Focal cerebral arteriopathy of childhood
Focal cerebral arteriopathy of childhood, also known as transient cerebral arteriopathy, is characteristically an acute monophasic disease, with unilateral stenosis of the distal internal carotid artery and/or the proximal middle/ anterior cerebral arteries, causing infarction in the lenticulostriate territory.
Because of their considerable overlap, focal cerebral arteriopathy of childhood (FCA) and transient cerebral arteriopathy (TCA) has been used interchangeably. However, some more precise definitions have been proposed.
FCA could be seen as a descriptive umbrella term: a diagnosis established early in the course of the disease, which includes various entities.
Since the great majority of cases of FCA regress or stabilize under 6 months, they can be restrospecively renamed as TCA.
Additionally, a large proportion of these cases are associated with varicella infection in the previous 12 months, thus fulfilling the criteria for post-varicella arteriopathy 1.
More recently, subtypes of focal arteriopathy have been proposed, which include FCA-i (inflammatory) and FCA-d (dissection) 2.
Non-atherosclerotic arteriopathies account for 29–53% 2 of stroke events in children, including FCA, arterial dissection and Moyamoya disease. FCA has rarely been described in young adults.
Most cases are presumed to be inflammatory. Varicella-zoster virus is a well known and established cause of FCA, but other pathogens, including other herpes viruses, likely play a role.
- parenchyma: lenticulostriate territory infarction
- MR angiography: unilateral stenosis/ irregularity of distal ICA, proximal MCA and/or proximal ACA. Progression of stenosis can happen during the first 3 – 6 months, then followed by stabilization or normalization.
- vessel-wall imaging: wall thickening and concentric enhancement postcontrast study
An arterial banding pattern is pathognomonic in FCA-i, but uncommon (24%) 3.
Treatment and prognosis
Focal cerebral arteriopathy is associated with a high risk of recurrent stroke (≤ 25% within 1 year) 4.
In its early phase, it can rapidly progress over days to weeks. Children with progressive arteriopathies have a higher risk of recurrent ischemia.
A definitive diagnosis requires imaging over time. Most pediatric stroke centers perform serial imaging at around 3 and 12 months to monitor for vascular progression or stabilization. Progression beyond 12 months suggests a progressive arteriopathy such as Moyamoya disease or primary angiitis of the CNS.
- Richard Roger Warne, Saipriya Ramji, Wui K. Chong. Pediatric Stroke and Radiological Approach. (2021) doi:10.1007/978-3-319-61423-6_35-2
- Heather J. Fullerton, Nicholas Stence, Nancy K. Hills, Bin Jiang, Catherine Amlie-Lefond, Timothy J. Bernard, Neil R. Friedman, Rebecca Ichord, Mark T. Mackay, Mubeen F. Rafay, Stéphane Chabrier, Maja Steinlin, Mitchell S.V. Elkind, Gabrielle A. deVeber, Max Wintermark, the VIPS Investigators. Focal Cerebral Arteriopathy of Childhood. (2018) Stroke. doi:10.1161/STROKEAHA.118.021556
- M. Wintermark, N.K. Hills, G.A. DeVeber, A.J. Barkovich, T.J. Bernard, N.R. Friedman, M.T. Mackay, A. Kirton, G. Zhu, C. Leiva-Salinas, Q. Hou, H.J. Fullerton, the VIPS Investigators. Clinical and Imaging Characteristics of Arteriopathy Subtypes in Children with Arterial Ischemic Stroke: Results of the VIPS Study. (2017) American Journal of Neuroradiology. 38 (11): 2172. doi:10.3174/ajnr.A5376 - Pubmed
- Fearn ND, Mackay MT. Focal cerebral arteriopathy and childhood stroke. Curr Opin Neurol. 2020 Feb;33(1):37-46. doi: 10.1097/WCO.0000000000000787. PMID: 31815778.
- Stéphane Chabrier, Guillaume Sébire, Joel Fluss. Transient Cerebral Arteriopathy, Postvaricella Arteriopathy, and Focal Cerebral Arteriopathy or the Unique Susceptibility of the M1 Segment in Children With Stroke. (2016) Stroke. doi:10.1161/STROKEAHA.116.014606