CSVT)

Hemorrhagic Intracerebral Subarachnoid

Neonates and Infants (Birth-1yr.)

Stroke is rare in neonates, occur in approx.1 in 4,000 term live births

Clinical signs of are often subtle Congenital cyanotic heart disease is the most common

cause of embolic cerebral infarct in neonates

Common Etiologies• Hypoxia-ischemia• Thrombo-embolism• Infection• Metabolic disease• Trauma

Hypoxic-Ischemic Injury (HII)

Occurs in 1-4 infants per 1,000 live births Usually related to complicated or difficult delivery Patterns

- Peripheral

- Basal ganglia–thalamus Pattern depends on the duration and severity of the

insult

Hypoxic-Ischemic Injury Patterns

Depends on the severity and duration of hypoxic or ischemic event .

Mild to moderate Acute (<10 min) - Usually no significant clinical or imaging sequelae Prolonged (15–25 min) - shunting maintains blood flow to vital structures

(peripheral or watershed pattern of injury)

Imaging Involves parasagittal, watershed, and borderzone Seen in cerebral cortex and subcortical white matter with the parieto-

occipital and posterior temporal lobes typically more affected than the anterior regions

Severe (profound) Acute (<10 min) - inadequate shunting - metabolically active regions are most susceptible - basal ganglia - thalamus pattern of injury Prolonged (15–25 min) - catastrophic - total brain injury

Imaging DWI - restricted within the first 24 hrs., compare with ADC

Pseudonormalization at 1 wk Most useful 1–5 days after an asphyxia event.

T1 - days 3–7 - hyperintensity in posterolateral putamina, ventrolateral thalami, and corticospinal tracts Absent posterior limb sign - normal hyperintense focus in posterior limb of internal capsule

on T1 may be lost T2 - 2–3 days - normal hypointense foci seen in posterolateral putamina, posterior limb of

internal capsule, and ventrolateral thalamus on T2-weighted images become indistinct or abnormally iso- or hyperintense relative to adjacent white matter

Hypoxic-Ischemic Injury (HII)Patterns

Imaging

Modality Findings Timing

US Increased echo 2-10 days

CT Low attenuation 1-7 days

MRI

DWI Restricted 1-5 days

T1 T1 shortening 2 days - months

T2 T2 prolongation

T2 shortening

24 hours

6-7 days - months

Optimal Time to Perform Each Imaging TechniqueProton MR spectroscopy (MRS) is the most sensitive in 1st 24 hrs. Pseudonormalization at 24 hrs. (black box) due to fall in lactate. DWI is most optimal 1-5 days after injury after which is pseudonormalization.

Table demonstrates the sensitivity of conventional imaging modalities for the detection of stroke.

MRS

DWI

T1

T2

1 2 3 4 5 6Days of Life

Proton MR Spectroscopy

Increased specificity particularly first 24 hrs. of life

Healthy adult and term neonatal brains do not demonstrate a lactate peak

Pitfalls – Preterm infant (may have elevated lactate and decreased N-acetylaspartate - normal), CSF (normally has lactate, so exclude from voxel), pseudonormalization at 24 hrs.

Secondary rise in lactate levels, known as secondary energy failure, occurs after 24–48 hrs., with lactate level peaking at approximately day 5 after an hypoxic ischemic episode

Hypoxic-Ischemic InjuryUltrasound

May have hyperechogenicity in the thalami, globi pallidi, putamina, and periventricular white matter

Typically visible at 2–10 days of life

Decreased restive index (RI) - postulated to be due to impaired cerebral autoregulation

However, RI decreases as gestational age increases and must be correlated with gestational age for accurate results

         

New born – Transfontanellar US on day 1 of life shows slit like ventricles and slightly increased periventricular and thalamic echogenicity, better appreciated with high frequency transducer.

Periventricular Leukomalacia

Most common result of ischemic brain injury in premature

White matter adjacent to ventricles necrosis and cavitation shrinkage of cavities with focal enlargement of adjacent ventricle

US: periventricular echoes or cysts MRI: expansion of posterior portions of

ventricles from decreased volume; scalloping of lateral margin of ventricle; atrophy of splenium of corpus callosum; periatrial high T2 white matter signal

Neonates and Infants

USPremie 24 week s/p ex lap for spontaneous intestinal perforation , hypotensive and intubation. Ultrasound shows periventricular cysts on both sides, consistent with periventricular leukomalacia.

