Abstract

Perinatal arterial ischemic stroke (AIS) occurs in 1 in 4000 live births and is the most common stroke in children. Consequences include seizures and a spectrum of cognitive and motor disability. Infantile spasms (IS) is an epileptic encephalopathy of infancy with an incidence of 2 in 10,000 live births. Approximately 5% of IS is caused by perinatal AIS. Patterns of ischemic injury that may predispose infants to IS and predict treatment response have not yet been identified. This retrospective case series of infants with AIS and IS provides detailed descriptions of ischemic distribution, seizure presentation, treatment and outcomes. Inclusion criteria were: term birth, ischemic stroke or encephalomalacia in an arterial distribution identified or presumed to have occurred in the perinatal period, a diagnosis of infantile spasms. Patients with a watershed pattern of injury were excluded. The modified pediatric ASPECTS was used to qualify and quantify the type and distribution of stroke. Areas of injury were identified on MRI and scored from T2/Flair or DWI sequences. Eleven patients with AIS and IS were identified. Of nine who fit inclusion criteria, all had MCA territory involvement with 6/9 having basal ganglia injury. Five had ischemia identified retrospectively after developing IS and four presented as neonates. The highest ASPECTS (bilateral deep MCA) was associated with the worst outcome in motor function and epilepsy control. However, the second-highest ASPECTS (unilateral MCA, bilateral ACA) had mild motor deficits and no seizure recurrence after IS resolution. The three lowest ASPECTS (<10) involved unilateral cortical MCA strokes sparing the deep structures, and also had the best motor outcome and seizure control. The co-occurrence of perinatal AIS and IS often results in significant neurologic disability. Although there was no defined pattern of regional homogeneity, there was a trend towards basal ganglia injury with progression of epilepsy after IS. This study highlights that size of ischemic injury may be less important than location as a predictor of motor outcome and seizure intractability. Future research will focus on identifying areas of injury that may confer increased risk of IS compared to stroke patients who remain seizure-free.

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