stroke in pediatric population

1. Dr Rahi kiran
SR Neurology
GMC Kota

2.  Stroke-important cause of acquired brain injury in newborns
and children.
 Relatively rare-children- Arterial orVenous stroke.
 Incidence of Arterial ischemic stroke (AIS) and CSVT -
5/100,000/yr and affects 1 in 2000 newborns.

3.  A review of 13 years of data in New Jersey found equal rates of ischemic and
hemorrhagic stroke
 Neonates - higher risk than older children.
 Symptomatic ICH affects 1 in 100 full-term neonates.
 Asymptomatic subdural hemorrhage affects almost half of term neonates and can
occur in infants delivered by both vaginal and cesarean delivery
 arterial ischemic stroke around 1 in 4000 neonates

4.  Strokes also occur in utero, Unilateral spasticity accounts for
19% to 35% of the total CP
 rate of 0.67 cases of cerebral venous thrombosis (CVT) per
100,000 children per year, with neonates making up 43% of
cases

6.  Cardio embolism
 Congenital cardiac malformations
 Acquired
 Rheumatic heart disease/endocarditis
 Cardiomyopathies, arrhythmias
 Prosthetic , Prolapsed valves

7.  Vasculitis &Vasculopathies
 Infectious/ Immune/ Iatrogenic vasculitis
 Migraine
 Hypertensive encephalopathy
 Moya moya disease
 Metabolic & related disorders
 Homocystinuria
 MELAS
 Neuroectodermatoses

8.  Hematologic & Hypercoagulable states
 Hemoglobinopathies- Sickle cell anemia
 Polycythemia
 Thrombocytosis
 Leukemia, Lympho-reticular malignancy
 Protein C, S deficiency
 Antithrombin III deficiency
 Nephrotic syndrome

9.  spontaneous intracranial hemorrhage-
 55% arteriovenous malformation
 18% aneurysm(mc carotid bifurcation)
 14% cavernous malformation,
 14% unclear etiology

10. Sudden onset of pediatric stroke-no specific cause
delineated.
 Thrombotic occlusions of carotid artery or branches of
middle cerebral artery- frequently documented causes of
strokes in children.
 MRA- demonstrate significant vascular abnormalities in
75% of children with strokes.
 Infants- seizures, motor signs few, abnormal hand
preference.
 Older children- sudden onset of hemiparesis, seizures.

11.  Primary vascular disease- stenosis- occlusion of intracranial portion of
Internal carotid artery & proximal ACA and MCA.
 Chronic inflammatory, occlusive intracranial vasculopathy affecting ACA,
MCA associated with extensive network of collaterals.
 Changes in pulmonary, renal, pancreatic arteries.
 Multiple telangiectasias in basal ganglia- hazy, smoke like appearance-
Japanese word moyamoya applied.
 Pattern represents opening of collateral channels.

12.  Symptoms in childhood- females frequent.
 MultipleTIA with or without residua ,Sudden
hemiparesis,, Seizures- 33 % - children <6yrs.
 Disease is progressive
 Poor prognostic factors- early age of onset, typical
clinical pattern, involvement of dominant hemisphere
or both hemisphere, complete occlusion of cerebral
blood vessels.

13.  Cerebral angiography- definitive diagnosis.
 Revascularization procedures like - STA-MCA procedure(of
choice), Encephalo duroarterio synangiosis (EDAS) or
multiple burr holes procedure in treatment of moya moya
disease.

15.  Pulseless disease
 Chronic large vessel vasculitis of unknown etiology
 Predom. Involves aorta & branches.
 Females common-15-20 yrs.
 Arteritis involving aorta & its branches - Inflammation of vessel wall-
granulomatous inflammation in media- blood vessel dilatation & aneurysm
formation.
 Hypertension, absent pulses, vascular bruits.

16.  Non atherosclerotic, non inflammatory vascular disease that causes
abnormal growth within wall of artery.
 Common arteries- carotid & renal arteries.
 Cause for childhood stroke & secondary hypertension.
 Fibromuscular dysplasia of intra cranial vessels in children rare.
 Angiographic finding of string of beads appearance of artery.

