targets: medical students and postgraduate

1. Stroke in Children:
Clinical Guidelines
Prof Dr Hussein Abdeldayem
Prof of Ped Neurology, Alex Univ

2. CASE STUDY
• 4 YS DS with TF
• Sudden onset of
hemiplegia

3. WHO DEFINITION OF STROCK 2004
• “A clinical syndrome in which there
is rapidly developing signs of focal
or global disturbance of cerebral
functions, lasting more than 24
hours or leading to death, with no
apparent causes other than of
vascular origin”

4. Definition
• Transient ischaemic attacks (TIAs): ‘where the
neurological deficit resolves within 1 hr
(previously 24 hours).

5. Differs from Adult Stroke
• In adult: chronic risk factors
• In children:
1- congenital and developmental risk factors
2- rare
3-subtle presentation*
4- wide DD
5- no established tt
*Among neonates, signs of hemiparesis are generally
not seen before the first six months to one year of life

6. incidence
-2.5 – 3.0 children out of 100,000 affected every year.
-Newborn babies can develop a stroke 28/100,000
- More in boys
- Nearly half are hgic stroke
One of the top ten causesof childhood
deaths world wide

7. types
• A hemorrhagic stroke: a ruptured blood vessel
bleeding into the brain.
with: headache, disturb consc, vomiting
• An ischaemic stroke: +++
51% isch, 49% hgic
80% isch and 20% hgic (adult)

8. Hgic Stroke

9. Types (cont. )
• An ischaemic stroke : an embolism or
thrombus blocks a blood vessel in the brain
++
EMBOLI: sec
thrombus: minutes
warning: TIA
Arterial ischaemic stroke: ‘a clinical
stroke syndrome due to cerebral infarction
in an arterial distribution’.

10. Ischemic Stroke

11. types of stroke
ischemic hemorrhagic
thrombosis embolus
Stenosis
Spasm intraparenchymalsubarachnoid
arterial venous
I Ventricular

12. Fate
• About 10% of childhood strokes are fatal.

13. Fate
• Recurrence ( 20-30%)
• Motor impairment
• Seizures (10-20%)
• Language, Communication
• Cognition
• Headache (30%)
• Social interaction
• Learning

14. Motor impairment
• Hemiparesis/hemiplegia
• Spasticity (?CP)
• dystonia
• a mixed pattern

15. Language and communication
defects
• language input :(receptive aphasias)
• language processing (word-finding problems,
grammatical problems and other aphasias),
• speech production ( dysarthria )
• written language (dyslexia).
• Mutism: problems of social interaction and the
willingness to communicate

16. Blood supply of brain

18. pathophysiology
• Blood supply of the brain

19. • In children ischemic stroke
has been most commonly
reported in the distribution
of the middle cerebral artery.

20. Types of stroke (2)
According to time of onset :
1- prenatal
2- perinatal
2- pediatric

21. Prenatal and Perinatal stroke
• 17 times more common than pediatric stroke beyond
the newborn period.
• Arterial ischemia occurring during the 3 days
surround birth is reported to be responsible for 50%
to 70% of congenital hemiplegic cerebral palsy.
• Germinal matrix hge : prone with increase BP

22. Pathophysiology
• Features of perinatal period that influence
coagulation state
– Presence of fetal hemoglobins
– Fetal proteins
– High hematocrit and blood viscosity
– Concentrations of procoagulant and anticoagulant
proteins change with gestational and postnatal
age

23. Risk Factors for Perinatal Arterial Stroke
• Infants : Antiprothrombin factors
• in the mothers, antiphospholipid antibodies :
anticardiolipin (aCL) antibodies and lupus
anticoagulant (LA).,

24. Prenatal Stroke
• Focal cerebral infarction (stroke) secondary to
intrauterine or perinatal thromboembolism
related to thrombophilic disorders, especially
anticardiolipin antibodies, is an important
cause of hemiplegic CP
Nelson Textbook of Pediatrics 18th ed

25. Prenatal Stroke
• Family histories suggestive of thrombosis and
inherited clotting disorders may be present
and evaluation of the mother may provide
information valuable for future pregnancies
and other family members.
Nelson Textbook of Pediatrics 18th ed

26. Perinatal stroke and the risk of developing
childhood epilepsy.
• Childhood epilepsy is frequent after perinatal
stroke. Evidence of infarction on prenatal
ultrasonography and a family history of
epilepsy predict earlier onset of active
seizures
J Pediatr. 2007 Oct

