Strokes in children: Pathophysiology, Etiology & Management

1. STROKES IN
CHILDREN
DR ROBIN THOMAS
RESIDENT IN PEDIATRICS
JJMMC, DAVANGERE

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

3. Blood supply of Brain & Spinal cord

4. Blood supply of Brain

5. Circle of Willis

6. Circle of Willis

8. Blood supply of Brain
 Brain: 700-800 ml blood/min (55ml/100g/min)
 < 30 ml/100gm/min: leads to ischemia
 80 % blood to gray matter & 20 % to white matter.
 Blood supply to brain
 Carotid system- 2 Internal Carotid arteries
 Vertebro basilar system- 2 Vertebral arteries

9.  Internal carotid artery has 4 portions:
 1.Petrous PC3
 2.Cervical
 3.Cavernous
 4.Cerebral

10.  Branches of Cerebral portion of Internal Carotid artery are:
 Middle cerebral artery MAAPO
 Anterior cerebral artery
 Anterior choroidal artery
 Posterior communicating artery
 Ophthalmic artery

11.  Vertebro basilar system
 Brain stem, Thalamus, temporal lobe-inferior portion, occipital lobe
 1. Vertebral artery
 2. Basilar artery
 3. Posterior cerebral artery

12. Circle of Willis
 1. Middle cerebral artery (MCA)
 2. Anterior cerebral artery (ACA)
 3. Posterior cerebral artery (PCA)

13. Internal Capsule

16. Blood Supply of Internal capsule
 Anterior limb : Superior half- Lenticulo-striate artery- MCA
Inferior half- ACA- Huebners artery
 Genu : Lenticulo-striate artery
 Posterior limb : Superior half- Lenticulo-striate artery- MCA
Inferior half- Posterior communicating artery,
Anterior choroidal artery.

17. Venous drainage of Brain
 Sinuses & Veins
 Paired Sinuses : Cavernous sinus
Superior & Inferior petrosal sinus
Transverse sinus
Sigmoid sinus
 Unpaired Sinuses : Superior & Inferior Sagital sinus
Straight sinus
Anterior & Posterior Intercavernous sinus

18. Pathophysiology
 Interruption of blood flow to the part of the brain
 Rupture of blood vessels with bleeding into cerebral parenchyma.
 Blood supply of brain- carotid & vertebro-basilar circulations
 Numerous anastomosis at the level of circle of willis & through smaller
vessels in leptomeninges.
 Diencephalon- supplied by end arteries- anastomosis not adequate-arterial
occlusions have devastating effect.
 Water shed zones- portion of cerebral cortex b/w 2 major arteries- less
affected by arterial occlusions –damage when cerebral perfusion pressure
is reduced.

19. Pathophysiology
 Interruption of blood flow of arterial or thrombotic d/s- Ischemic stroke.
 Rupture of blood vessels with bleeding into cerebral parenchyma-
Hemorrhagic stroke.
 Trauma or intimal tears- ICA injury.
 Interruption of function – infections, inflammation of intima- severe meningitis,
post varicella angiopathy, mycoplasma pneumonia, borrelia, chlamydia, HIV,
Helicobactor, Hemolysing streptococci.
 Embolisation- Cardioembolism- CCHD, RHD, Prosthetic or Prolapsed
cardiac valves, Cardiomyopathy, Arrhythmias.

20.  Decreased cerebral blood flow- high cerebral metabolic rate & paucity of
energy stores in CNS.
 Cerebral metabolic rate for oxygen- 3.5 ml/100mg brain/min.
 Cerebral hypoxia- oxygen partial pressure < 40 torr.
 Glucose storage in brain- survival of cerebral tissue for 90 min.

21.  Preterm infants
 Sub ependymal germinal matrix- highly cellular well vascularized area
beneath the ependyma of lateral ventricles- major site of neurogenesis.
 Sub ependymal hemorrhage & Intraventricular hemorrhage- common form
of CVD in preterm infants.
 Bleeding in highly vascular sub ependymal germinal matrix & then rupture
into lateral ventricle.
 Hemorrhagic infarction of periventricular white matter-15% of infants with
intraventricular hemorrhage.

22.  Several factors- fluctuating cerebral blood flow, increase in cerebral
venous pressure, immature capillaries in germinal matrix, abnormal
platelet & coagulation function, low Apgar scores, low birth weight,
prolonged labour, apnea, bradycardia, large PDA, pneumothorax, group B
streptococcal sepsis, hypoxia, hypercapnia, acidosis.
 Bed side Ultrasonography- better understanding of subependymal
hemorrhage & intraventricular hemorrhage.

