TRANSIENT CEREBRAL ARTERIOPATHY - A DISORDER RECOGNIZED BY SERIAL ANGIOGRAMS IN CHILDREN WITH STROKE

Repeated clinical evaluation and cerebral arteriography during the evo lution of ischemic strokes of idiopathic origin allowed us to characte rize a transient cerebral arteriopathy. We retrospectively studied the clinical characteristics, course, and neuroimaging features of this d isorder in nine children. Of 34 children with ischemic strokes seen co nsecutively between 1954 and 1995, 9 (26%) were diagnosed as having tr ansient attack of the cerebral arterial wall, termed transient cerebra l arteriopathy. All of these patients had previously been in good heal th. The mean age at the time of the first stroke was 6 years (range, 2 9/12 years to 13 4/12 years). All children presented with acute hemip legia. A recurrence of the stroke took place 3 months at the latest af ter the initial infarct in three children (mean clinical follow-up 2 7 /12 years). Cerebral imaging in all the patients showed small subcorti cal infarcts located in basal ganglia or internal capsule. Arteriograp hy revealed multifocal lesions of the arterial wall (focal stenosis or segmental narrowing), mostly located in the initial parts of basal ar teries of the carotid system. Longitudinal arteriographic follow-up sh owed initial worsening of these arterial lesions (n = 5) for a maximum duration of 7 months followed by complete regression (n = 2), improve ment (n = 5), or stabilization of the lesions (n = 2). Five patients h ad a complete clinical recovery. Further studies are necessary to conf irm a presumed inflammatory cause of this arteriopathy.