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Interrelationship of superficial siderosis and microbleeds in cerebral amyloid angiopathy

Ashkan Shoamanesh1, Sergi Martinez-Ramirez1, Jamary Oliveira-Filho1

  • 1From the Hemorrhagic Stroke Research Program, Massachusetts General Hospital, Harvard Medical School, Boston, MA.

Neurology
|October 17, 2014
PubMed
Abstract

Insights

Cerebral amyloid angiopathy-related cortical superficial siderosis (cSS) is linked to lower cerebral microbleed (CMB) counts and specific APOE genotypes, suggesting distinct underlying mechanisms for cSS and CMBs in advanced CAA.

Area of Science:

  • Neurology
  • Neuroimaging
  • Genetics

Background:

  • Cerebral amyloid angiopathy (CAA) is a common cause of spontaneous lobar hemorrhage.
  • Cortical superficial siderosis (cSS) is a marker of microvascular damage in CAA.
  • The relationship between cSS and cerebral microbleeds (CMBs) in CAA is not fully understood.

Purpose of the Study:

  • To investigate the neuroimaging and genetic associations of CAA-related cSS.
  • To explore the relationship between cSS severity and CMB counts.
  • To examine the role of APOE variants in the development of cSS.

Main Methods:

  • MRI scans were analyzed for cSS presence and severity in 84 probable or definite CAA subjects.
  • CMB counts were assessed, and APOE variants (ε2, ε4) were genotyped.
  • Cross-sectional analysis compared CMB counts and APOE allele frequencies across cSS severity groups.

Main Results:

  • 48% of subjects had cSS.
  • APOE ε2 was significantly associated with both focal and disseminated cSS.
  • CMB counts decreased with increasing cSS severity, while APOE ε4 was linked to higher CMB counts.

Conclusions:

  • CAA-related cSS occurs in individuals with lower CMB counts and distinct APOE genotypes.
  • These findings suggest separate vasculopathic mechanisms for cSS and CMBs in advanced CAA.
  • Further research into these distinct pathways may inform therapeutic strategies.

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