23.  Term infants
 Intra cranial hemorrhage- birth trauma- forceps & breech deliveries.
 Tears of falx cerebri & tentorium cerebelli.
 C/F- apathy, seizures, high pitched cry, irregular respirations.
 Tense fontanel, moro reflex abnormalities.
 Focal ischemic lesions can occur- DIC, placental infarcts, embolism,
trauma to blood vessels.
 Ultrasonography & CT scans- diagnosis.

24.  Neuronal injury & death in hypoxic states- release of excitatory
neurotransmitters: L-glutamate & L-aspartate.
 Increased post synaptic stimulation of N-methyl-D-aspartate receptors-
entry of sodium & calcium into neurons & cell death.
 Delayed cell death- calcium influx- mitochondrial dysfunction with
breakdown of cell components & free radical formation.
 Major factor controlling cerebral metabolic rate-degree of neuronal activity.
 Seizures- cerebral metabolic rate increases & reduced to low levels in
coma.

25.  Cerebral blood flow maintained at high levels- substrate for brain
metabolic activity.
 < 3 yrs- 30-60 ml/100 mg brain/min.
 3-10 yrs- 105 ml/100 mg brain/min.
 Adults- 50 ml/100 mg brain/min.
 Mature brain- phenomenon of Auto regulation.
 Blood flow increases with increased neuronal activity.
 Perfusion pressure, intracranial pressure & vascular resistance.

26.  Increasing conc. of CO2- increase of blood flow- dilatation of intracranial
blood vessels.
 High conc. of O2- reducing blood flow- causing vasoconstriction.
 Most cerebral vascular accidents in children- impairment of arterial blood
flow- result of thrombosis or embolism.
 Localized region of metabolic acidosis- dilatation of surrounding blood
vessels- increased vascularity- luxury perfusion.
 Damage to neurons & glia- destruction of blood brain barrier- localized
cerebral edema- compress capillaries.

27.  Occlusion of venous structures- increased venous pressure- tendency for
blood vessel to rupture- bleeding & raised intra cranial pressure.
 Hemorrhage- intra parenchymal or extra cerebral- acts as mass lesion-
rise in intra cranial pressure.
 Damage to blood-brain barrier- promotes cerebral edema.
 Blood & blood products of erythrocytes- produce vascular spasm-add to
preexisting damage- cause meningeal irritation- can cause hydrocephalus.

28.  Cerebral infarction pathologic changes- neuronal death & perivascular
hemorrhage- influx of polymorpho-nuclear leukocytes- mononuclear cells
& macrophages.
 Astrocytosis results in formation of glial scar.
 Preterm infant with sub ependymal germinal matrix hemorrhage-
destruction of white matter- periventricular leukomalacia.
 Ventricular dilatation – destruction & disappearance of periventricular white
matter, post hemorrhagic hydrocephalus.

35. Etiology of Ischemic stroke
 Cardio embolism
 Congenital cardiac malformations
 Acquired
 Rheumatic heart disease/endocarditis
 Cardiomyopathies, arrhythmias
 Prosthetic , Prolapsed valves
 Cardiac interventions

36.  Vasculitis & Vasculopathies
 Infectious/ Immune/ Iatrogenic vasculitis
 Migraine
 Hypertensive encephalopathy
 Moya moya disease
 Hematologic & Hypercoagulable states
 Hemoglobinopathies- Sickle cell anemia
 Polycythemia
 Thrombocytosis

37.  Leukemia, Lympho-reticular malignancy
 Protein C, S deficiency
 Antithrombin III deficiency
 Nephrotic syndrome
 Metabolic & related disorders
 Homocystinuria
 Organic acidemia
 Leighs disease
 MELAS
 Neuroectodermatoses

38.  Trauma
 Blunt trauma- ICA.
 MELAS- Mitochondrial myopathy, encephalopathy, lactic acidosis, stroke
like episodes.

39. Etiology of Hemorrhagic stroke
 Vascular malformations- AVM/ Aneurysm
 Arterial Hypertension
 Iatrogenic
 Arteritis / Arteriopathies
 Cerebral venous occlusive disease
 Intracranial tumours
 Hematological- Leukemia, Thrombocytopenia,

40. Vascular disease in older children & infants
 Primary vascular diseases
 1. Acute infantile hemiplegia
 2. Moyamoya disease
 3. Takayasu arteritis
 4. Fibromuscular dysplasia
 5. Hemiplegic migraine

41. Acute infantile hemiplegia
 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.
 3 dimensional MRA- demonstrate significant vascular abnormalities in 75%
of children with strokes. (Wiznitzer & Masaryk, 1991)
 Infants- seizures, motor signs few, abnormal hand preference.
 Older children- sudden onset of hemiparesis, seizures.

