Investigation of hypertensive arteriopathy-related and cerebral amyloid angiopathy-related small vessel disease scores in patients from a memory clinic: a prospective single-centre study

ObjectiveThe severity of cerebral small vessel disease (SVD) is assessed through neuroimaging findings, including hypertensive arteriopathy (HA)-SVD and cerebral amyloid angiopathy (CAA)-SVD. HA-SVD and CAA-SVD have been collectively estimated as total scores: the HA-SVD and CAA-SVD scores, respectively. Previous reports suggest that HA-SVD scores are associated with cognitive function; however, the relationship between CAA-SVD scores and cognitive function remains unclear. Therefore, we examined the association between CAA-SVD scores and cognitive function. Furthermore, we developed a modified CAA-SVD score considering cortical microinfarcts and posterior dominant white matter hyperintensities, which are imaging findings of CAA, and examined the association between these scores and cognitive function in the same patient group.DesignProspective study.SettingSingle centre study from a memory clinic.ParticipantsSubjects were diagnosed with mild cognitive impairment (MCI) or mild dementia in our memory clinic between February 2017 and July 2019 and underwent clinical dementia rating scale and brain MRI assessment. A total of 42 patients (aged 75.3±9.12 years) were registered prospectively.Primary and secondary outcome measuresWe evaluated intellectual function, memory, frontal lobe function and constructional ability. Furthermore, the relationship between each score and cognitive function was examined.ResultsThe CAA-SVD score showed significant associations with cognitive function (R2=0.63, p=0.016), but the HA-SVD score did not (R2=0.41, p=0.35). The modified CAA-SVD score was also significantly associated with cognitive function (R2=0.65, p=0.008).ConclusionCognitive function is associated with the CAA-SVD score, and more efficiently with the modified CAA-SVD score, in memory clinic patients. Although we have not validated the weighting of the modified CAA-SVD score, these scores can be a predictor of cognitive deterioration in patients with MCI and mild dementia.

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Distribution of lacunes in cerebral amyloid angiopathy and hypertensive small vessel disease

Neurology ◽

10.1212/wnl.0000000000004007 ◽

2017 ◽

Vol 88 (23) ◽

pp. 2162-2168 ◽

Cited By ~ 43

Author(s):

Marco Pasi ◽

Gregoire Boulouis ◽

Panagiotis Fotiadis ◽

Eitan Auriel ◽

Andreas Charidimou ◽

...

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Small Vessel Disease ◽

Small Vessel ◽

White Matter Hyperintensity ◽

Amyloid Angiopathy ◽

Vessel Disease ◽

Close Relationship ◽

Cerebral Amyloid ◽

Highly Correlated ◽

The Relationship

Objective:To evaluate whether the burden of deep and lobar lacunes differs between patients with intracerebral hemorrhage (ICH) with definite/probable cerebral amyloid angiopathy (CAA) per the Boston criteria and hypertensive small vessel disease (HTN-SVD; ICH in basal ganglia, thalami, brainstem).Methods:We defined lobar and deep lacunes similar to the topographic distribution used for ICH and cerebral microbleeds (CMBs). We then compared their distribution between patients with CAA-ICH and those with strictly deep CMB and ICH (HTN-ICH). The independent associations of lacune location with the diagnosis of CAA-ICH and HTN-ICH were evaluated with multivariable models. The relationship between lobar lacunes and white matter hyperintensity (WMH) volume was evaluated by means of partial correlation analyses adjusted for age and a validated visual scale.Results:In our final cohort of 316 patients with ICH, lacunes were frequent (24.7%), with similar rates in 191 patients with CAA and 125 with HTN-ICH (23% vs 27.2%, p = 0.4). Lobar lacunes were more commonly present in CAA (20.4% vs 5.7%, p < 0.001), while deep lacunes were more frequent in HTN-ICH (15.2% vs 2.1%, p < 0.001). After correction for demographics and clinical and neuroimaging markers of SVD, lobar lacunes were associated with CAA (p = 0.003) and deep lacunes with HTN-ICH (p < 0.001). Lobar lacunes in 80% of the cases were at least in contact with WMH, and after adjustment for age, they were highly correlated to WMH volume (r = 0.42, p < 0.001).Conclusions:Lobar lacunes are associated with CAA, whereas deep lacunes are more frequent in HTN-SVD. Lobar lacunes seem to have a close relationship with WMH, suggesting a possible common origin.