Long-Term Sequelae

Long-term sequelae of the basal ganglia-thalamus pattern of injury include atrophy and chronic mineralization in the thalami, posterior limb of the internal capsule, and basal ganglia

Associated generalized loss of volume in cortical gray matter and subcortical white matter may occur from disruption of normal development of neurons and axonal pathways

Premature infants who suffer moderate hypotension typically sustain injury to the periventricular white matter with sparing of the subcortical white matter and cerebral cortex.

Term infants who suffer from moderate hypotension demonstrate injury to cerebral cortex, particularly watershed areas, which lie between the anterior and middle cerebral arteries, and between the middle and posterior cerebral arteries

Neonates and Infants

Periventricular Leukomalacia (PVL)

Etiology

Hypoxia/ischemia +/- Hypoxia/ischemia +/- maternal/fetal infectionmaternal/fetal infection

Differentiating glial cells have Differentiating glial cells have high metabolic activity and arehigh metabolic activity and are vulnerable to hypoxiavulnerable to hypoxia

Imaging Findings

T2/FLAIR hyperintense signal T2/FLAIR hyperintense signal

in the periatrial white matter in the periatrial white matter

due to gliosisdue to gliosis Periventricular white matter Periventricular white matter

volume lossvolume loss focal ventricular scalloping,

dilatation and apparent infolding of the adjacent gyri

8y/o ex-premie with grade III IVH and PVL. T2 MR shows expansion of the posterior portions of lateral ventricles 2nd to decreased posterior white matter volume, and scalloped of contour of occipital horns.

Ex-premie child Ex-premie adult

26 y/o with spastic quadriplegia with h/o premature birth, twin B, (26 wk gestation) and neonatal asphyxia. T2 MR shows similar findings of PVL.

Neonates and Infants

Periventricular leukomalacia persists in the adult brain, even though the insult occurred in the perinatal period.

*** *

3 y/o with spastic quadriplegia, h/o viral myocarditis at 3 months age. T2 image at 3 yrs. shows increased signal in deep posterior watershed regions. Posterior deep white matter watershed infarct.

Hypoxia-Ischemia Full-Term

Neonates and Infants

In term infants, moderate hypotension results in injury to the cerebral cortex particularly in watershed areas, which lie between the anterior and middle cerebral arteries and between the middle and posterior cerebral arteries.

Illustrations shows the location of watershed areas.

Full term with neonatal distress delivered by C. Section; cardiac workup negative. Acute Lt.. MCA infarct on Imaging. DWI at 4 days of age shows restricted diffusion in left MCA territory. T2 image at 4 days of age shows increased signal in left MCA territory.

T2

Vascular Occlusion

Left MCA > Right MCA > ACA > PCA. The reason for this distribution is unknown. Hypoxic-ischemic injury complicated by large vessel occlusion in the distribution of the MCA is common.

Hypoxia-Ischemia with Large Vessel Occlusion Full- Term

DWI

Neonates and Infants

Neonatal Hypoglycemia Glucose is vital to normal brain function Profound hypoglycemia will result in significant brain damage Acute signs include jitteriness, seizures, and vomiting Significant hypoglycemia:

Glucose < 20-40 mg/dL in premature infants Glucose < 30-35 mg/dL in first 24 hrs. in term infants Glucose < 40-45 mg/dL after 24 hrs. in term infants

Imaging studies reflect diffuse brain damage:•Most severe in parietal and occipital lobes bilaterally •Acute phase:

- Reduced diffusion with edema of cerebral cortex and underlying white matter

- Lack of gray/white matter distinction•Chronic phase: cortex and white matter show cystic encephalomalacia atrophy

Neonates and Infants

Neonatal Hypoglycemia

4 day old presented with seizures, found to have hypoglycemia. DWI and ADC maps demonstrate bilateral acute infarcts in the parieto-occipital lobes.