42.  CT or MRI scan- mass lesion, intracranial hemorrhage or arteriovenous
malformation.
 Angiography or MRA- moyamoya disease & fibro-muscular dysplasia-
visualization of major blood vessels.
 Lumbar puncture- infectious etiological condn.

43. Moyamoya- stenosis- occlusion of intra cranial portion of ICA & vessels of Circle
of Willis.

44. Stages of Moyamoya disease

45. Moyamoya- opening of collaterals- smoke like appearance.

46. Cerebral angiography- multiple telangiectasia in basal ganglia- hazy, smoke like
appearance.

47. Contracted internal elastic lamina & focal intimal thickening of vessel wall

48. Moya moya pattern- opening up of collaterals

49.  Moyamoya disease
 Primary vascular disease- stenosis- occlusion of intracranial portion of
Internal carotid artery & other vessels of circle of Willis.
 Abnormalities of elastica & focal intimal thickening.
 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.
 Symptoms in childhood- females frequent.

50.  Chronic inflammatory, occlusive intracranial vasculopathy affecting ACA,
MCA, PCA associated with extensive network of collaterals.
 Multiple transient ischemic attacks with permanent residua.
 Sudden hemiparesis & multiple transient ishemic attacks without
neurological signs.
 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.

51.  Differentiation from Simple arterial occlusion- recurrent transient
ischemic attacks, progressive mental deterioration, widespread areas of
infarction.
 Cerebral angiography- definitive diagnosis.
 Progressive vascular changes- occlusion of supra clinoid portion of
internal carotid artery, middle & anterior cerebral arteries & finally
posterior communicating & posterior cerebral arteries.
 Cortical atrophy, multiple areas of lucency in cortex & white matter,
ventricular dilatation.
 Moyamoya pattern- follow radiation of optic gliomas.

52. Extra cranial to intra cranial arterial anastomosis

53. Superficial temporal artery to MCA shunt- enhance cerebral blood flow

54. Laying STA on arachnoid membrane- STA to MCA shunt

55.  Surgical placement of extracranial to intracranial arterial anastomoses.
 Superficial artery to MCA shunt & laying Superficial temporal artery on
arachnoid membrane- enhance cerebral blood flow.
 No firm conclusions regarding usefulness of procedure.

56. Takayasu arteritis
 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- infiltration of T cells, NK cells, plasma cells,
macrophages- giant cells & granulomatous inflammation in media- blood
vessel dilatation & aneurysm formation.
 Hypertension, absent pulses, vascular bruits.
 Strokes in 5-10 % of patients.
 Corticosteroids & immunosuppresants.

57.  C/F- Pre pulseless phase of disease- fever, malaise, headache,
hypertension, abdominal pain, arthralgia.
 Later- diminished pulses, asymmetric blood pressures, claudication,
Raynauds phenomenon, renal failure, symptoms of pulmonary & cardiac
ischemia.
 Classification criteria for TA
 Angiographic abnormalities
 Decreased peripheral artery pulse or claudication
 BP difference b/w arm & legs >10 mmHg
 Bruits over aorta
 Hypertension

58. Takayasu arteritis- patterns of arterial involvement

59. Fibro muscular dysplasia
 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.

61. Migraine
 Hemiplegic migraine- transient hemiparesis with severe headache.
 Familial forms & sporadic forms.
 Transient loss of vision in one eye- amaurosis fugax- adolescent
migraineurs.

62. Vertebro basilar occlusion
 Decreased blood supply to posterior circulation of brain.
 Vertebral artery
 Basilar artery
 Posterior cerebral artery
 Blood supply: brain stem, thalamus, inferior portion of temporal lobe &
occipital lobe.

