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.

Terminology

Because of their considerable overlap, focal cerebral arteriopathy of childhood (FCA) and transient cerebral arteriopathy (TCA) hashave been used interchangeably. However, some more precise definitions have been proposed.

FCAFocal cerebral arteriopathy of childhood 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 FCAfocal cerebral arteriopathy of childhood regress or stabilize under 6 months, they can be restrospecivelyretrospectively renamed as TCAtransient cerebral arteriopathy.

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.

Epidemiology

Non-atherosclerotic arteriopathies account for 29–53% ² of stroke events in children, including FCAfocal cerebral arteriopathy of childhood, arterial dissection and Moyamoyamoyamoya disease. FCAFocal cerebral arteriopathy of childhood 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 FCAfocal cerebral arteriopathy of childhood, but other pathogens, including other herpes viruses, likely play a role.

Radiographic features

MRI

• parenchyma:lenticulostriate territory infarction
• MR angiography: unilateralstenosis/ 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

DSA

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≤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.

Differential diagnosis

A definitive diagnosis requires imaging over time. Most paediatric stroke centres perform serial imaging at around 3 and 12 months to monitor for vascular progression or stabilisation. Progression beyond 12 months suggests a progressive arteriopathy such as Moyamoyamoyamoya disease or primary angiitis of the CNS.