DWI ADC

9 y/o F with spastic quadriplegia, blindness, mental retardation and microcephaly had h/o premature birth & neonatal hypoglycemia. MR shows bilateral occipito-parietal old infarcts with dilatation of the occipital horns. Shunt for hydrocephalus resulted in right subdural collection.

* *T2

Neonates and Infants

9 m/o with hemiparesis and history of CHD. T2 image demonstrates increased signal in the right posterior MCA territory with dilatation of the occipital horn c/w old right posterior temporal-parietal infarct .

Most focal arterial ischemic infarcts in full-term neonates and infants involve the middle cerebral artery (MCA) distribution. Cyanotic congenital heart disease (CHD) is the most common cause of embolic cerebral infarct in neonates and infants.

Thromboembolic DiseaseNeonates and Infants

*

Febrile illness with dehydration and thrombocytosis at 6 wks of age with subsequent hemiparesis. T2 at 17 mo. shows increased T2 signal in left ACA/MCA territory and dilated left lateral ventricle c/w old large left ACA/MCA infarct. MRA of Circle of Willis shows decreased flow in left ICA, ACA and MCA.

Can be idiopathic or can be secondary to any of the following: Infection – (e.g. Meningitis, URI, sepsis Chronic inflammation and vasculitis (e.g.

inflammatory bowel disease such as

ulcerative colitis)Tissue damage – (e.g. burns, trauma) Rebound thrombocytosis (e.g. Iron deficiency anemia) Postsplenectomy – (e.g. ITP) Hemolytic anemia – (e.g. Sickle cell) Renal disorders – (e.g. Nephrotic syndrome) Malignancy – (e.g., osteosarcoma) Low birth weight/ preterm infants

ThrombocytosisNeonates and Infants

Abnormal increase in number of platelets thrombotic state

Protein C/S Deficiency

Protein C system Formed by proteins C and S Natural anticoagulants that

regulate coagulation cascade by inhibiting factors Va. and VIIIa

Deficiency of either protein can be inherited or acquired hypercoagulable state

Associated with cerebral infarction in children

Neonates and Infants

DWI in a patient with neonatal seizures at 4 days of age shows restricted diffusion in left frontal parietal region c/w acute infarct. MRA of Circle of Willis at 4 days of age shows a paucity of left perisylvian MCA branches.

DWI MRA

Thrombus Site Infarct LocationSagittal Sinus ParasagittalStraight Sinus / Vein of Galen ThalamusVein of Labbe, Transverse or Sigmoid Sinus

Temporal Lobe

Vein of Galen MalformationVenous infarcts are diagnosed based on characteristic location and appearance.

Newborn with a vein of Galen malformation diagnosed on prenatal ultrasound. Sagittal T1 image of a newborn shows a vein of Galen malformation. Post contrast image shows increased signal in right basal ganglia and thalamus after embolization suggesting deep venous infarct.

InfarctInfarct

MalformationMalformationpost embopost embo

T1T1 FS Post

Neonates and Infants

Children (1 yr-14 yrs.)

Etiologies:• Congenital heart disease• Blood dyscrasias• Metabolic disorders• Vasculopathies• Infection• Trauma• Venous thrombosis• Drug ingestion

Carotid DissectionCarotid Dissection

Rare and under recognized cause of stroke

7.5% of ischemic strokes in children Mostly extracranial ICA, typically

pharyngeal portion Recanalization occurs in 60% Risk of recurrent stroke or TIA is 12% Imaging Arteriography – String sign; double

lumen sign; short smooth tapered stenosis; and occlusion of parent artery

MR with FS T1 and contrast enhanced MRA are valuable

*

15 m/o F with non-accidental trauma. CT shows large hypodense right MCA infarct. No contract is identified in the right internal carotid on axial CTA of the neck . Sagittal image demonstrates short smooth tapered narrowing c/w right carotid dissection.

Children

rare in childhood Can occur spontaneously or 2nd

to injury (blunt trauma, hyperextension etc...)

Cervical segment of carotid artery > vertebral artery > proximal portions of middle cerebral artery

With a tear in the intima, blood separates the intima from the media forming a hematoma which progressively reduces the arterial lumen and can eventually occlude it

Vertebral Artery Dissection

•..

