@article{553a8ff19db042f7b76a98e9353cbdb6,
title = "Prevalence and natural history of superficial siderosis a population-based study",
abstract = "Background and Purpose-Superficial siderosis (SS) is characterized by hemosiderin deposition in the superficial layers of the central nervous system and can be seen during postmortem examination or with iron-sensitive magnetic resonance imaging techniques. The distribution of SS may predict the probable underlying cause. This study aimed to report the prevalence and natural history of SS in a population-based study. Methods-Brain magnetic resonance imaging scans from the MCSA (Mayo Clinic Study of Aging), a population-based study of residents 50 to 89 years of age in Olmsted County, Minnesota, were reviewed. Participants with imaging consistent with SS were identified from 2011 through 2016. An inverse probability weighting approach was used to convert our observed frequencies to population prevalence of SS. Additional data abstracted included amyloid positron emission tomography, Apolipoprotein E genotype, coexisting cerebral microbleeds, and extent of SS. Results-A total of 1412 participants had eligible magnetic resonance imaging scans. Two participants had infratentorial SS, restricted to the posterior fossa. Thirteen participants had cortical SS involving the cerebral convexities (7 focal and 6 disseminated). Only 3 of the participants with cortical SS (23%) also had cerebral microbleeds. The population prevalence of SS was 0.21% (95% confidence interval, 0-0.45) in those 50 to 69 years old and 1.43% (confidence interval, 0.53-2.34) in those over 69 years old. Apolipoprotein E ϵ2 allele was more common in those with SS (57.1% versus 15.0%; P<0.001). Compared with participants without SS, those with SS were also more likely to have a positive amyloid positron emission tomographic scan (76.9% versus 29.8%; P<0.001). Conclusions-SS may be encountered in the general elderly population. The association with increased amyloid burden and Apolipoprotein E ϵ2 genotype supports cerebral amyloid angiopathy as the most common mechanism. Longitudinal follow-up is needed to evaluate the risk of subsequent hemorrhage in cases of incidentally discovered SS.",
keywords = "Cerebral hemorrhage, Hemosiderin, Magnetic resonance imaging, Prevalence",
author = "Michael Pichler and Prashanthi Vemuri and Rabinstein, {Alejandro A.} and Jeremiah Aakre and Flemming, {Kelly D.} and Brown, {Robert D.} and Neeraj Kumar and Kejal Kantarci and Walter Kremers and Mielke, {Michelle M.} and Knopman, {David S.} and Jack, {Clifford R.} and Petersen, {Ronald C.} and Val Lowe and Jonathan Graff-Radford",
note = "Funding Information: Dr Vemuri receives research support from the National Institues of Health (NIH)/National Institute of Aging (NIA). Dr Kremers receives research funding from NIH, Department of Defense, Agency for Healthcare Research and Quality, AstraZeneca, and Roche. Dr Mielke is a consultant for Eli Lilly and Lysosomal Therapeutics, Inc. She receives research grants from the NIH/NIA, Department of Defense, Biogen, Roche, and Lundbeck. Dr Knopman serves on a Data Safety Monitoring Board for Lundbeck Pharmaceuticals and for the DIAN study (Dominantly Inherited Alzheimer Network); is an investigator in clinical trials sponsored by Biogen, TauRX Pharmaceuticals, Lilly Pharmaceuticals, and the Alzheimer{\textquoteright}s Disease Cooperative Study; and receives research support from the NIH. Dr Jack receives research support from the NIH/NIA, and the Alexander Family Alzheimer{\textquoteright}s Disease Research Professorship of the Mayo Foundation. Dr Petersen serves on data monitoring committees for Pfizer, Inc, Janssen Alzheimer Immunotherapy; is a consultant for Biogen, Roche, Inc, Merck, Inc, and Genentech, Inc; receives publishing royalties from Mild Cognitive Impairment (Oxford University Press, 2003); and receives research support from the National Institute of Health. Dr Graff-Radford is supported by NIH/NIA, the Mayo Clinic Myron and Jane Hanley Career Development Award in Stroke Research. The other authors report no conflicts. Funding Information: Award Number K76AG057015 (PI: Dr Graff-Radford) and NIH grants R01 NS097495 (PI: Dr Vemuri), U01 AG06786 (PI: Dr Petersen), P50 AG16574 (PI: Dr Petersen), R01 AG034676 (PI: Dr Rocca), R01 AG11378 (PI: Dr Jack), R01 AG041851 (PIs: Drs Jack and Knopman); the Gerald and Henrietta Rauenhorst Foundation grant, the Alexander Family Alzheimer{\textquoteright}s Disease Research Professorship of the Mayo Foundation, and the Elsie and Marvin Dekelboum Family Foundation. The funding sources were not involved in the article review or approval. MCSA (Mayo Clinic Study of Aging) data were as follows: U01 AG006786: (Dr Petersen) Mayo Clinic Study of Aging (MCSA); P50 AG016574: (Dr Petersen) Alzheimer{\textquoteright}s Disease Research Center (ADRC); R01 AG034676: (Dr Rocca) Rochester Epidemiology Project (REP); R01 AG011378: (Dr Jack) Evaluating and Extending Our Hypothetical Model of Alzheimer{\textquoteright}s Biomarkers; R01 AG041851: (Drs Jack/Knopman) Validating the New Criteria for Preclinical Alzheimer{\textquoteright}s disease; and R01 NS097495: (Dr Vemuri) Development, Validation, and Application of an Imaging-based CVD Scale. Publisher Copyright: {\textcopyright} 2017 American Heart Association, Inc.",
year = "2017",
doi = "10.1161/STROKEAHA.117.018974",
language = "English (US)",
volume = "48",
pages = "3210--3214",
journal = "Stroke",
issn = "0039-2499",
publisher = "Lippincott Williams and Wilkins",
number = "12",
}