Cortical superficial siderosis progression in cerebral amyloid angiopathy

Pongpitakmetha, Thanakit; Fotiadis, Panagiotis; Pasi, Marco; Boulouis, Grégoire; Xiong, Li; Warren, Andrew; Schwab, Kristin; Rosand, Jonathan; Gurol, M. Edip; Greenberg, Steven M.; Viswanathan, Anand; Charidimou, Andreas

doi:10.1212/wnl.0000000000009321

Cited by 21 publications

(24 citation statements)

References 45 publications

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“…The early recurrence of ICH might be on the same side of SS. These findings were consistent with the recent follow-up study [ 23 ]. This may provide additional insights into the mechanisms of ICH recurrence in patients with CAA.…”

Section: Discussionsupporting

confidence: 94%

Early MRI imaging and follow-up study in cerebral amyloid angiopathy

et al. 2021

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Aim To study the imaging features of leukoaraiosis (LA) and hemorrhage in cerebral amyloid angiopathy (CAA) patients. Methods The earliest MRI images of probable CAA patients and non-CAA patients were collected. The characteristics of LA in the two groups were analyzed. Cerebral micro bleeding (CMB), superficial siderosis (SS), and intracranial hemorrhage (ICH) were recorded in the follow-up study. The space relationship between CMB or SS and ICH was assessed. Results We found that 10/21 (47.6%) patients had occipital prominent LA and 14/21 (66.7%) patients had subcortical punctate LA before the ICH, which was higher than that of the ones in the control group (p = 0.015 and 0.038, respectively). The recurrence rate of ICH was 100% (3/3) in patients with diffuse SS and 36.4% (4/11) in patients without. The recurrence rate of ICH was 60% (3/5) in patients with multiple-lobe CMBs and 44.4% (4/9) in those without. The location of the ICH and CMB was inconsistent. ICH occurred in the ipsilateral cerebral hemisphere of SS in three patients with diffuse SS. Conclusion LA, diffuse SS, and multiple-lobe CMBs are important imaging characteristics of CAA, which may help make early diagnosis and predict the recurrence of ICH.

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Section: Discussionsupporting

confidence: 94%

Early MRI imaging and follow-up study in cerebral amyloid angiopathy

et al. 2021

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“…Lastly, we did not assess cSS progression over time, which has been reported to occur in $30% of patients with CAA. 27,28 Our study adds supporting evidence to the association between cSS and CAA, and suggests that cSS may be a potent MRI marker for CAA in the general population. Community-dwelling older persons with cSS may be at higher risk for ICH and future neurological events.…”

Section: Discussionsupporting

confidence: 68%

Cortical superficial siderosis in the general population: The Framingham Heart and Rotterdam studies

Shoamanesh

Akoudad

Himali

et al. 2021

International Journal of Stroke

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Objective We aimed to characterize cortical superficial siderosis, its determinants and sequel, in community-dwelling older adults. Methods The sample consisted of Framingham ( n = 1724; 2000–2009) and Rotterdam ( n = 4325; 2005–2013) study participants who underwent brain MRI. In pooled individual-level analysis, we compared baseline characteristics in patients with cortical superficial siderosis to two reference groups: (i) persons without hemorrhagic MRI markers of cerebral amyloid angiopathy (no cortical superficial siderosis and no microbleeds) and (ii) those with presumed cerebral amyloid angiopathy based on the presence of strictly lobar microbleeds but without cortical superficial siderosis. Results Among a total of 6049 participants, 4846 did not have any microbleeds or cortical superficial siderosis (80%), 401 had deep/mixed microbleeds (6.6%), 776 had strictly lobar microbleeds without cortical superficial siderosis (12.8%) and 26 had cortical superficial siderosis with/without microbleeds (0.43%). In comparison to participants without microbleeds or cortical superficial siderosis and to those with strictly lobar microbleeds but without cortical superficial siderosis, participants with cortical superficial siderosis were older (OR 1.09 per year, 95% CI 1.05, 1.14; p < 0.001 and 1.04, 95% CI 1.00, 1.09; p = 0.058, respectively), had overrepresentation of the APOE ɛ4 allele (5.19, 2.04, 13.25; p = 0.001 and 3.47, 1.35, 8.92; p = 0.01), and greater prevalence of intracerebral hemorrhage (72.57, 9.12, 577.49; p < 0.001 and 81.49, 3.40, >999.99; p = 0.006). During a mean follow-up of 5.6 years, 42.4% participants with cortical superficial siderosis had a stroke (five intracerebral hemorrhage, two ischemic strokes and four undetermined strokes), 19.2% had transient neurological deficits and 3.8% developed incident dementia. Conclusion Our study adds supporting evidence to the association between cortical superficial siderosis and cerebral amyloid angiopathy within the general population. Community-dwelling persons with cortical superficial siderosis may be at high risk for intracerebral hemorrhage and future neurological events.

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“…Cortical siderosis, microbleeds, LH, and WMH are MRI findings needed to diagnose CAA-RI [ 7 , 8 ]. All of them were observed in our patients (Fig.…”

Section: Discussionmentioning

confidence: 99%

Clinical and radiological features of cerebral amyloid angiopathy-related inflammation

2021

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Objectives We want to report the clinical and radiological features of our cohort of patients diagnosed with cerebral amyloid angiopathy-related inflammation (CAA-RI) according to the Boston Criteria and additionally to disclose some atypical clinical characteristics observed in some of them to provide more knowledge about this novel entity. Methods We describe 5 patients with probable CAA-RI according to a validation study of proposed criteria for the diagnosis of CAA-RI at University Hospital Josep Trueta of Girona. We consider some clinical characteristics which include the response to immunotherapy, CSF findings, and MRI features. The patient’s neurologic outcomes were assessed using the modified Rankin Scale (mRS). Results We collected 5 patients admitted for probable CAA-RI. Most were women and the median age was 72 years. The median mRS score at the onset of disease was 1. Parietal lobes were most affected clinically as well as radiologically. Two patients had intracranial hemorrhage. Decreased levels of CSF amyloid beta 42 and 40 protein were observed. Corticosteroids were used in four patients and a remarkable improvement was observed in all of them. Conclusions CAA-RI is a condition that predominantly affects parietal lobes according to our case series and this involvement seems to be directly related to a greater burden of microbleeds, cortical siderosis, WMH, and lobar hemorrhages on these lobes. Decreased levels of CSF amyloid beta protein plus increased total tau protein should be considered as part of the diagnostic criteria of CAA-RI. We recommend corticosteroids using, as they have been demonstrated to be very effective in managing CAA-RI.

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Cortical superficial siderosis progression in cerebral amyloid angiopathy

Cited by 21 publications

References 45 publications

Early MRI imaging and follow-up study in cerebral amyloid angiopathy

Early MRI imaging and follow-up study in cerebral amyloid angiopathy

Cortical superficial siderosis in the general population: The Framingham Heart and Rotterdam studies

Clinical and radiological features of cerebral amyloid angiopathy-related inflammation

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