9 y/o F with stroke-like symptoms.Selective left vertebral angiogram shows Selective left vertebral angiogram shows vertebral artery dissection. MR shows high T2 signal in left superior cerebellum indicative of left cerebellar infarct from idiopathic vertebral artery dissection

Children

Vertebral Angiogram T1

Stroke in DrowningChildren

5 year old boy - Accidental Drowning:5 year old boy - Accidental Drowning:

Initial CT shows diffuse Initial CT shows diffuse low attenuation in both cerebral hemispheres low attenuation in both cerebral hemispheres with with sulcal and ventricular effacement suggesting global hypoxia. Follow up MRI sulcal and ventricular effacement suggesting global hypoxia. Follow up MRI shows shows restricted diffusion and and high FLAIR signal c/w bilateral infarcts in the signal c/w bilateral infarcts in the basal ganglia , midbrain and cortical and subcortical regions.

CT ADCFLAIR

Duchenne Muscular Dystrophy• No direct causal relationship established• Mostly attributed to cardiomyopathy, cardiac failure and arrhythmias

14 y/o M with Duchenne Muscular Dystrophy: NCCT shows dense MCA sign and large hypodense area in left MCA territory,

confirmed on DWI and ADC as area of restricted diffusion suggesting acute infarct. Also noted is an old infarct in right basal ganglia.

Children

CT DWI ADC

Meningitis

5 y/o F presented with fever, altered mental status and left hemiparesis, diagnosed with meningitis. MR shows extensive meningeal thickening and enhancement, mostly perimesencephalic consistent with meningitis. DWI and ACD demonstrate an acute right thalamocapsular infarct.

Meningitis and encephalitis are the etiologies of stroke in 10% of arterial ischemic stroke

Etiology Vascular inflammation and thrombosis

due to reduced cerebral vascular perfusion 2nd to systemic hypotension,

increase intracranial pressure and lowers CSF glucose

Middle ear and paranasal sinus infections can cause cerebral venous sinus thrombosis (CVST)

T1 Post Contrast ADC

DWI

Children

Cerebral Sinovenous Thrombosis (CSVT)

Defined as the presence of thrombus or flow interruption within cerebral veins or dural venous sinuses

Incidence is 0.67 per 100,000 children per yr. over 40% occur in newborns Location: superior sagittal, transverse or sigmoid sinuses Risk factors: dehydration, intracranial infection, hypercoagulable states,

anemia, pregnancy and mass lesion compressing the dural sinus

Imaging Findings MR - MR and MRV are studies of choice - MR: acute clot appears hypointense (deoxyhemoglobin) on T2; subacute

clot appears hyperintense (methemoglobin) on T1 and T2 CT - NCCT: hyperdense cortical vein, subcortical infarct (commonly

hemorrhagic), and brain edema - CCT: filling defect in dural sinus Cerebral angiography is still the gold standard especially when CT or MRI

are not definitive

Children

Venous Thrombosis Incidence of sinovenous thrombosis, as per the Canadian Pediatric Ischemic Stroke Registry, was 0.67 cases per 100,000. Children and neonates were the most commonly affected age group. In neonates, clinical presentation included seizures and diffuse neurologic signs. Clinical presentation in non-neonates typically includes decreased level of consciousness, headache, focal neurological sign and cranial nerve palsies.

Post contrast T1 shows thrombosis in superior sagittal and transverse sinuses in this10 y/o M with headaches. Increase signal is noted in the parasagittal white matter c/w venous infarcts. Illustration demonstrates the location of the intracranial venous structures.

T1 Post FLAIR

Children

Fibromuscular Dysplasia (FMD)

Common in adult women, rarely in children 20-30% have cerebrovascular involvement Affects medium and large arteries -- unknown etiology Overgrowth of smooth muscle and fibrous tissue in the

vessel wall Different appearances: Type I “String of beads”

(classic), type II long tubular stenosis, type III confined to portion of arterial wall

Renal artery (60%) > ICA or vertebral arteries (35%) > iliac arteries (3%) > visceral arteries (2%)

Fibromuscular dysplasia is a well-recognized cause of stroke and transient ischemic attacks in adults but a seldom-recognized cause of stroke in children.

