TY - JOUR
T1 - Prevalence and Natural History of Superficial Siderosis
T2 - A Population-Based Study
AU - Pichler, Michael
AU - Vemuri, Prashanthi D
AU - Rabinstein, Alejandro
AU - Aakre, Jeremiah
AU - Flemming, Kelly
AU - Brown, Robert D Jr.
AU - Kumar, Neeraj
AU - Kantarci, Kejal M
AU - Kremers, Walter K
AU - Mielke, Michelle M
AU - Knopman, David S
AU - Jack, Clifford R Jr.
AU - Petersen, Ronald Carl
AU - Lowe, Val
AU - Graff-Radford, Jonathan
PY - 2017/12/1
Y1 - 2017/12/1
N2 - 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.
AB - 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.
KW - cerebral hemorrhage
KW - hemosiderin
KW - magnetic resonance imaging
KW - prevalence
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U2 - 10.1161/STROKEAHA.117.018974
DO - 10.1161/STROKEAHA.117.018974
M3 - Article
C2 - 29070715
AN - SCOPUS:85038410666
VL - 48
SP - 3210
EP - 3214
JO - Stroke
JF - Stroke
SN - 0039-2499
IS - 12
ER -