Introduction

Perinatal stroke is increasingly recognized as an important cause of neurological morbidity including cerebral palsy, epilepsy, and behavioral disorders, as well as impaired visual function and language development. The estimated incidence of perinatal stroke is approximately 1/4000.

Perinatal stroke can be classified by blood supply (venous vs. arterial), age at stroke (fetal vs. neonatal), age at diagnosis (neonatal symptomatic vs. presumed perinatal/neonatal asymptomatic), or type of stroke (ischemic vs. hemorrhagic). Investigators have used a variety of terms, including “perinatal stroke,” “arterial ischemic stroke,” and “perinatal arterial stroke” to describe the conditions. A recent workshop of the National Institute of Child Health and Human Development and the National Institute of Neurological Disorders and Stroke focused on refining the terminology. Ischemic perinatal stroke (IPS), now the term of choice, is defined 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.” The group further divided IPS into three categories based on the timing of diagnosis: (1) fetal ischemic stroke, diagnosed prior to birth using fetal imaging or following stillbirth on the basis of neuropathologic examination, (2) neonatal ischemic stroke, diagnosed after birth and ≤ 28th postnatal day (including preterm infants), and (3) presumed perinatal ischemic stroke (PPIS), diagnosed in children > 28 days of age in whom the ischemic event is presumed to have occurred between the 20th week of fetal life and the 28th postnatal day.