64. Vertebro basilar occlusion
 Sudden onset or stuttering progression
 Brain stem localization- corticospinal & cerebellar signs with oculomotor
abnormalities.
 Locked in syndrome- infarction at the level of Basilar artery. (Golden etal
1987)
 Child alert, quadriplegia, facial diplegia, absent horizontal eye movements.
 Child cannot speak, preserved vertical eye movements.
 Diagnosis considered in a child in coma after a vascular accident but has
spontaneous eye opening.

65.  Subclavian steal syndrome
 Retrograde flow of blood in vertebral artery, due to proximal stenosis of
subclavian artery.
 Follows correction of coarctation of aorta
 Headache, dizziness, visual field defects after exercise, seizures.
 Angiographic findings characteristic.
 Treatment- ligating left vertebral artery or placing a subclavian artery graft.

67. Cerebrovascular disease secondary to medical conditions
 Cardiac disease
 Clinical scenario
 9/12 old infant k/c/o TOF came with h/o Left sided hemiparesis, seizures,
depressed state of consciousness.
 MRI brain showed Right MCA infarct with cerebral edema.

68.  CVA- complication of cyanotic congenital heart disease.
 TOF, TGA- common.
 Mech : Arterial thrombosis, venous sinus thrombosis, or embolism.
 “Any child < 2 yrs with CHD , who has acute onset of neurological
signs- CVA should be considered as primary diagnosis ‘’
 After 2 yrs , Brain abscess most common.
 Embolic strokes- children with cyanotic congenital heart disease- R-L
shunt bypasses lungs, which normally filter small emboli.


69.  Bacterial endocarditis- congenital heart disease & Rheumatic heart
disease- potential sources of emboli.
 Thrombi can form on prosthetic cardiac valves- imp. cause of cerebral
emboli.

70. Hematologic & Neoplastic diseases
 Sickle cell anemia (SCA)
 most common Hemoglobinopathy assoc. with CVA.
 Stroke in children <19 yrs of age with SCA- 8 %.
 Incidence- 700 per 1 lakh children with SCA.
 Stroke in SCA- large vessel disease, venous occlusion, subarachnoid or
intracerebral hemorrhage.
 Neuroimaging- occlusion of large cerebral vessels or watershed infarction
secondary to disease of large vessels.
 Fewer shows isolated subcortical or small cortical branch occlusion.

71.  Neuro pathological examn. confirm infarctions in area supplied by anterior-
middle cerebral artery & thrombi in distal cervical & proximal intracranial
carotid arteries.
 Strokes in children with SCA- highest incidence in 5-10 yrs.
 Hemiparesis- most common symptom.
 Aphasia- 20 %, Seizures- 15 %, TIA- 10 %
 Persistent neurologic deficits & neuro psychologic abnorm.
 Children with HBSS or SB thalassemia- highest incidence of stroke-
monitor with trans cranial USG.

72.  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 %.
 Intracranial hemorrhage- serious complication of any bleeding disorder.
 Intracerebral, subarachnoid, subdural, intra spinal hemorrhage.
 Symptoms- headache, seizures, depressed state of consciousness.
 Intra spinal hemorrhages- weakness, back pain.

73. Hemophilia
 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
after h/o head trauma.

74.  Homozygous deficiency of Protein C in newborns- purpura fulminans &
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 & 6/12- Vitamin K deficiency.
 Hemolytic uremic syndrome- seizures, depressed consciousness,
subarachnoid hemorrhage, hemiparesis, thrombotic strokes.

75. Leukemia
 Intracranial hemorrhage- 20%.
 Intracranial bleeding occurs in acute stage of leukemia with extremely
elevated high leukocyte count.
 Intracerebral or extra cerebral hemorrhage- results from increased blood
viscosity.
 Multiple small thrombi, damage to blood vessels.
 Children with high leukocyte count- develop dural sinus occlusion with
increased intracranial pressure & headache.
 CVA also occur after bone marrow transplantation.

76. Infectious diseases
 Acute bacterial meningitis- treatment delayed- infectious arteritis-
multiple areas of arterial narrowing & occlusion.
 Occlusion of veins or dural sinuses- complication of meningitis & follow
otitis media, mastoiditis, sinusitis & infection of scalp & face.
 Clinical picture- convulsions, coma, changing neurologic signs, nuchal
rigidity, signs of infection.
 Otitis media & mastoiditis- cause lateral sinus thrombosis associated
with abducens palsy & increased intracranial pressure.

