Stroke in Neonates

KEY POINTS

• 1.

  Perinatal arterial ischemic stroke most often results from the convergence of multiple stroke risk factors specific to the perinatal period and has a low risk of recurrence.

• 2.

  Neonatal arterial ischemic stroke refers to the most common presentation of perinatal stroke when focal seizures or diffuse neurologic signs lead to diagnosis soon after birth. A subset of patients remain undiagnosed in the neonatal period and present as having presumed perinatal arterial ischemic stroke, with emerging neurologic deficits later in infancy.

• 3.

  Cardiac disease, although present in a minority of cases, is a risk factor for stroke recurrence and should be investigated with echocardiography after perinatal stroke diagnosis.

• 4.

  In the absence of multiple thromboembolic events and/or a family history of thrombosis, extensive thrombophilia evaluations are generally not recommended because they do not predict recurrence or alter management.

• 5.

  Management of perinatal stroke should focus on supportive care with the primary goal of promoting adequate perfusion to the brain and minimizing extension of the injury. Although the presentations of perinatal stroke and hypoxic-ischemic encephalopathy may overlap, treatment with hypothermia should not be delayed if hypoxic-ischemic encephalopathy is suspected.

Introduction

The perinatal period represents one of the highest-risk times of life for stroke, and the consequences are significant in terms of neurologic morbidity across the lifespan. In 2007 an international workshop convened by the National Institutes of Neurological Disorders and Stroke defined ischemic perinatal stroke as a “group of heterogeneous conditions in which there is focal disruption of cerebral blood flow secondary to arterial or cerebral venous thrombosis or embolization, between 20 weeks of fetal life through the 28th postnatal day, confirmed by neuroimaging or neuropathologic studies.” Perinatal stroke can be divided into distinct syndromes based on the pathophysiology, presentation, and developmental stage at which it occurs. Neonatal arterial ischemic stroke (NAIS), cerebral sinovenous thrombosis (CSVT), and neonatal hemorrhagic stroke typically present acutely in the neonatal period, whereas presumed perinatal arterial ischemic stroke (PPAIS), periventricular venous infarction, and presumed perinatal hemorrhagic stroke occur pre- or perinatally but frequently do not manifest until later in infancy ( Fig. 50.1 ).

Epidemiology

Ischemic perinatal stroke occurs in 1 in 2300 to 5000 live births, with most presenting within the first week of life, significantly higher than the weekly risk of stroke in an adult with known risk factors. Arterial strokes represent the majority of these, with most presenting acutely in the neonatal period (NAIS). The incidence of CSVT is less common, occurring in 2.6 per 100,000 births. ^, A male predominance has been demonstrated in multiple studies of perinatal stroke. ^, Hemorrhagic stroke is significantly less common in the perinatal population and is often excluded from studies of perinatal stroke, but one study identified a population prevalence (excluding isolated germinal matrix hemorrhage) of 6.2 in 100,000 live births over a 10-year period, also with a male predominance.