27. Stroke Awareness
• Survival from risky disease : PMT, LK, Cyanotic
CHD
• Improving of radiography

28. CP
Neonates - <6 months
• Seizures
• Irritability, drowsiness, bulge AF, jitterness,
apnea
• Focal motor defect ( hemiparesis): >6 mo age

29. CP
> 6 months
• As adult: focal motor, ataxia, aphasia
sensation troubles
• ICHge: severe headache
• SAH: meningism
• Developmental delay (17%- SCD)

30. Potential signs
• Motor Disorder
• Sensory
• Reflexes
- focal weakness
- initial hypotonia then spasticity
- Abnormal plantar reflex ( Babinski’s)

32. Aetiology
Cong/Acq/ Idiopathic
CONGENITAL: Cardiac, SCD, Coag abn
ACQUIRED:
• 1- TRAUMA/ drug induced
• 2-VASCULAR
• 3-INFLAMMATORY/autoimmune
• 4-NEOPLASM
• 5-systemic: METABOLIC, DEHYDRATION &
DEGENERATIVE

33. Coagulopathies and
Hematologic Disorders
RBC: SCD, polycythemia
Platelets: ITP-Thrombocytosis,
Protein C deficiency
Protein S deficiency
Factor V (Laiden) mutation
Anticardiolipin antibodies
Lupus anticoagulant antithrombin III
deficiency
Cong coagulation defects 8-9-VWF
DIC
Liver dysfunction with coagulation defects,
Vit K defeciency
Malgnancy: Leukemia
Metabolic/syndrome
Homocystinuria
Mitochondrial disorders
Sulfite oxidase
deficiency
Fabry’s syndrome
DS
Ehler’s – Danlos
-Moyamoya syndrome

34. Hematologic causes
• Sickle cell disease:
Overt or silent strokes
1-Occlusion of small vessels by deformed sickled
cells
2-Membranes of SRC are abnormal ,so have a
pro coagulant activity
3-VWF enhances adherence of sickle red cells to
endothelium in cases of high rates of flow
(linear sheer stress)

35. Consequences of Arterial Occlusive Stroke in Sickle
Cell Disease
Age: 5 y (1-18y)
1-disturbed learning and intellectual problems
2 -Neovascularization occurs in the areas that
are left underperfused by the stroke. The
network of small, delicate vessels that appear
as cloud-like puffs on an arteriogram are
called "moyamoya

36. Moyamoya disease
Basal arterial occlusion with telangectasia
Chronic , progressive, non inflammatory vasculopathy
Bilateral slowly progressive occlusion of ICA
As occlusion is slowly progressive multiple anastomosis
between external & internal carotid
New vascular net work at the base of the brain
composed of collaterals(basillar meningeal& choroidal
arteries)

37. • It is not a disease but secondary to other
disorders
Sickle cell anaemia
VSD
Down syndrome
TOF
NFI

38. MR angiography :telangectasis produces hazy
appearance like a puff of smoke ( japanese word
:moyamoya)

39. Moyamoya disease in a 9-year-old boy.
©1999 by Radiological Society of North America

40. Disorders of coagulation:
1- anticoagulant deficiency
Protein c
Protein s
AT III
2- Factor v leiden
3- prothrombin mutation

41. Protein C, Protein S, AntithrombinIII
deficiency
• Protein C and protein S are inhibitors of factor
V(Leiden) and antithrombin III opposes the
normal procoagulant activity of factors II, IX,
X, XI, and XII by promoting the irreversible
formation of inactive complexes of these
factors.
• inherited or acquired

44. Congenital Heart
Disease
Acquired Heart Disease
VSD,
ASD,
PDA
Aortic stenosis
Mitral stenosis
Coarctation
Cardiac rhabdomyoma
Complex congenital
heart defects as FT
Rheumatic heart disease
Prosthetic heart valve
Libman-Sacks endocarditis
Bacterial endocarditis
Cardiomyopathy
Myocarditis
Atrial myxoma
Arrhythmia

45. Cardiac causes
Cyanotic heart disease (<2 years)
1. Venus sinus thrombosis: polycythemia,
dehydration ,viral illness and iron deficiency
anaemia
2. Embolic arterial occlusion in children due to
vegetations or RT to LT shunt
Cardiomyopathy , prothetic valve
,endocarditis, cardiac cath and surgical
repair

