Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy(CME)

Department of Neuroradiology, University Hospital Munich, Marchioninistrasse 15, 81377 Munich, Germany.
Neurology (Impact Factor: 8.29). 04/2010; 74(17):1346-50. DOI: 10.1212/WNL.0b013e3181dad605
Source: PubMed


Cerebral amyloid angiopathy (CAA) typically presents with lobar intracerebral macrohemorrhages (ICH) or microbleeds (MBs). Several case reports also found superficial siderosis (SS) in patients with CAA. We aimed to assess the value of SS for the in vivo diagnosis of CAA, and tested whether the inclusion of SS as a criterion alters the sensitivity and specificity of the Boston criteria for CAA-related hemorrhage.
We retrospectively analyzed the T2*-weighted MRIs of 38 patients with histopathologically proven CAA and of 22 control patients with histopathologically proven non-CAA ICHs regarding the presence of ICHs, MBs, and SS. We compared the sensitivity and specificity of the classic Boston criteria to that of modified criteria, which included SS as a criterion.
ICHs were present in 71% of the patients with CAA, and in all control patients. MBs were found in 47.4% of patients with CAA and in 22.7% of controls. SS was detected in 60.5% of patients with CAA, but in none of the controls. The classic criteria had a sensitivity of 89.5% for CAA-related hemorrhage, while inclusion of SS increased their sensitivity to 94.7% (not significant). On the contrary, the specificity of the Boston criteria was 81.2% both for the classic and for the modified criteria.
Superficial siderosis (SS) occurs with high prevalence in cerebral amyloid angiopathy (CAA) and is rare in non-CAA forms of intracerebral hemorrhages. Thus, we propose that inclusion of SS in the Boston criteria might enhance their sensitivity for CAA-related hemorrhage without loss of specificity.