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Abstract 3208: Sensitivity and Reliability of SWI Compared to T2* GRE MRI for Detection of Microbleeds in Cerebral Amyloid Angiopathy

Stroke ◽

10.1161/str.43.suppl_1.a3208 ◽

2012 ◽

Vol 43 (suppl_1) ◽

Author(s):

Ah-Ling Cheng ◽

Cheryl R McCreary ◽

M. L Lauzon ◽

Richard Frayne ◽

Mayank Goyal ◽

...

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Small Vessel Disease ◽

Intraclass Correlation ◽

Case Series ◽

Cerebral Small Vessel Disease ◽

Small Vessel ◽

Amyloid Angiopathy ◽

Healthy Controls ◽

Vessel Disease ◽

Cerebral Amyloid

Introduction: Case examples and small case series suggest that MRI susceptibility weighted imaging (SWI) may be more sensitive for cerebral microbleed (CMB) detection compared to MRI T2* gradient-recalled echo (GRE). However, there are few data on CMB counts measured by SWI vs. GRE, or inter-rater reliability, in groups of patients with cerebral small vessel disease. We used data from a prospective cohort study of cerebral amyloid angiopathy (CAA), a cerebral small-vessel disease marked by high numbers of CMBs, to quantify the sensitivity and reliability of SWI vs. GRE for CMB detection. Methods: Nine patients with symptomatic CAA (mean age 71±8.3; 7 males and 2 females) and 21 healthy non-CAA controls (mean age 68±6.3; 10 M/11 F) underwent T2* GRE and SWI on a 3.0T MR scanner. Probable CAA was diagnosed according to the Boston criteria prior to study entry using information from clinical MRI with GRE sequences. Two raters (labeled 1 and 2) independently interpreted the GRE and SWI scans blinded to clinical information. The phase-filtered magnitude image was used for SWI interpretation. Agreement reliability was assessed using the kappa coefficient (where a kappa of ≥0.60 indicates good agreement) or the intraclass correlation coefficient (ICC). Results: Overall, the raters identified 1,432 CMBs in the 9 CAA cases (range 1-434 per patient) and 8 CMBs in the healthy controls (range 0-3). Rater 1 identified CMBs in 5/21 healthy controls on SWI and 5/21 on GRE, while rater 2 identified CMBs in 4/21 on SWI and 3/21 on GRE (kappa 0.70 for GRE and 0.57 for SWI). In CAA cases more CMBs were seen on SWI compared to the GRE sequence but the difference was significant only for rater 1 (rater 1: on average 85% more per patient on SWI than on GRE, p=0.008; rater 2: 19% more, p=0.25). Among CAA cases the reliability between raters was poor for GRE (ICC 0.36) but excellent for SWI (0.94, p<0.05 for comparison with GRE). Review suggested that the differing reliability was because rater 1 was less likely than rater 2 to identify faint lesions on GRE as CMB, whereas these lesions were more conspicuous on SWI. If SWI rather than GRE were used to determine CAA status according to the Boston criteria, all 9 CAA cases would remain classified as probable CAA but 2/21 controls would be reclassified as either possible (n=1) or probable (n=1) asymptomatic CAA based on the detection of one or more lobar microbleeds on SWI. Conclusions: SWI confers greater reliability as well as greater sensitivity for CMB detection compared to GRE, and should be the preferred sequence for quantifying CMBs. SWI may more frequently identify lobar microbleeds that could represent asymptomatic CAA. Further research is needed to determine whether the Boston criteria require revision to take into account the greater sensitivity of SWI for CMB detection.

