Transient Cerebral Arteriopathy, Postvaricella Arteriopathy, and Focal Cerebral Arteriopathy or the Unique Susceptibility of the M1 Segment in Children With Stroke.

Abstract

See related article, p 2443. Like many disorders, the incidence of arterial ischemic stroke (AIS) throughout life is represented by a U-shaped curve. The peripartum period carries the highest risk, whereas people from 29 days to 18 years have the lowest risk. The incidence then gradually increases from adolescence to old age. Furthermore, the mechanisms of AIS are also age dependent. Placental–cerebral embolism is the most widely accepted hypothesis in perinatal AIS, whereas long-standing and diffuse arterial disease (namely, atherosclerosis) is the leading cause in adults. In childhood as well, stenosing arteriopathies are the most frequent causes of AIS.1–4 Some of them, such as cervical dissection, moyamoya, sickle-cell arteriopathy or other specific diagnoses (postirradiation arteriopathy, neurofibromatosis, fibromuscular dysplasia, reversible vasoconstriction syndrome…) are well recognized. Nevertheless, after exclusion of these disorders, a large proportion of children (notably those previously healthy) are diagnosed with another type of arterial disease, which is characteristically focal, intracranial, and monophasic. Because the nature of the arterial insult is largely unknown, patients with these features have been reported under diverse nosography (Table), which refers to the angiographic appearance and the time course rather than to a pathophysiological mechanism. Each definition has its proper advantages and limitations. Yet …