PediaNotes

Pediatric Stroke

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1. Introduction and Definition

  • Definition: A stroke is defined as the sudden onset of a focal neurological deficit lasting > 24 hours (or leading to death) caused by a disturbance in blood supply to the brain.
  • Pediatric Stroke Classification:
    1. Perinatal Stroke: 20 weeks gestation to 28 days of life.
    2. Childhood Stroke: > 28 days to 18 years of age.
  • Types:
    • Arterial Ischemic Stroke (AIS): Obstruction of an artery (Commonest).
    • Cerebral Sinovenous Thrombosis (CSVT): Thrombosis of venous sinuses.
    • Hemorrhagic Stroke: Rupture of a vessel.

2. Epidemiology

  • Incidence: 2–13 per 100,000 children per year.
  • Mortality: A top 10 cause of death in children.
  • Morbidity: > 60% of survivors have permanent neurological deficits (Cerebral Palsy, Epilepsy).

3. Etiology and Risk Factors (The “Black Box”)

Unlike adults (where atherosclerosis/HTN dominates), pediatric stroke is multifactorial.

CategorySpecific Causes / Risk Factors
Arteriopathies (50-80% of AIS)• Focal Cerebral Arteriopathy (FCA): Transient inflammatory stenosis (often post-Varicella).
• Moyamoya Disease: Chronic progressive stenosis of distal ICA.
• Arterial Dissection: Post-trauma (even minor neck trauma) or spontaneous.
• Vasculitis: PAN, Takayasu, Primary CNS vasculitis.
Cardiac Disorders (25%)• Congenital Heart Disease: Cyanotic > Acyanotic (R-to-L shunt).
• Procedures: Cardiac catheterization, ECMO.
• Infection: Infective Endocarditis.
• Cardiomyopathy/Arrhythmias.
Hematologic• Sickle Cell Disease: Highest risk (10% risk without screening).
• Prothrombotic States: Protein C/S deficiency, Factor V Leiden, Antithrombin III deficiency.
• Iron Deficiency Anemia: Thrombocytosis/hyperviscosity.
Infectious• Post-Varicella Angiopathy: Stroke 3–12 months after Chickenpox.
• Meningitis: Bacterial (Pneumococcal/TB) causing vasculitis.
• Head/Neck Infections: Mastoiditis (Risk of CSVT).
Metabolic / Genetic• Homocystinuria: Thromboembolism.
• MELAS: Mitochondrial Encephalomyopathy, Lactic Acidosis, Stroke-like episodes.
• Fabry Disease.

4. Clinical Presentation

Symptoms vary significantly by age.

A. Neonates / Infants

  • Seizures: Focal clonic seizures are the most common presentation.
  • Encephalopathy: Lethargy, apnea, poor feeding.
  • “Handedness”: Pathological early handedness (< 1 year) indicates contralateral hemiparesis.

B. Older Children

  • Hemiparesis: Sudden onset weakness of face/arm/leg.
  • Speech: Aphasia or dysarthria.
  • Visual: Hemianopia.
  • Headache/Vomiting: More common in Hemorrhagic stroke or CSVT.
  • Ataxia/Vertigo: Posterior circulation stroke.

5. Differential Diagnosis (Stroke Mimics)

  1. Seizures (Todd’s Paresis): Post-ictal weakness.
  2. Hemiplegic Migraine: Strong family history; headache follows aura.
  3. Intracranial Infection: Abscess, ADEM (Acute Disseminated Encephalomyelitis).
  4. Metabolic: Hypoglycemia (focal signs possible).
  5. Tumor: Acute hemorrhage into a tumor.

6. Diagnostic Evaluation

A. Neuroimaging (Stat)

  • MRI Brain with DWI (Diffusion Weighted Imaging): Gold Standard.
    • DWI: Shows restriction (bright) within minutes of ischemia.
    • ADC Map: Confirms true restriction (dark).
  • MRA / MRV (Angiography/Venography): Essential to visualize arteriopathy (Moyamoya, dissection) or sinus thrombosis.
  • CT Head: Less sensitive for acute ischemia but excellent for Hemorrhage. Used if MRI is unavailable or for rapid exclusion of bleed.

