Focal cerebral arteriopathy (FCA)

Created 22/09/2022, last revision 02/05/2023


  • 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 (usually the distal ICA segment, M1, or A1 segments)
    • 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vascular imaging may reveal progression or bilateral involvement (which excludes TCA); therefore, the name TCA has been replaced by FCA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 radiologic entity with a heterogeneous etiology;  it is one of the most common causes of stroke in children (up to 50%)  [Rosa, 2015]
  • FCA is associated with a high risk of recurrence (up to 25%/year) [Fullerton, 2016]

FCA (focal cerebral arteriopathy of childhood)
FCA-d  (focal cerebral arteriopathy – dissection type)
FCA-i  (focal cerebral arteriopathy – inflammatory type)
TCA  (transient cerebral arteriopathy)
VIPS  (Vascular Effects of Infection in Pediatric Stroke)


  • FCA is a syndrome with heterogeneous etiology
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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 show wall inflammation
  • neurosonology
  • 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

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Recanalization therapy in acute stroke
Antithrombotic drugs
  • 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 duration of therapy is 2-16 weeks
    • start with 10-20 mg/kg of methylprednisolone for 3-5 days, then per os with gradual taper [Steinlin, 2017]
  • the same spectrum of procedures as with moya-moya
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Focal cerebral arteriopathy (FCA)