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Small vessel disease burden in cerebral amyloid angiopathy without symptomatic hemorrhage

Neurology ◽

10.1212/wnl.0000000000003655 ◽

2017 ◽

Vol 88 (9) ◽

pp. 878-884 ◽

Cited By ~ 16

Author(s):

Gregoire Boulouis ◽

Andreas Charidimou ◽

Michael J. Jessel ◽

Li Xiong ◽

Duangnapa Roongpiboonsopit ◽

...

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Small Vessel Disease ◽

Summary Score ◽

Small Vessel ◽

Amyloid Angiopathy ◽

Perivascular Spaces ◽

Cognitive Symptoms ◽

Vessel Disease ◽

Age Related ◽

Cerebral Amyloid

Objective:Cerebral amyloid angiopathy (CAA) is a common age-related small vessel disease (SVD). Patients without intracerebral hemorrhage (ICH) typically present with transient focal neurologic episodes (TFNEs) or cognitive symptoms. We sought to determine if SVD lesion burden differed between patients with CAA first presenting with TFNEs vs cognitive symptoms.Methods:A total of 647 patients presenting either to a stroke department (n = 205) or an outpatient memory clinic (n = 442) were screened for eligibility. Patients meeting modified Boston criteria for probable CAA were included and markers of SVD were quantified, including cerebral microbleeds (CMBs), perivascular spaces, cortical superficial siderosis (cSS), and white matter hyperintensities (WMHs). Patients were classified according to presentation symptoms (TFNEs vs cognitive). Total CAA-SVD burden was assessed using a validated summary score. Individual neuroimaging markers and total SVD burden were compared between groups using univariable and multivariable models.Results:There were 261 patients with probable CAA included. After adjustment for confounders, patients first seen for TFNEs (n = 97) demonstrated a higher prevalence of cSS (p < 0.0001), higher WMH volumes (p = 0.03), and a trend toward higher CMB counts (p = 0.09). The total SVD summary score was higher in patients seen for TFNEs (adjusted odds ratio per additional score point 1.46, 95% confidence interval 1.16–1.84, p = 0.013).Conclusions:Patients with probable CAA without ICH first evaluated for TFNEs bear a higher burden of structural MRI SVD-related damage compared to those first seen for cognitive symptoms. This study sheds light on neuroimaging profile differences across clinical phenotypes of patients with CAA without ICH.

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Total small vessel disease burden and brain network efficiency in cerebral amyloid angiopathy

Journal of the Neurological Sciences ◽

10.1016/j.jns.2017.09.015 ◽

2017 ◽

Vol 382 ◽

pp. 10-12 ◽

Cited By ~ 8

Author(s):

Raffaella Valenti ◽

Yael D. Reijmer ◽

Andreas Charidimou ◽

Gregoire Boulouis ◽

Sergi Ramirez Martinez ◽

...

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Disease Burden ◽

Small Vessel Disease ◽

Brain Network ◽

Small Vessel ◽

Amyloid Angiopathy ◽

Network Efficiency ◽

Vessel Disease ◽

Cerebral Amyloid

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Amyloid Depositions and Small Vessel Disease in Patients with Cerebral Amyloid Angiopathy: a Case Series

Dementia and Neurocognitive Disorders ◽

10.12779/dnd.2020.19.2.74 ◽

2020 ◽

Vol 19 (2) ◽

pp. 74

Author(s):

Yun Jeong Hong ◽

Si Baek Lee ◽

Seong Hoon Kim ◽

Dong Woo Ryu ◽

Yongbang Kim ◽

...

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Small Vessel Disease ◽

Case Series ◽

Small Vessel ◽

Amyloid Angiopathy ◽

Vessel Disease ◽

Cerebral Amyloid

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Interhemispheric distribution of amyloid and small vessel disease burden in cerebral amyloid angiopathy‐related intracerebral hemorrhage

European Journal of Neurology ◽

10.1111/ene.14301 ◽

2020 ◽

Vol 27 (8) ◽

pp. 1664-1671

Author(s):

M. Planton ◽

J. Pariente ◽

F. Nemmi ◽

J‐F. Albucher ◽

L. Calviere ◽

...

