title: Cortical superficial siderosis: detection and clinical significance in cerebral amyloid angiopathy and related conditions.
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.
authors.affiliation: 1 Stroke Research Group, Department of Brain Repair and Rehabilitation, UCL Institute of Neurology and The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK 2 Stroke Research Centre, Massachusetts General Hospital, Boston, MA, USA.
authors.forename: Andreas
authors.initials: A
authors.lastname: Charidimou
authors.affiliation: 3 Department of Neuroradiology, University Hospital Munich, Marchioninistrasse 15, 81377 Munich, Germany.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Linn
authors.affiliation: 4 Department of Radiology and Department of Epidemiology, Erasmus MC University Medical Centre, Rotterdam, Netherlands.
authors.forename: Meike W
authors.initials: MW
authors.lastname: Vernooij
authors.affiliation: 5 Institute for Stroke and Dementia Research, Ludwig Maximilians University, Munich, and Department of Neurology, SLK-Kliniken, Heilbronn, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Opherk
authors.affiliation: 4 Department of Radiology and Department of Epidemiology, Erasmus MC University Medical Centre, Rotterdam, Netherlands.
authors.forename: Saloua
authors.initials: S
authors.lastname: Akoudad
authors.affiliation: 6 UMR 894 INSERM-Université Paris 5, Sorbonne Paris Cité, Paris, France.
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Baron
authors.affiliation: 2 Stroke Research Centre, Massachusetts General Hospital, Boston, MA, USA.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Greenberg
authors.affiliation: 1 Stroke Research Group, Department of Brain Repair and Rehabilitation, UCL Institute of Neurology and The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK 7 Lysholm Department of Neuroradiology, UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery, London, UK.
authors.forename: Hans Rolf
authors.initials: HR
authors.lastname: Jäger
authors.affiliation: 1 Stroke Research Group, Department of Brain Repair and Rehabilitation, UCL Institute of Neurology and The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK d.werring@ucl.ac.uk.
authors.forename: David J
authors.initials: DJ
authors.lastname: Werring
mesh_terms: D000818:Animals; D001921:Brain / Q000473:pathology*; D016657:Cerebral Amyloid Angiopathy / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D002543:Cerebral Hemorrhage / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D015995:Prevalence; D012806:Siderosis / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology
keywords: cerebral amyloid angiopathy; cortical superficial siderosis; intracerebral haemorrhage; magnetic resonance imaging; small vessel disease