17.  Hemiplegic migraine- transient hemiparesis with severe headache.
 Familial forms & sporadic forms.
 Transient loss of vision in one eye- amaurosis fugax
 The risk is more apparent for individuals who have migraine with aura,
smokers, and women who use oral contraceptives

18.  Children with complex congenital heart disease are
at risk for cardioembolic stroke, thrombotic stroke,
watershed infarcts from drops in perfusion pressure
and CVT
 27% of children with stroke associated with cardiac
disease had recurrent stroke.
 Mechanical heart valves, prothrombotic conditions,
and infection - vegetation

19.  CVA- complication-TOF, TGA- common.
 “Any child < 2 yrs with CHD , who has acute onset of neurological
signs- CVA should be considered as primary diagnosis ‘’
 Chronic hypoxemia in severe cases of congenital heart disease may
lead to polycythemia,

20.  Sickle cell anemia (SCA)
 most common Hemoglobinopathy assoc. with CVA.
 Incidence- 700 per 1 lakh children with SCA.
 Stroke in SCA- large vessel disease, venous occlusion or intracerebral
hemorrhage(very rare)

21.  Strokes in children with SCA- highest incidence in 5-10 yrs.
 Hemiparesis- most common symptom, Aphasia- 20 %, Seizures- 15 %,
TIA- 10 %
 Children with HBSS - highest incidence of stroke- monitor with trans
cranial USG.

22.  Blood transfusion & exchange transfusion- standard mode
of treatment of acute stroke in SCA.
 Periodic blood transfusions- decrease productions of sickle
cells- reduce recurrence of strokes by 90 %.
 incidence of first stroke in children with sickle cell anemia
dropped from 0.88 per 100 person-years to 0.17 per 100
person-years
 exchange transfusion -The Stroke Prevention in Sickle Cell
Trial (STOP) - keep the HbS < 30% reduced the risk of
recurrent stroke by 90%

23.  Bleeding occurs in 25 %.
 Bleeding more common in factor IX deficiency.
 Intra cranial Hemorrhage common in children <18 yrs, esp. <3yrs.
 Serious permanent deficit- 50 %, mortality- 35 %.
 Complications of labour or delivery produce intracranial hemorrhage in
newborns with hemophilia.
 Treatment- replace deficient clotting factors- performed prophylactically.

24.  Homozygous deficiency of Protein C in newborns- venous thrombosis-
thrombosis of cerebral veins.
 Strokes –significantly reduced levels of protein C.
 ITP- major intracranial hemorrhage can occur.
 Subdural, intra parenchymal, intra ventricular hemorrhage- infants b/w
2 weeks - 12- Vitamin K deficiency.
 Hemolytic uremic syndrome- seizures, depressed consciousness,
subarachnoid hemorrhage, hemiparesis,.

25.  Stroke affects approximately 1% of children with cancer
 at risk for both intracranial hemorrhage and ischemic infarction.
 Intracranial hemorrhage- 20% , secondary to thrombocytopenia
 infarction or CVT due to leukostasis
 CVA also occur after bone marrow transplantation.

26.  Acute bacterial meningitis- vasculitis, vasospasm,
intracranial aneurysm formation.
 Stroke common sequel of Severe meningitis- Group B
streptococcal & Listeria (<2 months), H. Influenza,
Pneumococcal (>2 months),Tuberculous meningitis.
 Occlusion of veins or dural sinuses- follow otitis media,
mastoiditis, sinusitis & infection of scalp & face.
 Post varicella angiopathy, mycoplasma pneumonia, borrelia-
burgdorferi, chlamydia pneumonia, HIV, helicobactor pylori,
hemolysing streptococci- predisposition for stroke.

27.  HIV : marantic endocarditis,
arteriopathy of medium and small vessels or
aneurysms,
secondary infection,
ART - dyslipidemia – accelerated atherosclerosis
 Brucellosis
 Lymes disease

28.  SLE- 40% of patients have neurologic abnormalities- psychiatric,
behavioral abnormalities & focal neurologic signs.
 HSP- headaches, mental status changes, seizures, focal neurologic
deficits, involvement of peripheral nerves.
 Kawasaki disease- aseptic meningitis, hemiparesis.

29.  Infants with fever & dehydration- primary venous or sinus thrombosis.
 Hypernatremic dehydration- seizures, depressed state of
consciousness.
 Juvenile onset Insulin dependent Diabetes mellitus- acute
hemiparesis.

30.  MELAS SYNDROME- epilepsia partialis continua or status epilepticus,
repeated strokes.
 MELAS - L-Arginine improves endothelial dysfunction- in treating the
stroke-like episodes
 Homocystinuria may lead to infarction, presumably through elevated
homocysteine levels and subsequent vascular injury.
 Homocysteine – FA, B6, B12

31.  Fabry disease is an X-linked LSD - deficiency of α-galactosidase -
accumulation of glycolipids in the endothelial wall.
 Both male and female heterozygotes are susceptible to cerebral
thrombosis - increase in vasoreactivity in damaged vessels or
endothelial and leukocyte activation.
 Males may be more severely affected but rarely show cerebrovascular
involvement before age 23.

32.  Trauma to carotid artery- delayed onset of neurological signs-
thrombosis in vessel & extension into cerebral vessels.
 Severe cerebral edema- death.
 Permanent neurological residua- seizures & neuropsychological
deficits.

