Childhood Stroke

Dr Dinesh Udainiya
DM(Neurology)

The challenge..
How to cover this
enormous topic in 30
minutes and give
some insightful advice
to the practicing
paeds

Epidemiology
Incidence 8/100,000/year (1.3-13)
Incidence in neonates 1/4,000/year
Incidence increasing
More sensitive imaging

Death in 6% (top 10 causes of death in children)

Neurologic deficits in 2/3
20-30% recurrence risk

Children are not little adults

Incidence is rare
Subtle neurologic
presentation
Underdiagnosis and
delay in diagnosis
Multiple types of
stroke
Multiple risk factors

Type of Stroke

STROKE
Acute Ischemic Stroke
(AIS)

Hemorrhagic Stroke
(HS)

Cerebral Venous
Thrombosis (CVT)

Vascular malformations

Infection

ITP/Hemophilia

Dehydration

Brain tumors

Prothrombotic states

Acute Ischemic Stroke

Incidence is 3/100,000, year
Neonates account for 25% of AIS
median age 5 yrs
Male predominance (60%)
Predominance in African-American
population

Clinical Features of AIS

Canadian Registry
51% hemiparesis
48% seizures
speech disorder
headache, lethargy, confusion

Neonates
<25% hemiparesis
Lethargy and seizures predominance

Risk Factors for AIS

Vascular

AIS
Embolic

Intra-vascular

Vascular Risk Factors

Vascular

Arteriopathies

Transient

Vasospastic

Vasculitis

Systemic vascular
disease

Infectious

Progressive

Connective tissue disease

Drugs

Embolic Risk Factors

Embolic
Congenital
Heart Disease
Cyanotic Heart
Disease

Acquired Heart
Disease

Cardiomyopathy
PFO

Arrhythmia

Trauma

Intravascular Risk Factors

(The Hematologists Domain)
Intravascular
Hematologic
Disorders

Sickle cell

Prothrombotic
States

Acquired

Iron deficiency
Leukemia

Metabolic

Hyper
homocysteinemia
Congenital

Hyperlipidemia

The Acquired Prothrombotic States

Meds

Acquired
Lupus
Anticoagulants

Pregnancy

The Congenital Prothrombotic

States
ATIII
Lipoprotein
a

MTHFR

Protein C

Congenital

APC
resistance

Protein S

PT20210
Plasminogen

Inter-relations
Vascular

Embolic

Intravascular

AIS Risk Factors

Pediatric Stroke Study :

525 children (ages 29 days to 19 years) with
arterial ischemic stroke
Vascular imaging found arteriopathy in 277
(53%)

In the 277 cases with arteriopathy:

Focal cerebral arteriopathy of childhood (25%)
Primary or secondary moyamoya (22%)
Dissection (20%)
Vasculitis (12%)
Sickle cell disease arteriopathy (8%)
Postvaricella arteriopathy (7%)
Miscellaneous types (4%)
Unspecified (3%)

Focal cerebral arteriopathy (FCA) of

childhood
Term used by the International Pediatric Stroke
Study
Unexplained focal arterial stenosis in a child with
arterial ischemic stroke

Focal cerebral arteriopathy (FCA) of

childhood
Etiology unknown, probably multifactorial
Possible causes:
Inflammation and vasculitis due to infection (eg
antecedent varicella infection) or autoimmune
disease
Thromboembolic arterial occlusion or stenosis
Intracranial dissection
Arterial spasm
Prothrombotic factors

Moyamoya syndrome
Progressive stenosis of the internal carotid
arteries and formation of collateral vessels
Name moyamoya means puff of smoke in
Japanese and describes the look of the tangle of
tiny vessels formed to compensate for the
blockage

Arterial Dissection
Definite or probable trauma is identified in some
cases
Spontaneous dissection also occurs
Connective tissue disorders such as vascular
Ehlers- Danlos syndrome and Marfan syndrome
can predispose to dissection

Vasculitis
Inflammatory changes in the cerebral vessels
Primary

Kawasaki disease
Secondary

Collagen vascular diseases (such as

Lupus)

Infections (bacterial meningitis, viral

infections)

Other abnormities of vessel structure

Arterial tortuosity syndrome
Vasospasms resulting from subarachnoid

hemorrhage
Fibromuscular dysplasia

Metabolic disorders
Several metabolic conditions associated with
arterial ischemic stroke
Generally through effects on the vessel wall
CADASIL
MELAS