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Available from: Jennifer Linn, Feb 19, 2014
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    • "Within the past decade, a second type of brain siderosis restricted to the supratentorial compartment and the convexities of the cerebral hemispheres, has been identified and referred to as 'cortical' superficial siderosis (cSS) (Linn et al., 2008). cSS has a different range of potential causes and clinical presentation to classical siderosis, but in older individuals is emerging as a key feature of cerebral amyloid angiopathy (CAA), a common and important age-related cerebral small vessel disorder (Linn et al., 2010; Viswanathan and Greenberg, 2011; Charidimou et al., 2012a). cSS is associated with characteristic clinical symptoms , including transient focal neurological episodes (Greenberg et al., 1993), and might be a marker of future intracerebral haemorrhage (ICH) risk in CAA patients (Charidimou et al., 2013c; Linn et al., 2013). "
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    ABSTRACT: Cortical superficial siderosis describes a distinct pattern of blood-breakdown product deposition limited to cortical sulci over the convexities of the cerebral hemispheres, sparing the brainstem, cerebellum and spinal cord. Although cortical superficial siderosis has many possible causes, it is emerging as a key feature of cerebral amyloid angiopathy, a common and important age-related cerebral small vessel disorder leading to intracerebral haemorrhage and dementia. In cerebral amyloid angiopathy cohorts, cortical superficial siderosis is associated with characteristic clinical symptoms, including transient focal neurological episodes; preliminary data also suggest an association with a high risk of future intracerebral haemorrhage, with potential implications for antithrombotic treatment decisions. Thus, cortical superficial siderosis is of relevance to neurologists working in neurovascular, memory and epilepsy clinics, and neurovascular emergency services, emphasizing the need for appropriate blood-sensitive magnetic resonance sequences to be routinely acquired in these clinical settings. In this review we focus on recent developments in neuroimaging and detection, aetiology, prevalence, pathophysiology and clinical significance of cortical superficial siderosis, with a particular emphasis on cerebral amyloid angiopathy. We also highlight important areas for future investigation and propose standards for evaluating cortical superficial siderosis in research studies. © The Author (2015). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email:
    Brain 06/2015; DOI:10.1093/brain/awv162 · 9.20 Impact Factor
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    • "According to these criteria, the detection of multiple (2) strictly lobar hemorrhages (large or small, symptomatic or asymptomatic) without known underlying cause in individuals aged .55 years is highly specific of the disease [4]. However, validation studies of these criteria have been mostly based on patients admitted because of lobar ICH [4] [13] [14], precluding the study of the diagnostic value of lobar MBs without symptomatic ICH. At a community level, the Rotterdam and Framingham studies have shown that healthy elderly individuals with strictly lobar MBs have an increased frequency of the APOE-ε4 allele (compared with patients with MBs not strictly confined to lobar regions) [11] [15], which is in agreement with increased APOE-ε4 frequencies seen in patients with " probable CAA " [16]. "
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    ABSTRACT: The Boston criteria are the basis for a noninvasive diagnosis of cerebral amyloid angiopathy (CAA) in the setting of lobar intracerebral hemorrhage (ICH). We assessed the accuracy of these criteria in individuals with lobar microbleeds (MBs) without ICH. We identified individuals aged >55 years having brain magnetic resonance imaging (MRI) and pathologic assessment of CAA in a single academic hospital and a community-based population (Framingham Heart Study [FHS]). We determined the positive predictive value (PPV) of the Boston criteria for CAA in both cohorts, using lobar MBs as the only hemorrhagic lesion to fulfill the criteria. We included 102 individuals: 55 from the hospital-based cohort and 47 from FHS (mean age at MRI 74.7 ± 8.5 and 83.4 ± 10.9 years; CAA prevalence 60% and 46.8%; cases with any lobar MB 49% and 21.3%; and cases with ≥2 strictly lobar MBs 29.1% and 8.5%, respectively). PPV of "probable CAA" (≥2 strictly lobar MBs) was 87.5 (95% confidence interval [CI], 60.4-97.8) and 25 (95% CI, 13.2-78) in hospital and general populations, respectively. Strictly lobar MBs strongly predict CAA in non-ICH individuals when found in a hospital context. However, their diagnostic accuracy in the general population appears limited. Copyright © 2015 The Alzheimer's Association. Published by Elsevier Inc. All rights reserved.
    Alzheimer's & dementia: the journal of the Alzheimer's Association 06/2015; 128. DOI:10.1016/j.jalz.2015.04.009 · 12.41 Impact Factor
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    • "It is estimated that repeated cSAH leads to haemosiderin deposits in the subpial layers of the supratentorial brain [19]. In addition to other lobar haemorrhagic lesions, SS depicted predominantly in the supratentorial area has been increasingly recognised as one of the CAA-related abnormal findings [24]. "
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    ABSTRACT: Sporadic cerebral amyloid angiopathy (CAA) is common cause of cerebrovascular disorders that predominantly affect elderly patients. When symptomatic, cortical-subcortical intracerebral haemorrhage (ICH) in the elderly is the most well-known manifestation of CAA. Furthermore, the clinical presentation varies from a sudden neurological deficit to seizures, transient symptoms and acute progressive cognitive decline. Despite its clinical importance, this multifaceted nature poses a diagnostic challenge for radiologists. The aims of this study were to expound the characteristics of neuroimaging modalities, which cover a wide spectrum of CAA-related imaging findings, and to review the various abnormal findings for which CAA could be responsible. Radiologically, in addition to typical ICH, CAA leads to various types of abnormal findings, including microbleed, subarachnoid haemorrhage, superficial siderosis, microinfarction, reversible oedema, and irreversible leukoaraiosis. Taking into consideration the clinical importance of CAA-related disorders such as haemorrhagic risks and treatable oedema, it is necessary for radiologists to understand the wide spectrum of CAA-related imaging findings. • To describe the characteristics of imaging modalities and findings of CAA-related disorders. • MRI, especially gradient echo sequences, provides the useful information of CAA-related haemosiderin depositions. • To understand the wide spectrum of CAA-related neuroimaging and clinical features is important.
    Insights into Imaging 02/2014; 5(3). DOI:10.1007/s13244-014-0312-x
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