Superficial siderosis in the general population

Vernooij, Meike W.; Ikram, M. Arfan; Hofman, Albert; Krestin, Gabriël P.; Breteler, Monique M.B.; Lugt, Aad van der

doi:10.1212/wnl.0b013e3181ae7c5e

Cited by 115 publications

(94 citation statements)

References 17 publications

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“…10 In agreement with our findings, the mean age of persons with cSS was higher than in those without cSS (mean age 79.9 vs 69.6 years; p , 0.001), and also compared with persons who had CMBs but no cSS (mean age 71.8 years). 10 Because the severity of CAA is age-related, 1,3 these findings, together with our data showing an association of cSS with chronic ICH, suggest that cSS might be a marker Table 2 Characteristics The p values refer to differences between CAA patients with vs without cSS, using x 2 tests and the Fisher exact test for categorical variables, and 2-sample t tests or Mann-Whitney U tests depending on the distribution of continuous variables. b There were 10 patients with missing data for one or more of these variables: hypertension (n 5 9), taking antithrombotics (n 5 8), and history of symptomatic ICH (n 5 7).…”

Section: Resultssupporting

confidence: 91%

“…[5][6][7][8] In CAA, cSS has a characteristic predilection for the cerebral convexities, reflecting linear blood residues in the superficial layers of the cerebral cortex or in the subarachnoid space. [8][9][10] cSS may have clinical relevance as an important cause of transient focal neurologic episodes (sometimes called "amyloid spells"), 11,12 and a potential "warning sign" for future symptomatic ICH. 13 Although cSS is a promising diagnostic neuroimaging marker of CAA, 8 the strength of the association and underlying mechanisms have not been systematically studied.…”

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confidence: 99%

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Prevalence and mechanisms of cortical superficial siderosis in cerebral amyloid angiopathy

et al. 2013

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Objective: We investigated the prevalence and clinical-radiologic associations of cortical superficial siderosis (cSS) in patients with probable cerebral amyloid angiopathy (CAA) compared to those with intracerebral hemorrhage (ICH) not attributed to CAA. Methods:We conducted a retrospective multicenter cohort study of 120 patients with probable CAA and 2 comparison groups: 67 patients with either single lobar ICH or mixed (deep and lobar) hemorrhages; and 22 patients with strictly deep hemorrhages. We rated cSS, ICH, white matter changes, and cerebral microbleeds.

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Section: Resultssupporting

confidence: 91%

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confidence: 99%

Prevalence and mechanisms of cortical superficial siderosis in cerebral amyloid angiopathy

et al. 2013

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“…2,24 One study reported cSS in 60.5% (n 5 38; mean age 70 6 6.4 years) of patients with histopathologically proven CAA, compared with no control subjects with histopathologically proven non-CAA ICH (n 5 22; mean age 54 6 18 years). 13 Another recent study found cSS in 40% of patients with a clinic-radiologic diagnosis of probable CAArelated ICH, but only 5% of patients with purely deep ICH, presumed to be due to hypertensive arteriopathy.…”

Section: Resultsmentioning

confidence: 99%

Cortical superficial siderosis and intracerebral hemorrhage risk in cerebral amyloid angiopathy

et al. 2013

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Objective: To investigate whether cortical superficial siderosis (cSS) on MRI, especially if disseminated (involving more than 3 sulci), increases the risk of future symptomatic lobar intracerebral hemorrhage (ICH) in cerebral amyloid angiopathy (CAA).Methods: European multicenter cohort study of 118 patients with CAA (104 with baseline symptomatic lobar ICH) diagnosed according to the Boston criteria. We obtained baseline clinical, MRI, and follow-up data on symptomatic lobar ICH. Using Kaplan-Meier and Cox regression analyses, we investigated cSS and ICH risk, adjusting for known confounders.Results: During a median follow-up time of 24 months (interquartile range 9-44 months), 23 of 118 patients (19.5%, 95% confidence interval [CI]: 12.8%-27.8%) experienced symptomatic lobar ICH. Any cSS and disseminated cSS were predictors of time until first or recurrent ICH (logrank test: p 5 0.0045 and p 5 0.0009, respectively). ICH risk at 4 years was 25% (95% CI: 7.6%-28.3%) for patients without siderosis; 28.9% (95% CI: 7.7%-76.7%) for patients with focal siderosis; and 74% (95% CI: 44.1%-95.7%) for patients with disseminated cSS (log-rank test: p 5 0.0031). In Cox regression models, any cSS and disseminated cSS were both independently associated with increased lobar ICH risk, after adjusting for $2 microbleeds and age (hazard ratio: 2.53; 95% CI: 1.05-6.15; p 5 0.040 and hazard ratio: 3.16; 95% CI: 1.35-7.43; p 5 0.008, respectively). These results remained consistent in sensitivity analyses including only patients with symptomatic lobar ICH at baseline. Conclusions:Our findings indicate that cSS, particularly if disseminated, is associated with an increased risk of symptomatic lobar ICH in CAA. cSS may help stratify future bleeding risk in CAA, with implications for prognosis and treatment. Neurology ® 2013;81:1666-1673 GLOSSARY CAA 5 cerebral amyloid angiopathy; CI 5 confidence interval; CMB 5 cerebral microbleed; cSS 5 cortical superficial siderosis; FLAIR 5 fluid-attenuated inversion recovery; GRE 5 gradient-recalled echo; HR 5 hazard ratio; ICH 5 intracerebral hemorrhage.Sporadic cerebral amyloid angiopathy (CAA) is a highly prevalent, age-related small-vessel disease 1 caused by amyloid-b deposition in cortical and leptomeningeal vessel walls. 2 CAA is a major cause of lobar intracerebral hemorrhage (ICH), particularly in elderly patients.2-5 Spontaneous ICH is one of the most catastrophic forms of stroke, with a high risk of recurrence [6][7][8] ; CAA-related lobar ICH may carry a greater risk than deep ICH presumed to be due to hypertensive arteriopathy, 8,9 but this is currently difficult to predict. Predisposing factors for lobar ICH and lobar ICH recurrence in CAA include APOE e4 and e2 alleles, 10 hemorrhagic neuroimaging markers of CAA such as lobar cerebral microbleeds (CMBs), 11 and anticoagulant or antiplatelet use. 12 Little is known about cortical superficial siderosis (cSS), a recently identified neuroimaging marker of CAA, 13 and the risk of subsequent