48. Vasculitis
Meningitis/encephalitis
Systemic infection,
SLE
Polyarteritis nodosa
Granulomatous angiitis
Takayasu's arteritis
Rheumatoid arthritis
Dermatomyositis
IBD, HUS
Drug abuse (cocaine,
amphetamines),
Drug induced
Systemic Vascular
Disease
Systemic hypertension
Volume depletion or
systemic hypotension
Hypernatremia
Superior vena cava
syndrome
Diabetes

49. VasoSpastic
Disorders
Structural Anomalies of the
Cerebrovascular System
Migraine
Ergot poisoning
Vasospasm with
subarachnoid
hemorrhage
Arterial fibromuscular dysplasia
Agenesis or hypoplasia of the
internal carotid or
vertebral arteries
Arteriovenous malformation AVM
Hereditary hemorrhagic
telangiectasia
Sturge-Weber syndrome
Intracranial aneurysm

50. Trauma
Child abuse
Fat or air embolism
Foreign body embolism
Carotid ligation (e.g.,
ECMO)
Vertebral occlusion
following abrupt cervical
rotation
Blunt cervical arterial
Trauma: Neck trauma,
intraoral trauma
Posttraumatic arterial
dissection
Arteriography
Posttraumatic carotid
cavernous fistula
Coagulation defect with
minor trauma
Amniotic fluid/placental
embolism
Penetrating intracranial
trauma

51. Drugs:
Amphetamines
Cocaine
Sympathomimetics
L asparginase

52. Inflammatory
• Meningitis
• Encephalitis
(due to vasculitis, venous thrombosis or
parenchymal necrosis)
( bacterial & varicella)
• Local head and neck infection
Sebrie et al 1999

53. Metabolic
MELAS
• 1. Mitochondrial emcephalopathy, lactic acidosis, and
stroke
• 2. Point mutation of mitochondrial DNA
• 3. Normal developmental milestones in first few years
of life.
• 4. Episodic vomiting, lactic acidosis, and proximal
muscle weakness
• 5. Stroke syndromes - migranous HA, seizures, or
hemiplegia
• 6. Encephalopathy/dementia are progressive.
• 7. Hearing loss, visual defects, and
short stature are typical
Systemic Disorder
Metabolic

54. • Mitochondrial disorder
(MELAS):
Mitochondrial accumulations and abnormalities
in sm of intramuscular vessels , brain
arterioles and in blood vessels of choroid
plexus
(mitochondrial angiopathy)

55. • Fabry’s disease
X-linked, defniciency of alpha-galactosidase
angiokeratomas, corneal and lenticular opacities, and
painful acroparesthesias ,hypohydrosis,
Vascular complications:(renal failure, cerebral thrombosis.?

56. Homocystinuria:
Thromboembolic episodes involving both large
and small vessels specially brain
Damage to the vascular endothelium and
increased platelet adhessivness 2ry to
elevated homocystine level

57. Metabolic Hyperhomocysteinemia
• HC is a highly reactive amino acid toxic to
vascular endothelium
– Pro-atherogenic and pro-thrombotic effect on
blood vessels
• HC can potentiate the auto-oxidation of
LDL
• HC is emerging as a potentially modifiable
risk factor for atherosclerosis

58. prothrombin gene G20210A mutation.
• Prothrombin G20210A mutation is an
important prothrombotic condition for venous
thrombosis. Recently, some studies have also
considered it to be a risk factor for arterial
ischemic stroke in children.
J Child Neurol.2007 Mar;22(3):329-31

59. Clinical presentation
• F:M = 3:2
• Girl + headache+ bilateral UMNL signs
1. Sudden hemiplegia of face and limbs associated
with hemianopia ,hemianaesthesia and aphasia
recovery may occur followed by new episodes of
focal neurological findings on same or other side
2-recurrent TIA with episodic hemiparesis for min or
hrs with no loss of consciousness

60. Scope of the guidelines
• the diagnosis, investigation and management
of acute arterial ischaemic stroke in children
beyond the neonatal period

61. Stroke Guidelines
1st
• Families/carers should be given actual
information about their child’s condition as
soon as possible after diagnosis . This should
be simple and consistent, avoiding technical
terms and jargon
• Children should be given information about
their condition at an appropriate level .