33.  External trauma to carotid artery- hematoma on lateral portion of
neck, Horners syndrome,TIA followed by lucid interval –then sudden
onset of hemiplegia or hemiparesis.
 trauma to cervical spine- sudden twisting or jerking of head- injure
carotid or vertebral arteries.
 Basilar skull fracture can cause laceration of carotid artery at foramen
magnum- severe bleeding from mouth & ipsilateral ear.

34.  Arteriovenous malformations
 4 types of vascular malformations- Arteriovenous malformations, venous
angioma, capillary telangiectasias, cavernous angioma.
 Admixture of normal & abnormal blood vessels.
 Surrounding brain contains areas of fibrosis, inflammation, glotic
changes, calcification.
 Seizures- common clinical abnormality than hemorrhage.
 Subarachnoid, intraparenchymal, or combined can occur.

35.  Vascular malformations may be located in cerebellum & brainstem.
 50 % of children with intracranial arteriovenous malformations have
bruits heard over head.
 “A cranial bruit heard in an infant younger than 4 months of age ,
even in the presence of loud cardiac murmur , is always assoc. with
intracranial arteriovenous malformation”

36.  CT with contrast, MRI, Arteriography.
 RX- surgical accessibility of lesion.
 Total surgical excision is curative,
 Embolization of lesion may be effective.
 Stereotactic radiosurgery with linear accelerator – effective modality
for some patients.

37.  Arteriovenous malformation ofVein of Galen- direct connection
between branches of carotid or vertebral circulation &Vein of Galen.
 Vein undergoes aneurysmal dilatation because of high pressure &
arteries divide forming a network of vessels adjacent to the vein.
 Development of malformation in infancy forms a hemodynamically
significant arteriovenous shunt.

38.  Neonatal period- signs & symptoms of high output congestive heart failure.
 Children- systolic heart murmur, cranial bruit, cardiomegaly, hepatomegaly,
tachycardia, respiratory distress, & pulmonary edema.
 Death – cardiac failure.
 Presentation in later infancy- Hydrocephalus, Subarachnoid hemorrhage.
 Dilated veins over scalp, intracranial bruits.
 Poor prognosis- death from hemorrhage, increased intracranial pressure or
cardiac failure.

39.  Presentation in later life- Headache, signs of intracranial hemorrhage- convulsions & focal
neurological signs.
 Signs of brain stem dysfunction & raised intracranial pressure.
 Calcification within malformation on CT scan.
 Arteriography- diagnostic.
 RX- difficult- location, surrounding network of blood vessels, poor cardiovascular status of
pt.
 Microsurgical techniques & staged surgical procedures.
 Embolization not effective.

40.  Uncommon in children less than 10 yrs.
 Located in either anterior or posterior circulation.
 Sudden onset of massive subarachnoid hemorrhage & depressed state
of consciousness.
 Commonly occur on anterior cerebral artery or internal carotid artery.
 Usually >1cm – intracranial hemorrhage, seizures.
 Surgery, microsurgical techniques- definitive RX for aneurysms.
 Aneurysm not removed- 50 % will bleed- serious neurological deficits.

41.  Sturge weber syndrome
 Port wine stain on face & scalp , capillary venous angioma of meninges,
vascular abnormality within cortex & white matter of ipsilateral
hemisphere.
 Intractable Seizures, hemiparesis, mental retardation.
 Eye- glaucoma, angioma of retina & choroid.
 Early excision of abnormal areas of cortex.
 Affected area is large- hemispherectomy

42.  Common in children under 3 yrs, often during first year of life.
 Septic venous sinus thrombosis- most common in neonates.
 Occlusion of sagittal sinus in older children- syndrome of pseudo tumor
cerebri , headache, CN 6 palsy- false localizing sign, papilledema, visual
loss.
 Good prognosis.
 CT, MRV
 anticoagulation

43.  Clinical- seizures, increased intracranial tension, decreased level of
consciousness.
 Predisposing factors- otitis media, mastoiditis.
 Otitic hydrocephalus- when otitis media & mastoiditis led to lateral
sinus thrombosis & increased intracranial pressure.
 Vigorus RX for otitis media & mastoiditis

44.  Cavernous sinus- CN 3, 4, 6, ophthalmic division of CN5, internal
carotid artery.
 Rupture of artery- massive arteriovenous shunt with proptosis, bruit,
involve. of CNs.
 Internal carotid artery becomes thrombosed in segment- cavernous
sinus- massive hemispherical infarction.
 Predisposing factors- infection of orbit, paranasal sinus, skin of
periorbital & malar areas.

