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 journalArticle

13 Citations (Scopus)

Abstract

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
JournalStroke
Volume48
Issue number5
DOIs
Publication statusPublished - May 1 2017
Externally publishedYes

Fingerprint

Cerebral Small Vessel Diseases
Siderosis
Cerebral Amyloid Angiopathy
CADASIL
Population Control
Atlases

Keywords

  • CADASIL
  • 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

Cite this

Wollenweber, F. A., Baykara, E., Zedde, M., Gesierich, B., Achmüller, M., Jouvent, E., ... Duering, M. (2017). Cortical Superficial Siderosis in Different Types of Cerebral Small Vessel Disease. Stroke, 48(5), 1404-1407. https://doi.org/10.1161/STROKEAHA.117.016833

Cortical Superficial Siderosis in Different Types of Cerebral Small Vessel Disease. / Wollenweber, Frank Arne; Baykara, Ebru; Zedde, Marialuisa; Gesierich, Benno; Achmüller, Melanie; Jouvent, Eric; Viswanathan, Anand; Ropele, Stefan; Chabriat, Hugues; Schmidt, Reinhold; Opherk, Christian; Dichgans, Martin; Linn, Jennifer; Duering, Marco.

In: Stroke, Vol. 48, No. 5, 01.05.2017, p. 1404-1407.

Research output: Contribution to journalArticle

Wollenweber, FA, Baykara, E, Zedde, M, Gesierich, B, Achmüller, M, Jouvent, E, Viswanathan, A, Ropele, S, Chabriat, H, Schmidt, R, Opherk, C, Dichgans, M, Linn, J & Duering, M 2017, 'Cortical Superficial Siderosis in Different Types of Cerebral Small Vessel Disease', Stroke, vol. 48, no. 5, pp. 1404-1407. https://doi.org/10.1161/STROKEAHA.117.016833
Wollenweber FA, Baykara E, Zedde M, Gesierich B, Achmüller M, Jouvent E et al. Cortical Superficial Siderosis in Different Types of Cerebral Small Vessel Disease. Stroke. 2017 May 1;48(5):1404-1407. https://doi.org/10.1161/STROKEAHA.117.016833
Wollenweber, Frank Arne ; Baykara, Ebru ; Zedde, Marialuisa ; Gesierich, Benno ; Achmüller, Melanie ; Jouvent, Eric ; Viswanathan, Anand ; Ropele, Stefan ; Chabriat, Hugues ; Schmidt, Reinhold ; Opherk, Christian ; Dichgans, Martin ; Linn, Jennifer ; Duering, Marco. / Cortical Superficial Siderosis in Different Types of Cerebral Small Vessel Disease. In: Stroke. 2017 ; Vol. 48, No. 5. pp. 1404-1407.
@article{debf7142caef45e5b20c086cc9df4fb0,
title = "Cortical Superficial Siderosis in Different Types of Cerebral Small Vessel Disease",
abstract = "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.",
keywords = "CADASIL, cerebral amyloid angiopathy, cerebral small vessel diseases, intracranial hemorrhages, magnetic resonance imaging",
author = "Wollenweber, {Frank Arne} and Ebru Baykara and Marialuisa Zedde and Benno Gesierich and Melanie Achm{\"u}ller and Eric Jouvent and Anand Viswanathan and Stefan Ropele and Hugues Chabriat and Reinhold Schmidt and Christian Opherk and Martin Dichgans and Jennifer Linn and Marco Duering",
year = "2017",
month = "5",
day = "1",
doi = "10.1161/STROKEAHA.117.016833",
language = "English",
volume = "48",
pages = "1404--1407",
journal = "Stroke",
issn = "0039-2499",
publisher = "Lippincott Williams and Wilkins",
number = "5",

}

TY - JOUR

T1 - Cortical Superficial Siderosis in Different Types of Cerebral Small Vessel Disease

AU - Wollenweber, Frank Arne

AU - Baykara, Ebru

AU - Zedde, Marialuisa

AU - Gesierich, Benno

AU - Achmüller, Melanie

AU - Jouvent, Eric

AU - Viswanathan, Anand

AU - Ropele, Stefan

AU - Chabriat, Hugues

AU - Schmidt, Reinhold

AU - Opherk, Christian

AU - Dichgans, Martin

AU - Linn, Jennifer

AU - Duering, Marco

PY - 2017/5/1

Y1 - 2017/5/1

N2 - 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.

AB - 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.

KW - CADASIL

KW - cerebral amyloid angiopathy

KW - cerebral small vessel diseases

KW - intracranial hemorrhages

KW - magnetic resonance imaging

UR - http://www.scopus.com/inward/record.url?scp=85017016568&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85017016568&partnerID=8YFLogxK

U2 - 10.1161/STROKEAHA.117.016833

DO - 10.1161/STROKEAHA.117.016833

M3 - Article

AN - SCOPUS:85017016568

VL - 48

SP - 1404

EP - 1407

JO - Stroke

JF - Stroke

SN - 0039-2499

IS - 5

ER -