77.  Facial skin & para nasal sinus infections- produce cavernous sinus
thrombosis with proptosis, conjunctival reddening, retinal hemorrhages &
extra ocular palsies.
 Retropharyngeal abscess- produces compression or thrombosis of
carotid artery.
 Post varicella angiopathy, mycoplasma pneumonia, borrelia-burgdorferi,
chlamydia pneumonia, HIV, helicobactor pylori, hemolysing streptococci-
predisposition for stroke.

78.  Cranial infections
 Stroke common sequel of Severe meningitis- H. Influenza, Pneumococcal,
Tuberculous meningitis.
 Purulent material around basal cisterns & orbito frontal area, circle of
Willis- envelops small arteries & veins- vasculitis & thrombus.

79. Collagen vascular disease & Vasculitis
 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.

80. Metabolic disorders
 Infants with fever & dehydration- primary venous or sinus thrombosis.
 C/F- multiple seizures, changing neurological signs, convulsions, coma,
increased intracranial pressure.
 Hypernatremic dehydration- seizures, depressed state of
consciousness.
 Pathology- multiple hemorrhagic lesion in white matter.
 Juvenile onset Insulin dependent Diabetes mellitus- acute
hemiparesis.

81.  MELAS SYNDROME- epilepsia partialis continua or status epilepticus,
repeated strokes.
 MRI- multiple areas of hyper intense signal in cortex & subcortical white
matter, sparing deep white matter.

82. Trauma & physical agents
 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.
 Children- falling on stick held in mouth, lolli pop injury .

83.  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.
 Bone abnormalities of upper cervical spine & odontoid, 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.
 Radiation & chemotherapy.

84. Vascular malformations
 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.

85.  Intracerebral hematoma- focal neurological signs & increased intracranial
pressure.
 Subarachnoid hemorrhage- sudden onset of headache, meningeal signs.
 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” (Cohen and Levin 1978)

86.  CT with contrast, MRI, Arteriography.
 RX- surgical accessibility of lesion.
 Total surgical excision is curative, but best approach to treatment ??
 Embolization of lesion may be effective.
 Stereotactic radiosurgery with linear accelerator – effective modality for
some patients.

87. Vein of Galen malformations
 Arteriovenous malformation of Vein 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.

88.  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.

89.  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 effective in some.

90. Aneurysms
 Uncommon in children less than 10 yrs.
 Located in either anterior or posterior circulation.
 Sudden onset of massive subarachnoid hemorrhage & depressed state of
consciousness.
 CN II, III or both.
 Commonly occur on anterior cerebral artery or internal carotid artery ,
distal portions of cerebral vasculature.
 Usually are >1cm – intracranial hemorrhage, seizures.

91. Ruptured Berry Aneurysm- saccular aneurysm, common form of
cerebral aneurysm.

92.  Surgery, microsurgical techniques- definitive RX for aneurysms.
 Aneurysm not removed- 50 % will bleed- serious neurological deficits.
 Mycotic aneurysms- bleeding.

93. Neuro cutaneous syndromes
 Sturge weber syndrome
 Port wine stain on face & scalp , capillary venous angioma of meninges,
vascular abnormality within cortex & white matter of ipsilateral hemisphere.
 Highest risk for brain involve.- bilateral port-wine stain, unilateral with
involv. of all three divisions of trigeminal nerve, involves eyelid.
 Brain- gliosis, calcification & neuronal loss.
 Seizures, hemiparesis, mental retardation.
 Eye- glaucoma, angioma of retina & choroid.

94.  Progressive abnormalities with areas of calcification, intractable seizures,
intellectual & behavioural deterioration.
 Abnormalities of regional cerebral blood flow & progressive cerebral
atrophy.
 Early excision of abnormal areas of cortex.
 Affected area is large- hemispherectomy.
 Lasers- reduce or eliminate port-wine stain.

95.  Dural venous sinus thrombosis
 Venous sinuses- major pathway for drainage of intracranial circulation.
 Significant proportion of CSF drains into sagittal sinus through pacchionian
granulations.
 Thrombosis of major venous sinuses causes increased intracranial
pressure by impeding venous outflow & interfering with resorption of CSF.

96.  Sagital sinus thrombosis
 Sagital sinus drains vast majority of cortical veins over brain convexity.
 Partial or complete.
 Occlusion of sagittal sinus causes stasis & thrombosis in connecting
cortical veins- assoc. hemorrhage over brain surface.
 Common in children under 3 yrs, often during first year of life.
 Thrombosis extends into cortical veins- rapidly increasing intracranial
pressure, changes in level of consciousness, seizures, focal motor
impairment.