Children

Fibromuscular Dysplasia

3 y/o F with acute right hemiplegia and hypertensive encephalopathy. HTN workup demonstrated renal FMD. Further workup showed intracranial and cervical FMD associated with left frontal and parietal infarcts.

T2 image shows left frontal and parietal encephalomalacia. There is decreased flow in the left ICA, ACA and MCA on an MRA of the Circle of Willis . Neck MRA lateral view demonstrates FMD affecting the mid cervical IAC.

Children

T2 MRA Circle of Willis Neck MRA

Sickle Cell Disease 

  25% have CVD by age 45  -  Ischemic in children  -  Hemorrhagic in adults   Most common hematologic risk factor for

stroke High recurrence rate Narrowing of distal ICA, proximal MCA, and

ACA  Sometimes associated with Moyamoya

syndrome with progressive vasculopathy of intracranial ICA with distal collateral vessels

Caused by anemia and hypercoagulable state Silent infarcts occur in MCA territory and/or

border zones  Occasionally develop sinovenous thrombosis

or anterior spinal artery syndrome

Children

FLAIR MRA

30 y\o M SSD with history of strokes in childhood. MRI shows old infarcts in both ACA territories. MRA shows stenotic ACAs with attenuated branches and prominent lenticulostriate collaterals on the right.

Moyamoya

Described in 1969 by Suzuki and Takaku, means “hazy, like a puff or cloud of smoke.”

Associated with wide range of entities (Sickle cell disease, phakomatoses, Down’s syndrome) or idiopathic 20%

Imaging Anterior circulation > vertebrobasilar system MRA: Narrowing of supraclinoid ICA MRA: Multiple collateral vessels from A1/M1 MRI: “Holes” in basal ganglia on T1 & T2

- Collateral flow in thalamoperforating and lenticulostriate arteries accounts for “puff of smoke” in basal ganglia.

- Dilated and more numerous lenticulostriate arteries

Location of the supraclinoid carotid artery

Segments of the Circle of Willis

Children

Moyamoya 2nd to Sickle Cell Disease

15 y/o M with h/o SSD and headaches: MRI shows prominent bilateral collateral lenticulostriate vessels. Magnified views shows findings to better advantages. Illustration demonstrates the location of the lenticulostriate arteries.

Angiographic ICA staging of steno-occlusive lesions:

Stage I: Narrowing of the carotid bifurcation

Stage II: Dilatation of the ACA and MCA with appearance of ICA moyamoya

Stage III: Partial disappearance of ACA and MCA with intensification of ICA moyamoya

Stage IV: Advanced steno-occlusive stages in ICA with small amount of ICA moyamoya

Stage V: Absence of the ACA and MCA with further reduction of ICA moyamoya

Stage VI: Blood supply only from ECA with almost complete disappearance of ICA moyamoya

T1

Children

Sickle Cell DiseaseSickle cell disease causes progressive cerebral vasculopathy in some patients,

causing overt strokes in 5%-10% and silent strokes in 11%-17% of children.

Children

5 y/o M with multiple previous episodes of TIA and stroke. Neck MRA shows absence of flow in right ICA compared to normal left ICA; MRI brain finding (small area of increase T2 signal in the right centrum semiovale) underestimate right brain vascular pathology. MRA of Circle of Willis shows prominent collateral lenticulostriate vessels. MR findings underestimate left brain vascular pathology.

Young Adults (15-30 years old)

Etiologies

Cardiac emboli Arterial dissection Recent Pregnancy Coagulopathy Vasculitis Smoking and Drug abuse Premature atherosclerosis,

dyslipidemia and hypertension

GRE

Drug AbuseDrug Abuse

21 y/o F with 10 yr. h/o using molly (molecular pure ecstasy). CT and MR GRE and T1 images shows acute hemorrhage in right frontal lobe and thin right hemispheric subdural hemorrhage. DSA image shows vasoconstriction of the ACA branches.

