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Patient Story

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A 16-year-old boy with a history of idiopathic dilated cardiomyopathy and severe ventricular dysfunction was admitted to the hospital for vomiting, confusion, involuntary movements, and slurred speech. On physical examination, he was found to be lethargic and to have dysmetria with the finger-to-nose test, ataxia, and incoordination of rapid alternating movements. He was found to have decreased biventricular systolic dysfunction and a large thrombus at the apex of the left ventricle on cardiac echocardiogram, and a left cerebellar stroke on CT scan of the brain (Figure 204-1). He was treated with anticoagulation and supportive care, with improvement of his neurologic symptoms. He eventually underwent a successful orthotopic heart transplant.

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FIGURE 204-1

Acute/subacute left cerebellar infarct on brain CT of a 16-year-old with dilated cardiomyopathy and severe ventricular dysfunction. (Used with permission from Camille Sabella, MD.)

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Introduction

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Cerebral vascular accidents are uncommon in children. Ischemic and hemorrhagic strokes are often due to an underlying cause which should be identified and addressed. Acute treatment is largely supportive. Prevention of recurrence is critical.

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Synonyms

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Stroke.

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Epidemiology

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  • Approximately 2 CVAs per 100,000 person per year.1

  • Approximately 50 percent of strokes are ischemic and 50 percent are hemorrhagic.1

  • Ten percent of hemorrhagic strokes are due to central venous sinus thrombosis.2

  • Fifty percent of patients presenting with a focal neurologic deficit have a previously identified risk factor for stroke.3

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Etiology and Pathophysiology

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  • Ischemic CVAs can be secondary to a wide range of underlying causes.

    • The cause can be determined in approximately 2/3 of patients.2

    • Major causes include congenital heart disease, sickle cell disease, infections, and prothrombotic states.

    • Moyamoya is rare in the US, but the most common cause in Japan.

    • Fibromuscular dysplasia more commonly presents in young adults.

  • Hemorrhagic CVAs occur when vessels bleed into the brain, usually as the result of a vascular abnormality or clotting disorder.

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Risk Factors

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Ischemic Stroke
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  • Congenital heart malformations (Figure 204-1).

  • Cerebrovascular abnormalities.

  • Sickle cell disease (10% have a clinically evident stroke by age 20 years; Figure 204-2).

  • Collagen vascular diseases.

  • Infection (meningitis and sepsis).

  • Prothrombotic risk factors (elevated homocysteine, elevated lipoprotein (a), protein C and protein S deficiencies, Factor V Leiden, and anti-phospholipid antibodies).

  • Migraine with aura, especially with smoking, pregnancy, or oral contraceptive use.

  • Trauma.

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FIGURE 204-2

Brain MRI showing remote infarction in the distribution of the middle cerebral artery and volume loss at the right frontoparietal cortex in a patient with sickle cell disease. (Used with permission from Stefanie Thomas, MD.)

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Hemorrhagic ...

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