97.  Septic venous sinus thrombosis- most common in neonates.
 Spinal fluid resorption impaired-compromise of spinal flow between
arachnoidal granulations & blood in sinus-communicating hydrocephalus.
 Venous stasis promotes vascular congestion of brain parenchyma-
increase in intracranial pressure.
 Sagital sinus thrombosis-distension of veins over scalp & superior
forehead.

98.  Occlusion of sagittal sinus in older children- syndrome of pseudo tumor
cerebri , headache, CN 6 palsy- false localizing sign, papilledema, visual
loss.
 Good prognosis.
 Predisposing factors leading to sagittal sinus thrombosis- dehydration,
malnutrition, debilitating d/s, febrile illness, congenital heart disease,
hypercoagulable states.
 Spinal fluid studies-normal in early course, later xanthochromic or flankly
blood with increased protein concn.

99.  CT scan- increased density within sinuses-diagnosis of venous sinus
thrombosis. Superficial hemorrhage & cerebral edema.
 Delta sign- CT scan with contrast shows enhancement around thrombotic
sinus.
 MRA- flow void- thrombosis in the area of sinus.
 Conventional angiogram- venous phase of study well documented.
 No role for anticoagulant therapy.
 Interventional thrombolytic therapy- useful in some.
 RX for raised intracranial tension.

100.  Lateral sinus thrombosis
 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, surgical intervention?

101.  Cavernous sinus thrombosis
 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.

102.  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.

103. Spinal cord vascular abnormalities
 Thrombotic & embolic d/s of spinal cord- rare.
 Arteriovenous malformation- back pain, gait abnormalities, bladder &
bowel dysfunction.
 Neurological examn.- long tract signs, asymmetric tendon reflexes.
 Angioma of skin of back- 20 % accentuated by Valsalva maneuver.
 Bruits over spine- rare.
 Subarachnoid hemorrhage, no localizing neurological abnorm.
 Spinal cord arteriovenous malformation- multiple episodes of subarachnoid
hemorrhage?

104.  High resolution CT or MRI scans- diagnosis.
 Selective spinal angiography- outline feeding vessels & extend of
malformation.
 Surgical excision possible in many.

105. History, Physical examination, Clinical presentation

106. Clinical presentation
 Older children : Hemiplegia, Hemi sensory loss, Aphasia &
other neurological deficits.
 Younger children : subtle, variable findings, seizure,
early hand preference, limp during walking.

107. Conditions which mimic stroke
 Todds paralysis ( Transient post ictal hemiparesis )
 Hemiplegic migraine
 Syndrome of alternating hemiplegia
 ICSOL ( Intracranial space occupying lesion )
 Acute disseminated encephalomyelitis

108. History & Physical examination
 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.


109. Localization of Hemiplegia
 1. Hemispheric lesion
 a. Cortex - cortical features like seizures, dysplasia.
motor deficits are minimum.
Cortical type of sensory loss - Parietal lobe function-
( loss of tactile localization, 2 point discrimination,
stereognosis, graphaesthesia, sensory inattention ).
 b. Corona radiata - absence of cortical features
motor deficits- unequal weakness of limbs on
C/L side. Either UL>LL or LL>UL.
Dulling of Primary modalities of sensation- touch,
superficial pain, temperature.

110.  c. Internal capsule : C/L hemiplegia, uniform weakness of limbs on
C/L side, dulling of primary modalities of
sensation-touch, superficial pain, temperature.
Homonymous hemianopia
2. Brain stem : Crossed hemiplegia
I/L LMN cranial nerve palsy
C/L hemiplegia

111.  Brain stem
 a. Midbrain : Weber syndrome : I/L 3rd CN & 4th CN palsy,
C/L hemiplegia.
 b. Pons : Millard Gubler syndrome : I/L 6th CN & 7th CN palsy,
C/L hemiplegia.
 c. Medulla : C/L hemiplegia , I/L CN involv.

112. Diagnostic Approach
 Confirmation of presence of Cerebrovascular lesion.
 Exclude other causes of neurological dysfunction.
 Etiology of stroke

113. Evaluation of child with Stroke
 Standard Evaluation
 CT scan (plain)
 If CT scan is normal , MRI scan (plain)

114. Ischemic Stroke-Investg.
 ECG, CXR, Echocardiography
 Carotid Doppler studies
 Hematological studies
 CBC, Platelet count, ESR.
 Liver & Renal function test.
 Lumbar puncture.