Cocaine and amphetamines have the strongest association with stroke May be most common predisposing condition for stroke in patients <35 y/o 12.1% of patients between 15-44 y/o with AIS have recent drug use Mechanism of injury mostly hypertensive surges, vasospasm, enhanced platelet aggregation, cerebral vasculitis, accelerated atherosclerosis an cardio embolism

CT T1 DSA

Young Adults

Vasculitis

Diagnosis of exclusion Caused by wide range of entities

- Collagen vascular disease (systemic)- CNS infection: Meningitis, encephalitis- Drugs- Autoimmune disorder (i.e. primary angiitis of CNS)Imaging Findings Angiography: - Long segments of multiple focal arterial narrowing - Sensitivity = 70% CT/MRI – nonspecific findings - Single or multiple infarcts of various sizes - Hyperintense white matter lesions on T2 - Leptomeningeal enhancement

MRI advocated as sensitive screening tool with high negative predictive value

Young Adults

Primary Angiitis of the CNS

Necrotizing Vasculitis with a predilection for the central nervous system (CNS), histologically referred to as granulomatous angiitis of the CNS, of unclear etiology

Nonspecific symptoms include headache, malaise, mental status change, focal neurological deficits and seizure.

Rapidly progressive and frequently fatal Commonly involves vessels of brain

parenchyma and leptomeninges with predilection for small arteries and arterioles

Arteriogram- Focal or multifocal segmental stenoses of

small and medium sized vessels in parenchyma and leptomeninges

- May be abnormal in approximately 85% of cases

- May be negative in 15% of cases – when disease involves precapillary arteriole

Young Adults

14 y/o F with headache and altered mental status. T2 image shows frontal and bilateral parietal cortical and subcortical encephalomalacia.

Patent Foramen Ovale (PFO)Remnant of embryological development with clinically important consequences including thromboembolism, stroke and migraine headaches. The proposed mechanisms for stroke include paradoxical embolization, in situ thrombosis within the canal of the PFO, and concomitant hypercoagulable states.

PFO is “the back door to the brain”

Small venous thrombi not filtered by the pulmonary vasculature enter the systemic circulation

Can result in infarcts in multiple organs

24y/o M with a PFO presented with multiple pulmonary emboli and right hemiplegia . CT shows a large left MCA territory infarct and a smaller right frontal infarct.

Young Adults

Stroke Differential Diagnosis

Complicated migraine typically resolves within 24 hours   Family history of migraine or hemiplegic migraine Todd’s paresis (post ictal hemiparesis) Intracranial neoplasms Intracranial infections like meningitis, brain abscess, herpes

encephalitis Alternating hemiplegia Metabolic abnormalities  - Hypoglycemia -  MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke) 

Strokes in Infants Children and Young Adult Etiologies

Hypoxia-ischemia - Premature or full term newborn with perinatal distress - Newborn with hypoglycemia - Infant with hypoperfusion (e.g. viral myocarditis with hypotension)

Thrombo-embolism - Cardiac causes (congenital heart disease, vascular dissection, patent foramen

ovale, mitral valve prolapse) - Polycythemia - Trauma - Vasculopathy (Sickle cell disease, Moyamoya, FMD, Kawasaki)

Infection (viral or bacterial meningitis) - Maternal drug abuse

Coagulopathy - (Protein C/S deficiency, Factor V Leiden, antiphospholipid antibody syndrome) Vascular malformation (Vein of Galen malformation, AVM) Metabolic disorders (mitochondrial disorders, hyperhomocysteinemia, lysosomal

storage disorders, hyperlipoproteinemia, disorders of cholesterol and triglyceride metabolism)

Thank you for viewing our exhibit! Thank you for viewing our exhibit!

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Esse, Katherine, et al. "Epidemic of illicit drug use, mechanisms of action/addiction and stroke as a health hazard." Brain and behavior 1.1 (2011): 44-54.

Roach, E. Steve, et al. "Management of stroke in infants and children a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young." Stroke 39.9 (2008): 2644-2691.

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References

We hope you learned more about strokes in infants, children and young adults after reviewing our educational exhibit. Feel free to email us if you have any questions or comments at:

pritish.bawa@downstate.edu