62. Stroke Guidelines
2nd
Presentation and diagnosis
• All children with acute stroke should be
referred to, or have their management
discussed with, a consultant paediatric
neurologist

63. Stroke Guidelines
3rd
Brain Imaging
• Cross-sectional brain imaging is mandatory in
children presenting with clinical stroke
• Brain magnetic resonance imaging (MRI) is
recommended for the investigation of children
presenting with clinical stroke

64. Stroke Guidelines
4th
• If brain MRI will not be available within 48 hours,
computed tomography (CT) is an acceptable initial
alternative

65. Stroke Guidelines
5th
Further Non-invasive cerebrovascular imaging :
- M R Angiography
- CT angiography
- ultrasound with Doppler techniques
- combination
MRV (magnetic resonance venography)

66. Stroke Guidelines
6th
• All children with clinical stroke should have
regular assessment of conscious level and vital
signs
• Brain imaging should be undertaken urgently
in children with clinical stroke who have a
depressed level of consciousness at
presentation or whose clinical status is
deteriorating

71. History
Subsequent
Lab

72. • CBC (WBC, RBC*, Platelets)
• Electrolytes
• PT/PTT
• Liver functions
• Lipid profiles
• ESR
• Echocardiograph: Transthoracic cardiac
echocardiography
• * CSF examination
Initial
Lab
*Hemoglobin electrophoresis

73. Stroke Guidelines
8th
Echocardigram:
- for congenital and acquired cardiac disease
- Transthoracic cardiac echocardiography should
be undertaken within 48 hours after
presentation in all children with arterial
ischaemic stroke

74. A lumbar puncture
• LP will screen for metabolic derangements
and CNS inflammatory and infectious
disorders.

75. • Prothormbic tendency factors
• Other relevant tests
Subsequent
Lab

76. prothrombotic Factors
• protein C deficiency
• protein S deficiency
• Antithrombin III
• Factor V Leiden
• antiphospholipid antibodies:anticardiolipin (aCL) antibodies
and lupus anticoagulant (LA).,
• activated protein C resistance
• lipoprotein-associated phospholipase A2 (Lp-PLA2)*
• high-sensitivity C-reactive protein (hs-CRP) *
• increased plasma homocysteine ( & urine)
• prothrombin G20210A and MTHFR TT677 mutations and A
1298C
*FDA approved for stroke recurrence

77. • homocysteine levels, levels of protein C,S,and
antithromin III, Leiden factor V, RPR, C reactive
protein/ESR,lipid panel, troponin, creatinine
kinase, electrolytes, glucose, HbA1C, diffusion
weighted and perfusion weighted MR,
transesophageal echocariogram, EKG with
rhythm strip, CBC with platelets, chest X ray?

78. Other relevant tests
• ANA
• HIV
• Lactic acid, pyruvic acid ( blood , CSF)

79. Acute care
• General measures
• Specific measures
• Prevention/prophylaxis
Recovery after stroke depends on the severity
of the stroke and the speed of treatment

80. Acute care
general measures
Stroke Guidelines
9th
• Hospital
• ABCD care
• Temperature should be maintained within
normal limits
• Oxygen saturation should be maintained
within normal limits

81. Acute care
general measures Stroke Guidelines
10th
• i - swallowing safety
• ii-feeding and nutrition
• iii-communication
• Iv- pain
• v-moving and handling requirements
• vi- positioning requirements
• vii- risk of pressure ulcers

82. Acute care
general measures
Pain:
• Children affected by stroke should be assessed
for the presence of pain using non verbal
technique
• All pain should be treated actively, using
appropriate measures including positioning,
handling, and medication
Reduce intracranial tension : mannitol IV

83. Specific treatment

84. Acute care
specific medical treatments (cont.)
If diagnosed within 3 hrs:
• Thrombolytics: streptokinase, tissue
plasminogen activator (tPA)

85. Acute care
specific medical treatments
• Aspirin (5 mg/kg/day)* should be given once
there is radiological confirmation of arterial
ischaemic stroke, except in patients with
evidence of intracranial haemorrhage on
imaging and those with sickle cell disease
- LMWH (1 week)
- Warfarin ( for 6 months)
*max 300 mg

86. SCD stroke
1- exchange transfusion
2- prevention: repeat transfusion 4-6 weeks to keep
hemoglobin S <20- 30%

87. • Neonate + noncardioembolic AIS do not use
anticoagulant or aspirin
• Neonate+cardioembolic AIS use anticoagulant
therapy (UFH or LMWH ) for 3 months
• Child+AIS treat with anticoagulant for 5-7 days or
until cardio-embolic or vascular dissection has been
excluded.
Antithrombotic therapy in children: the Seventh ACCP Conference on Antithrombotic Chest 2004 Sep;126 (3 Suppl):645S-687S.