45.  Clinical- conjunctival suffusion- peripheral conjunctival capillaries,
conjunctival edema, retinal edema.
 Ptosis- CN 3.
 External opthalmoplegia- CN3, 4, 6.
 Septic cavernous sinus thrombosis- medical emergency- vigorous
antibiotic therapy.
 No role for anticoagulant therapy.

46.  Cocaine, amphetamines, marijuana
 Vasospasm – ischemia, rise in BP - ICH

47.  CECR1 – ADA 2
 AB collagen type IV alpha
 glutathione peroxidase gene (GPX3)
 ADAMTS13 -VWF

48.  Older children : Hemiplegia, Hemi sensory loss, Aphasia &
other neurological deficits.
 Younger children(< 1 yr) : subtle, variable findings,
seizure(mc),
early hand preference, limp during walking.

49.  Todds paralysis-Transient post ictal hemiparesis(< 24hrs,
rarely few days)
 Hemiplegic migraine (temporary motor deficit)
 Syndrome of alternating hemiplegia
 ICSOL ( Intracranial space occupying lesion )
 ADEM, MS, vasculitis

50.  H/o delivery , perinatal period and basic developmental milestones.
 Development of a hand preference before 1 year of age may be a sign of a mild
hemiparesis
 H/O ear, throat, mastoid infection.
 H/O intra oral or neck trauma.
 H/O cardiac d/s.
 H/O Hematological disorders.
 H/O multifocal seizures, raised intracranial pressure, vomiting- ? Superior sagittal sinus
thrombosis.
 H/O Hemiparesis & seizures in first two years of life- ?
Arterial occlusions.

51.  face - signs of dysmorphic features
 head circumference - macrocephaly due to hydrocephalus ,
microcephaly due to tissue loss
 Skin – pallor, cyanosis, petechiae, neurocutaneous markers
 Auscultate – bruit/ murmur

52.  Standard Evaluation
 CT scan (plain)
 If CT scan is normal , MRI scan (plain)

53.  ECG, CXR, Echocardiography
 Carotid Doppler studies
 Collagen vascular screen
 PT, APTT.
 Protein C, Protein S, Antithrombin 111.
 Others-VDRL, HIV, Urinalysis, Lactate/Pyruvate levels, Homocysteine
 Urinary & blood aminoacids

54.  TCD - screen children with sickle cell anemia - high flow velocities -
higher stroke risk.
 Magnetic resonance angiography (MRA).
 Conventional angiography - the most accurate method - vascular
malformations , moyamoya, vasculitis, and dissection
 Digital subtraction angiography (DSA).

55.  CBC, Platelet count, clotting factors.
 LFT.
 Imaging studies.

56.  Arterial Ischemic Stroke
 No randomized control trial on children with AIS.
 Treatment primary directed towards stabilizing systemic factors &
management of underlying causes.
 Supportive care
 Manage raised intracranial pressure, blood pressure & fluid balance.
 Blood glucose carefully monitored-Hyperglycemia exacerbate infarct
size.
 Maintain normal body temperature.
 Aggressive antiepileptic treatment.

57.  Antithrombotic therapies
 Use of anticoagulant therapy increasing in pediatric AIS.
 No clear guidelines are available on the use of heparin in pediatric
arterial stroke
 protein C or S deficiency, or with antiphospholipid antibody syndrome -
long-term anticoagulation
 Neonatal AIS - Antithrombotic therapy is rarely indicated in most
cases because of negligible recurrence risk

58.  Oral anticoagulation
 Congenital or acquired heart disease
 Severe hypercoagulable states
 Arterial dissection
 Recurrent AIS orTIA while on aspirin.

59.  Thrombolytic agents
 No evidence-based guidelines are currently available on using
intravenous or intra-arterial thrombolytics in children..
 Non thrombotic therapies
 Transfusion therapy
 Neuroprotective agents – no role
 Immunosuppressants

60.  Surgical evacuation of hematomas, insertion of ventricular or
lumboperitoneal shunts & rarely revascularization procedures.
 Rehabilitation therapy
 Speech therapy
 Occupational therapy
 Physical & psychological therapy

61.  Stroke in children relatively rare.
 Recurrence is low in neonates
 There are fundamental, etiologic & developmental differences
in children compared with adults .
 Multiple causes for stroke in children & many risk factors.
 Hemorrhagic stroke higher mortality than ischemic stroke.

62.  Bradley’s textbook of neurology 7th ed
 Nelson textbook of Pediatrics.19th edition;2080-2086.
 Arterial Ischemic Stroke in Children andYoung Adults,Warren
D et al. Continuum ReviewArticle, 2017
 PediatricArterial Ischemic Stroke, Moharir M. Continuum
ReviewArticle, 2014