115.  Imaging studies
 Magnetic resonance angiography (MRA).
 Digital subtraction angiography (DSA).
 Collagen vascular screen
 PT, APTT.
 Protein C, Protein S, Antithrombin 111.
 Others- VDRL, HIV, Urinalysis, Lactate/Pyruvate levels, Homocysteine
 Urinary & blood aminoacids/ organic acids

116. Hemorrhagic stroke
 CBC, Platelet count, clotting factors.
 LFT.
 Imaging studies.

117. Treatment
 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.

118.  Aggressive antiepileptic treatment.
 Antithrombotic therapies
 Use of anticoagulant therapy increasing in pediatric AIS.
 Avoid anticoagulation in hemorrhage, hypertension, or bleeding diathesis.
 Heparin
 Use anticoagulation in children at high risk of recurrence/extension of
thromboembolic stroke & who are at minimum risk of secondary
hemorrhage.

119.  Loading dose of Heparin 75-100 U/kg iv over 10 min followed by initial
maintenance dose of 28 U/kg/hr in children>1yr & 18 U/kg/hr in
older children.
 Adjust Heparin dose to maintain APTT in range of 60-85 sec.
 Low molecular weight Heparin
 Greater safety & efficacy.
 Monitoring done-Antifactor Xa assay once weekly or monthly.
 SVT in infants- LMW Heparin for 7-14 days followed by Coumadin for 3/12.

120.  Antiplatelet agents
 Traditional role of aspirin in prevention of recurrence after TIA or ischemic
stroke.
 Adults-Aspirin reduces stroke by 25 %.
 Clopidogrel-no control trials, but may be good choice.
 Dose-3-5 mg/kg/day.

121.  Oral anticoagulation
 Warfarin used for secondary prevention of stroke if aspirin fails.
 Congenital or acquired heart disease
 Severe hypercoagulable states
 Arterial dissection
 Recurrent AIS or TIA while on aspirin.


122.  Thrombolytic agents
 Tissue plasminogen activator
 Urokinase
 Streptokinase
 Dissolve the existing thrombus & recanalise the occluded vessel.
 Non thrombotic therapies
 Transfusion therapy
 Neuroprotective agents
 Immunosuppressants

123.  Surgical evacuation of hematomas, insertion of ventricular or
lumboperitoneal shunts & rarely revascularization procedures.
 Revascularization procedures like Encephalo duroarterio synangiosis
(EDAS) or Pirl Synangiosis- important in treatment of moya moya
disease.
 Rehabilitation therapy
 Speech therapy
 Occupational therapy
 Physical & psychological therapy

124. Summary
 Stroke in children relatively rare.
 There are fundamental, etiologic & developmental differences in children
compared with adults .
 Multiple causes for stroke in children & many risk factors.
 Ischemic stroke-interruption of blood flow of arterial or thrombotic d/s.
 Hemorrhagic stroke-rupture of blood vessels with bleeding into cerebral
parenchyma.
 Hemorrhagic stroke higher mortality than ischemic stroke.

125. Bibliography
 1. Adam K, Gabrielle D. Pediatric stroke syndromes. Nelson textbook of
Pediatrics.19th edition;2080-2086.
 2. Gerald SG, Keeneth FS. Pediatric Neurology. Edition; 787-803.
 3. Veena K. Stroke in the children. 2nd edition;239-246.
 4. Gerald MF. Clinical Pediatric Neurology.6th edition; 249-265.
 5. Vishram S. Textbook of Clinical Neuro anatomy.3rd edition; 230-249.
 6. Amlie LC, Sebire G, Fullerton HJ. Recent developments in childhood
arterial ischemic stroke. Lancet Neurol 2010;7:425-435.
 7. Benseler SM, Silverman E, Aviv RI etal. Primary central nervous system
vasculitis in children. Arthritis Rheum 2011; 54:1291-1297.

126.  8. Birnbaum J, Hellmann DB. Primary angitis of the central nervous
system. Arch Neurol.2008;66:704-709.
 9. Danchaivijitr CT, Saunders DE etal. Evolution of cerebral arteriopathies
in childhood arterial ischemic stroke.2006;59:620-626.
 10. Chollet F, Tardy J etal. Fluoxitine for motor recovery after acute
ischemic stroke (FLAME): a randomized placebo controlled trial. Lancet
Neurol.2011;10:123-130.