88. Acute care
specific medical treatments (cont.)
• Treatment of underlying cause

89. A Cardiac Source of Embolism and AIS
• The decision to use anticoagulation should be
discussed with a consultant paediatric
cardiologist and paediatric neurologist

90. 3- prevention
Stroke recurrence
• Arterial ischaemic stroke recurs in between 6%
and 20% of all children.
• The risk of recurrence* is increased in children
with multiple risk factors and in those with
protein C deficiency, increased levels of
lipoprotein (a) and vascular disease
*(Lanthier et al2000)

91. Stroke recurrence Stroke Guidelines
11th
1- Aspirin
• Patients with cerebral arteriopathy other than
arterial dissection or moyamoya syndrome or
those with sickle cell disease should be
treated with aspirin (1–3 mg/kg/day)

92. Stroke recurrence prevention
2- Anticoagulation
• should be considered:
1- until there is evidence of vessel healing, or for a
maximum of six months, in patients with arterial
dissection
2- if there is recurrence of arterial ischaemic stroke despite
treatment with aspirin
3- in children with cardiac sources of embolism, following
discussion with the cardiologist managing the patient
4- until there is evidence of recanalisation or for a
maximum of six months after cerebral venous sinus
thrombosis

93. Stroke recurrence In children with
sickle cell disease:
• should be considered:
1- regular blood transfusion (every three to six weeks)
should be undertaken to maintain the HbS% <30% and
the Hb between 10–12.5 g/dl (C)
2- transfusion may be stopped after two years in patients
who experienced stroke in the context of a precipitating
illness (eg aplastic crisis) and whose repeat vascular
imaging is normal at this time (C)
3- after three years a less intensive regime maintaining
HbS <50% may be sufficient for stroke prevention(C)

94. Stroke recurrence Stroke Guidelines
12th
moyamoya syndrome
• Children should be referred for evaluation to a
centre with expertise in evaluating patients for
surgical re-vascularisation

95. Stroke recurrence
• Advice should be offered regarding
preventable risk factors for arterial disease in
adult life, particularly smoking, exercise and
diet
• Blood pressure should be measured annually
to screen for hypertension

97. DD of stroke like events
1- Alternating hemiplegia:
2-18 month, intermittent episodes of hemiplegia from
one side to the other associated with
choreoathetosis and dystonia for min or weeks and
then resolve ,occasionally associated with migrain
Progressive MR and developmental disabilities , poor
prognosis
Radiology & lab are free

98. • Todds paralysis
• Stroke like episodes: migraine
• Focal post viral encephalitis, ADAM, HIV
• hyperlipidemia
• Familial hemiplegic migraine

99. Rehabilitation
Use of assessment measures
• The assessment tools selected should be
appropriate for the child’s age and
developmental and functional level

100. Goal of Rehabilitation
• Help children with physical disabilities
improve FUNCTION and PARTICIPATE more
fully in family, social, educational and
recreational activities.

101. Rehabilitation Tools
• Motor and cognitive exercises
• Medications
• Injections
• Braces
• Adaptive equipment
• Referral to therapies
• Referral to surgeries
• Recreational involvement

102. • Child +AIS+cardioembolic treat with
anticoagulant 5-7 days followed by oral
anticoagulant for 3-6 mon
• ALL children +AIS TREAT WITH aspirin 2-5
mglkglday after the anticoagulant therapy

103. - Prevention of recurrence:
Low dose aspirin or oral anticoagulant for long
term prophylaxis in certain conditions?

104. 1. Cardiac emboli
2. Disecting aneurysm
3. Severe prothrombotic conditions
4. High grade cerebral artery stenosis

105. 3- treatment of underlying cause
a) For SCD:
Exchange transfusion
Frequent transfusion
Bone marrow transplant
Hydroxy urea
• Moyamoya: consider
revascularization
• Prothrombotic
:consider
anticoagulant
• For others :low dose
asprin

106. • 4- rehabilitation therapy:
Speech , physiotherapy, occupational
&psychotherapy