ISCHEMIC STROKE / ETIOLOGY

Focal cerebral arteriopathy (FCA)

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vascular imaging may reveal progression or bilateral involvement (which excludes TCA), therefore, the name TCA has been replaced by FCA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]
  • 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
  • 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

Management

Recanalization therapy in acute stroke
Antithrombotic drugs
Corticosteroids
  • the combination of corticosteroids + aspirin appears to be superior to aspirin monotherapy [Steinlin, 2017]
  • no standardized dosing regimen has been established
    • according to one study, the suggested duration of therapy is 2-16 weeks
    • the recommended starting dose is 10-20 mg/kg of methylprednisolone for 3-5 days, then switch to oral therapy with a gradual taper [Steinlin, 2017]
Revascularization
  • the same spectrum of procedures as with moyamoya

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Focal cerebral arteriopathy (FCA)
link: https://www.stroke-manual.com/focal-cerebral-arteriopathy-fca/