ISCHEMIC STROKE / ETIOLOGY
Focal cerebral arteriopathy (FCA)
David Goldemund M.D.
Updated on 11/01/2024, published on 22/09/2022
Updated on 11/01/2024, published on 22/09/2022
Introduction
- focal cerebral arteriopathy (FCA) was originally referred to as transient cerebral arteriopathy (TCA)
- TCA was defined as a monophasic disease leading to unilateral intracranial stenosis (typically involving the distal ICA segment, M1, or A1 segments)
- however, follow-up vascular imaging may reveal progression or bilateral involvement (which excludes TCA), therefore, the name TCA has been replaced by FCA
- FCA is a radiological entity with a heterogeneous etiology; it is one of the most common causes of stroke in children (up to 50%) [Rosa, 2015]
- can be present in young adults as well [Bulder, 2012]
- associated with a high risk of recurrence (up to 25% per year) [Fullerton, 2016]
Etiology
- FCA is a syndrome with heterogeneous etiology
- transient cerebral arteriopathy (TCA) – approx. 30% of all FCAs [Bulder, 2012] [Steinlin, 2017]
- non-progressive, monophasic course
- unifocal and unilateral stenosis/irregularity of the large intracranial arteries of the anterior circulation (terminal ICA and its proximal branches)
- most likely para-infectious etiology associated with herpes virus (VZV, HS) infection, parvoviruses, enteroviruses, influenza A, mycoplasma pneumoniae, and also COVID-19 [Mirzaee, 2020]
- intracranial dissection
- the early stage of moyamoya disease
- arteriopathy associated with sickle cell disease
- the early stage of vasculitis
- vasculitis is more likely to be bilateral, affecting medium to small arteries
Clinical presentation
- stroke/TIA
- typically, subcortical structures (basal ganglia) are affected
- occasionally, a stepwise course has been reported
Diagnostic evaluation
Imaging methods
- demonstration of the vascular lesion in a typical location (distal ICA, M1, A1) ⇒ detection of FCA
- CT+CTA
- MR+MRA (optimal for imaging parenchyma and early ischemia)
- if vasculitis is suspected, obtain black blood sequences to visualize wall inflammation
- if vasculitis is suspected, obtain black blood sequences to visualize wall inflammation
- neurosonology to assess the flow patterns in the intracranial arteries, including collateral circulation
- the FCA Severity Score (FCASS) can assess the extent → see here
Laboratory studies
- etiological diagnosis
- serum chemistry panel
- CBC+ coagulation tests
- inflammatory parameters (CRP, ESR, etc.)
- D-Dimers
- immunologic tests (vasculitis)
- lumbar puncture
- genetic testing (e.g., for moyamoya, ACTA2 angiopathy)
- other specific tests in DDx (e.g., Fabry, etc.)
Differential diagnosis
- reversible cerebral vasoconstriction syndrome (RCVS)
- vasospasms
- dissection
- PACNS
- radiation-induced angiopathy
- drug-induced vasoconstriction
- partial recanalization of thrombotic occlusion