B. Etiological Workup (The “Stroke Protocol”)

  • Cardiac: Echocardiography (Transthoracic/Transesophageal) with bubble study (for PFO). ECG.
  • Vascular: Carotid Doppler (if dissection suspected).
  • Blood Work:
    • CBC (Sickle cell, Polycythemia, Platelets).
    • Coagulation profile (PT/APTT).
    • Thrombophilia screen (Protein C, S, AT3, Factor V Leiden, Lipoprotein-a, Homocysteine).
    • Inflammatory markers (ESR, CRP, ANA).
    • Lipid profile.

7. Management

A. Acute Management (The “Golden Hour”)

  1. Stabilization (ABC):

    • Maintain Oxygen saturation > 92%.
    • Blood Pressure: Permissive hypertension allowed (to maintain cerebral perfusion). Treat only if >95th percentile + 15-20%.
    • Glucose: Treat hypoglycemia immediately. Avoid hyperglycemia (worsens infarct).
    • Fever: Aggressive antipyretics (hyperthermia worsens outcome).
    • Seizures: Treat aggressively (Benzodiazepines Levetiracetam).

  2. Specific Reperfusion Therapy:

    • tPA (Tissue Plasminogen Activator): Controversial in children. Not routinely FDA approved. Considered only if:
      • Adolescent / Large child.
      • Within 4.5 hours window.
      • Confirmed large vessel occlusion.
    • Mechanical Thrombectomy: Increasing evidence for benefit in older children with large vessel occlusion (LVO) within 24 hours.

  3. Antithrombotic Therapy (Standard of Care):

    • Aspirin: (3–5 mg/kg/day). First-line for most Arterial Ischemic Strokes (non-cardioembolic, non-dissection).
    • Anticoagulation (LMWH/Unfractionated Heparin):
      • Indicated for Arterial Dissection (extracranial).
      • Indicated for Cardioembolic Stroke.
      • Indicated for Cerebral Sinovenous Thrombosis (CSVT).
      • Contraindication: Hemorrhagic stroke / large infarct with hemorrhagic transformation.

B. Disease-Specific Management

  • Sickle Cell Disease: Exchange Transfusion to reduce HbS < 30%. Chronic hypertransfusion program thereafter.
  • Moyamoya Disease: Surgical Revascularization (Pial synangiosis / EDAMS).
  • Autoimmune/Vasculitis: High-dose Steroids + Cyclophosphamide.

C. Chronic Management and Rehabilitation

  • Secondary Prevention: Long-term Aspirin (usually 2 years or lifelong depending on cause).
  • Rehabilitation: Physical therapy (constraint-induced movement therapy), Occupational therapy, Speech therapy.
  • Education: Individualized Education Plan (IEP) for cognitive deficits.

8. Prognosis

  • Recurrence: 20–30% overall. Highest risk in cases with Arteriopathy (Moyamoya/Vasculitis).
  • Neurological Outcome:
    • Hemiplegic Cerebral Palsy: Most common motor sequela.
    • Epilepsy: Develops in 25% of survivors.
    • Cognitive: Learning disabilities, ADHD, behavioral issues.
  • Plasticity: Young brains have higher plasticity, so motor recovery is often better than in adults, but cognitive deficits may emerge years later (“growing into the deficit”).

9. Summary Algorithm

  1. Suspicion: Sudden focal deficit.
  2. Confirm: MRI Brain (DWI) + MRA.
  3. Acute Tx: Neuroprotection + Aspirin (or LMWH if dissection/cardiac).
  4. Investigate: Echo + Blood work.
  5. Prevent: Treat underlying cause + Rehab.

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