ISCHEMIC STROKE / ETIOLOGY

Focal cerebral arteriopathy (FCA)

Created 22/09/2022, last revision 26/09/2022

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 (usually the siphon, M1, or A1 segments)
    • follow-up vessel imaging may reveal progression or bilateral involvement (which excludes TCA); therefore, the name TCA has been replaced by FCA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´s 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)

Etiology

  • FCA is a syndrome with heterogeneous etiology
Content available only for logged-in subscribers (registration will be available soon)

Clinical presentation

  • stroke/TIA
    • typically, subcortical structures (basal ganglia) are affected
  • occasionally, a stepwise course is described

Diagnostic evaluation

Imaging methods

  • demonstration of the unilateral vascular lesion in typical localization (distal ACI, M1, A1) – i.e., detection of FCA
  • CT+CTA
  • MR+MRA (optimal for imaging parenchyma and early ischemia)
    • if vasculitis is suspected,  perform black blood sequences to show wall inflammation
  • neurosonology
  • the FCA severity score (FCASS) can be used to assess the extent → see here

Laboratory studies

  • etiological diagnosis
    • serum chemistry panel
    • CBC+ coagulation tests
    • inflammatory parameters (CRP, ESR, etc.)
    • D-Dimers
    • immunological tests (vasculitis)
    • lumbar puncture
    • genetics (e.g. for moyamoya)
    • other specific tests in DDx (e.g., Fabry, etc.)

Differential diagnosis

Content available only for logged-in subscribers (registration will be available soon)

Management

Recanalization therapy in acute stroke
Antithrombotic drugs
Corticosteroids
  • the combination of corticosteroids + aspirin appears superior to aspirin monotherapy [Steinlin, 2017]
  • no standardized regimen of dosing
    • according to one study, the duration of therapy is 2-16 weeks, initially 10-20 mg/kg methylprednisolone for 3-5 days, then per os with gradual taper [Steinlin, 2017]
Revascularization
  • the same spectrum of procedures as with moya-moya
icon-angle icon-bars icon-times