Cortical Superficial Siderosis in Different Types of Cerebral Small Vessel Disease

Frank Arne Wollenweber, Ebru Baykara, Marialuisa Zedde, Benno Gesierich, Melanie Achmüller, Eric Jouvent, Anand Viswanathan, Stefan Ropele, Hugues Chabriat, Reinhold Schmidt, Christian Opherk, Martin Dichgans, Jennifer Linn, Marco Duering

Research output: Contribution to journalArticlepeer-review


Background and Purpose - Cortical superficial siderosis (cSS) has emerged as a clinically relevant imaging feature of cerebral amyloid angiopathy (CAA). However, it remains unknown whether cSS is also present in nonamyloid-associated small vessel disease and whether patients with cSS differ in terms of other small vessel disease imaging features. Methods - Three hundred sixty-four CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) patients, 372 population-based controls, and 100 CAA patients with cSS (fulfilling the modified Boston criteria for possible/probable CAA) were included. cSS and cerebral microbleeds were visually rated on T2∗-weighted magnetic resonance imaging. White matter hyperintensities were segmented on fluid-attenauted inversion recovery images, and their spatial distribution was compared between groups using colocalization analysis. Cerebral microbleeds location was determined in an observer-independent way using an atlas in standard space. Results - cSS was absent in CADASIL and present in only 2 population-based controls (0.5%). Cerebral microbleeds were present in 64% of CAA patients with cSS, 34% of patients with CADASIL, and 12% of population-based controls. Among patients with cerebral microbleeds, lobar location was found in 95% of CAA patients with cSS, 48% of CADASIL patients, and 69% of population-based controls. The spatial distribution of white matter hyperintensities was comparable between CAA with cSS and CADASIL as indicated by high colocalization coefficients. Conclusions - cSS was absent in CADASIL, whereas other small vessel disease imaging features were similar to CAA patients with cSS. Our findings suggest that cSS in combination with other small vessel disease imaging markers is highly indicative of CAA.

Original languageEnglish
Pages (from-to)1404-1407
Number of pages4
Issue number5
Publication statusPublished - May 1 2017
Externally publishedYes


  • cerebral amyloid angiopathy
  • cerebral small vessel diseases
  • intracranial hemorrhages
  • magnetic resonance imaging

ASJC Scopus subject areas

  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine
  • Advanced and Specialised Nursing


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