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“…15 Since patients with RCVS often do not have angiographically evident vasoconstriction early during their clinical course, 13 we relied on a history of severe or thunderclap headache with normal conventional angiograms and laboratory studies, absence of other potential causes of bleeding, and signs of clinical reversibility at discharge or during early follow-up, for a presumptive clinical diagnosis. Similarly in the absence of pathologic confirmation, patients were diagnosed with CAA if they were Ն55 years of age, had extensive leukoaraiosis or microhemorrhages, superficial siderosis on the gradient echo MRI sequences, [16][17][18] and no other potential explanation for hemorrhage. The results are summarized in table 2.…”

Section: Recurrent Hemorrhage (Intracerebral Hemorrhage)mentioning

confidence: 99%

“…We found a high prevalence of leukoaraiosis, microbleeds, and superficial siderosis, suggestive of CAA. [16][17][18] One patient, who did not exhibit these findings, had amyloidbeta-related angiitis on necropsy.…”

Section: Recurrent Hemorrhage (Intracerebral Hemorrhage)mentioning

confidence: 99%

Atraumatic convexal subarachnoid hemorrhage

Kumar¹,

Goddeau²,

Selim³

et al. 2010

Neurology

247

146

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Objective: To identify patterns of clinical presentation, imaging findings, and etiologies in a cohort of hospitalized patients with localized nontraumatic convexal subarachnoid hemorrhage.Methods: Twenty-nine consecutive patients with atraumatic convexal subarachnoid hemorrhage were identified using International Classification of Diseases-9 code from 460 patients with subarachnoid hemorrhage evaluated at our institution over a course of 5 years. Retrospective review of patient medical records, neuroimaging studies, and follow-up data was performed.Results: There were 16 women and 13 men between the ages of 29 and 87 years. Two common patterns of presentations were observed. The most frequent presenting symptom in patients Յ60 years (n ϭ 16) was a severe headache (n ϭ 12; 75%) of abrupt onset (n ϭ 9; 56%) with arterial narrowing on conventional angiograms in 4 patients; 10 (p ϭ 0.003) were presumptively diagnosed with a primary vasoconstriction syndrome. Patients Ͼ60 years (n ϭ 13) usually had temporary sensory or motor symptoms (n ϭ 7; 54%); brain MRI scans in these patients showed evidence of leukoaraiosis and/or hemispheric microbleeds and superficial siderosis (n ϭ 9; 69%), compatible with amyloid angiopathy (n ϭ 10; p Ͻ 0.0001). In a small group of patients, the presentation was more varied and included lethargy, fever, and confusion. Four patients older than 60 years had recurrent intracerebral hemorrhages in the follow-up period with 2 fatalities. Conclusion:Convexal subarachnoid hemorrhage is an important subtype of nonaneurysmal subarachnoid bleeding with diverse etiologies, though a reversible vasoconstriction syndrome appears to be a common cause in patients 60 years or younger whereas amyloid angiopathy is frequent in patients over 60. These observations require confirmation in future studies. Atraumatic localized convexal subarachnoid hemorrhage (cSAH) is an unusual presentation of subarachnoid bleeding, in which the bleeding is localized to the convexities of the brain without involvement of the adjacent parenchyma or extension into the interhemispheric fissures, basal cisterns, or ventricles. Since most saccular aneurysms arise from the circle of Willis, aneurysmal rupture is an unlikely source of cSAH. Diverse etiologies have been posited for its occurrence, including cortical vein occlusions, posterior reversible leukoencephalopathy syndrome (PRES), reversible cerebral vasoconstriction syndrome (RCVS), coagulopathy, cocaine use, lupus vasculitis, cavernoma, brain abscesses, and cerebral amyloid angiopathy (CAA). 1-13Existing information about this condition is largely derived from case reports and small case series, which carry inherent referral and diagnostic biases. We undertook this study to systematically evaluate and review the potential causes and patterns of clinical and radiologic presentation of localized cSAH from the inpatient population at our institution.

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Superficial siderosis in the general population

Abstract: Our results provide further indirect support for the presumed link between superficial siderosis and cerebral amyloid angiopathy (CAA). Whether superficial siderosis may be a marker for severity or worse prognosis of CAA needs to be further evaluated in longitudinal follow-up.

Cited by 115 publications

References 17 publications

Prevalence and mechanisms of cortical superficial siderosis in cerebral amyloid angiopathy

Prevalence and mechanisms of cortical superficial siderosis in cerebral amyloid angiopathy

Cortical superficial siderosis and intracerebral hemorrhage risk in cerebral amyloid angiopathy

Atraumatic convexal subarachnoid hemorrhage

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