- A CNS insult that causes objective evidence (clinical, radiographic, or pathologic) of damage in a vascular territory and clinical symptoms lasting <24 hours
- A stroke can be ischemic due to decreased arterial (arterial ischemic stroke [AIS]) or venous flow (cerebral sinovenous thrombosis [CSVT]) or hemorrhagic. Acute neurologic dysfunction and not causing evidence of brain injury is a transient ischemic attack (TIA) (typically last 1 to 2 hours).
- Acute hemiparesis, sensory loss, aphasia, cranial nerve deficits, ataxia, or altered consciousness (especially if occurring together) should prompt rapid evaluation for possible stroke to prevent delays in diagnosis. Acute neonatal stroke may present with seizures and depressed level of consciousness without focal signs. Silent strokes can occur with sickle cell disease and congenital heart disease.
- The neonatal period has the highest risk for pediatric stroke. Neonatal stroke occurs in approximately 1 in 4,000 live births.
- Incidence of AIS is 1.2 to 7.9 per 100,000 per year in children over a month of age.
- Hemorrhagic stroke incidence is estimated to be 0.5 to 5.1 per 100,000 children per year.
- CSVT occurs in 0.67 per 100,000 children per year.
Pediatric stroke occurs secondary to a plethora of etiologies as noted below. Some risk factors are genetic:
- Sickle cell disease: autosomal recessive (AR)
- Classic homocystinuria: CBS gene, AR
- Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like symptoms (MELAS): maternal inheritance
- Cerebral autosomal dominant (AD) arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL): NOTCH-3, AD
- Fabry disease: GLA gene, X-linked
- Ehlers-Danlos type IV: COL3A1 gene, AD
- Marfan syndrome: FBN1 gene, AD
- Moyamoya syndrome (RNF213, ACTA2)
- COL4A1 mutation
- Complex congenital heart defects with right to left shunt
- Cardiac rhabdomyoma or myxoma
- Infectious endocarditis, especially left sided
- Rheumatic heart disease
- Prosthetic heart valves
- Patent foramen ovale (PFO) with atrial septal aneurysm or known deep venous thrombosis (DVT) and large R>L shunt
- Congenital/genetic vasculopathies associated with Ehlers-Danlos type IV, Marfan syndrome, PHACES, neurofibromatosis type I, Down syndrome
- Moyamoya disease
- Fibromuscular dysplasia, postradiation vasculopathy, intracranial aneurysm, arterial agenesis or hypoplasia, dissection, transient cerebral arteriopathy, focal arteriopathy, arteriovenous malformation
- Vasculitis, inflammatory
- Systemic: systemic infections, varicella, lupus, hemolytic uremic syndrome, AIDS, Takayasu arteritis, drug abuse, Behçet disease
- Primary angiitis of the CNS
- Cranial or cervical infections (meningitis, encephalitis, sinusitis mastoiditis)
- Hematologic/coagulation disorders
- Severe anemia, polycythemia, or thrombocytosis
- Hemoglobinopathies especially hemoglobin SS (HgbSS) and Hgb S-thal
- Primary thrombophilia: antithrombin III deficiency, factor V Leiden homozygous mutation, protein S and C deficiencies, prothrombin G20210A homozygous mutation, hemophilia A and B
- Acquired thrombophilia: leukemia or other neoplasm, L-asparaginase treatment, anticardiolipin/antiphospholipid syndrome, estrogen-containing contraceptives, nephrotic syndrome, pregnancy/postpartum period, inflammatory bowel disease, thrombotic thrombocytopenic purpura
- Primary or acquired coagulopathies: anticoagulation, disseminated intravascular coagulation
- Blunt cervical or intraoral trauma (Consider cervical arterial dissection with high-speed MVA or cervical injury.)
- Carotid ligation (e.g., with extracorporeal membrane oxygenation [ECMO])
- Fat, air, foreign body, amniotic fluid embolism
- Catheter angiography
- Chiropractic manipulation
- Metabolic disorders
- Mitochondrial and related nuclear disorders
- Fabry disease
- Severe hypotension or hypertension
- Reversible vasoconstriction syndrome
- Cervical vessel compression
Recent evidence suggests that minor infection may increase the risk of pediatric stroke and that immunizations are protective.
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