Prevalence and Natural History of Superficial Siderosis: A Population-Based Study

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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.

Original languageEnglish (US)
Pages (from-to)3210-3214
Number of pages5
JournalStroke
Volume48
Issue number12
DOIs
StatePublished - Dec 1 2017

Fingerprint

Siderosis
Population
Amyloid
Apolipoprotein E2
Magnetic Resonance Imaging
Genotype
Cerebral Amyloid Angiopathy
Confidence Intervals
Hemosiderin

Keywords

  • cerebral hemorrhage
  • hemosiderin
  • magnetic resonance imaging
  • prevalence

ASJC Scopus subject areas

  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine
  • Advanced and Specialized Nursing

Cite this

@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 Vemuri, {Prashanthi D} and Alejandro Rabinstein and Jeremiah Aakre and Kelly Flemming and Brown, {Robert D Jr.} and Neeraj Kumar and Kantarci, {Kejal M} and Kremers, {Walter K} and Mielke, {Michelle M} and Knopman, {David S} and Jack, {Clifford R Jr.} and Petersen, {Ronald Carl} and Val Lowe and Jonathan Graff-Radford",
year = "2017",
month = "12",
day = "1",
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",

}

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 -