CADASIL
Cerebral Autosomal-Dominant Arteriopathy with
Subcortical Infarcts and Leukoencephalopathy
Mutation in the Notch3 gene, short arm of

chromosome 19
Progressive degeneration of smooth muscle
cells in the vessel wall
May present with migraine, TIA, or ischemic
stroke in late childhood or early adulthood

MELAS
Mitochondrial Encephalopathy with Lactic Acidosis
and Stroke-like episodes
Mutations of mitochondrial DNA
Metabolic stroke rather arterial stroke
Occurrence of stroke-like episodes: temporary
muscle weakness on one side of the body
(hemiparesis)
Other features: focal or generalized seizures,
recurrent migraine-like headaches, vomiting, short
stature, hearing loss and muscle weakness,
dementia

MELAS
Diagnostic criteria:
Stroke-like episodes
Encephalopathy characterized by seizures or
dementia
Blood lactic acidosis or
Presence of ragged red fibers in skeletal muscle
biopsy

The Diagnostic Work-Up

History
Trauma, infection, palpitations, mental status
chages, underlying disease
Previous DVTs, family history

Physical Exam
Marfanoid body habitus
Cutaneous lesions
Caf au lait spots
xanthoma

The Diagnostic Work-Up

Laboratory Studies
CBC, comprehensive metabolic panel, ESR
Toxicology and infectious studies
The hypercoagulation studies

Imaging Studies
CT
MRI/MRA/MRV
Echo

The hypercoagulation profile

Implicated in 38%-75% of childhood stroke
patients
Expensive
Rare disorders
Transient disorders
What can you do about it?
B12, folate, B6 in hyperhomocystenemia
Niacin in lipoprotein a

Therapy
Absence of RCT
Adapted from adults
Treat underlying risk factor
Prevent recurrence

Acute management of child with possible

stroke: The First 15 minutes

Goals:

1 cerebral perfusion
2 oxygenation
3 minimize demand for cerebral blood flow
Head of Bed Flat- if alert or 30 degree if not alert aspiration risk
IV Fluids: isotonic, at minimize+, no dextrose- as want to avoid
hyperglycemia check sugar
Oxygen only if needed keep sats >95%
Acetaminophen if temp > 37.0
Get a neuro exam you can follow serially
- Peds NIHSS- Peds stroke cards

Consensus on
Sickle cell disease
Acute therapy
Exchange transfusion

Preventive therapy
Blood transfusion every 3-6 weeks to maintain
HbS<30%
? stem cell transplant
Transcranial dopplers

Current recommendations
Neonatal AIS no therapy
Dissecting vasculopathy anticoagulation
3-6 months
Cardiogenic embolism anticoagulation
but no consensus on length of time
Vasculopathy ASA (no consensus on
dose 1-5mg/kg/day)
Recurrent stroke consider
anticoagulation

Current practice..
Most (>50%) will use LMWH/UH 5-7 days
in non neonatal period followed by ASA
Thrombolytic agents are rarely used in
pediatrics and their use is recommended
only in conjuction with clinical trials.

Other therapies
Treatment of infections
Immunosuppressive therapy: vasculitis
Transfusion therapy: sickle cell anemia
B-vitamins & folate : MTFHR
T/T of iron deficiency
Neuroprotective agents: experimental
Stem cell therapies: experimental

Neurosurgical treatment
Revascularization in Moya-Moya
Evacuation of hematoma
Decompressive craniectomy
Surgical resection of aneurysms and AV
malformations

Rehabilitation
Reduces neurological impairments
Aim to achieve developmentally relevant selfcare, school-related and play skills
Potential for on-going recovery over longer
periods of time
Ongoing modification of rehabilitation according
to growth and development

Motor Rehab
Spasticity, hemidystonias: physiotherapy,
Botox
Splinting, casting, movt therapy,
functional electrical stimulation
Muscle strengthening

Primary prevention
Transfusion therapy - sickle cell anemia
Early identification and treatment of dehydration
Prevention of meningitis: BCG, Pneumococcal
vaccination
Treatment of iron deficiency anemia
Varicella vaccine ?
Vit K prophylaxis in newborn

Outcomes of Childhood AIS

1991 85% long-term sequelae
2001 60% long-term sequelae
Hemiparesis, speech, learning and behavior
WORSE IF..

Multiple risk factors

CHD/progressive vasculopathy
Larger infarct
Stroke after neonatal period
Seizures with stroke

What do we need for the future?

Prospective cohort
studies
Standard evaluation of
risk and outcome
Develop therapy and
prevention strategies

Incidence studies
Case control studies
of risk factors
Outcome studies