Keyword(s):

Intracerebral Hemorrhage ◽

Cerebral Amyloid Angiopathy ◽

Disease Burden ◽

Small Vessel Disease ◽

Small Vessel ◽

Amyloid Angiopathy ◽

Vessel Disease ◽

Cerebral Amyloid

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Prevention of Cerebral Small Vessel Disease

Seminars in Neurology ◽

10.1055/s-0037-1603468 ◽

2017 ◽

Vol 37 (03) ◽

pp. 316-325 ◽

Cited By ~ 1

Author(s):

Eric Smith

Keyword(s):

Ischemic Stroke ◽

Intracerebral Hemorrhage ◽

Cerebral Amyloid Angiopathy ◽

Small Vessel Disease ◽

Unmet Need ◽

Cerebral Small Vessel Disease ◽

Small Vessel ◽

Amyloid Angiopathy ◽

Vessel Disease ◽

Cerebral Amyloid

AbstractCerebral small vessel disease can cause either ischemic stroke or intracerebral hemorrhage. Accounting for up to 25% of all strokes, it is also the second biggest contributor to the risk of dementia, and is the most common incidentally discovered finding on brain imaging. There are two main causes of cerebral small vessel disease: arteriolosclerotic small vessel disease (with hypertension as the main modifiable risk factor) and cerebral amyloid angiopathy (predominantly caused by β-amyloid deposits limited to the cerebral small arteries, arterioles, and capillaries). Prevention should include the treatment of hypertension and diabetes, if present, and the modification of lifestyle factors such as obesity and poor nutrition. Patients with small subcortical ischemic strokes should be treated with antithrombotics; dual antiplatelet therapy may be more effective than aspirin for the first 3 weeks following acute stroke, but is not more effective than aspirin for long-term prevention. Unresolved questions include the effectiveness of nonaspirin prevention strategies to prevent early recurrence or stroke extension in small subcortical ischemic stroke, and whether symptomatic or silent small vessel disease should influence decisions regarding selection for carotid revascularization or anticoagulation for atrial fibrillation. There is an unmet need for disease-modifying preventive therapies for cerebral amyloid angiopathy, the second most-common cause of spontaneous intracerebral hemorrhage.

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Abstract TMP95: Small Vessel Disease Burden and Clinical Symptoms in Cerebral Amyloid Angiopathy Patients Presenting Without Symptomatic Hemorrhage

Stroke ◽

10.1161/str.48.suppl_1.tmp95 ◽

2017 ◽

Vol 48 (suppl_1) ◽

Author(s):

Gregoire Boulouis ◽

Andreas Charidimou ◽

Michael J Jessel ◽

Li Xiong ◽

Duangnapa Roongpiboonsopit ◽

...

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Small Vessel Disease ◽

Summary Score ◽

Small Vessel ◽

Amyloid Angiopathy ◽

Perivascular Spaces ◽

Cognitive Symptoms ◽

Vessel Disease ◽

Age Related ◽

Cerebral Amyloid

Introduction: Cerebral amyloid angiopathy (CAA) is a common age-related small vessel disease (SVD). Patients without ICH typically present with transient focal neurological episodes (TFNEs) or cognitive symptoms. We sought to determine if SVD lesion burden differed between CAA patients first presenting with TFNEs versus cognitive symptoms. Methods: A total of 647 patients presenting either to a stroke department (n=205) or an outpatient memory clinic (n=442), were screened for eligibility. Patients meeting modified Boston criteria for probable CAA were included and markers of SVD were quantified including cerebral microbleeds (CMBs), perivascular spaces, cortical superficial siderosis (cSS), and white matter hyperintensities (WMH). Patients were classified according to presentation symptoms (TFNEs vs cognitive). Total CAA-SVD burden was assessed using a validated summary score. Individual neuroimaging markers and total SVD burden were compared between groups using univariable and multivariable models. Results: There were 261 probable CAA patients included. After adjustment for confounders, patients first seen for TFNEs (n=97) demonstrated a higher prevalence of cSS (p<.0001), higher WMH volumes (p=0.03) and a trend towards higher CMBs counts (p=.09). The total SVD summary score was higher in patients seen for TFNEs (adjusted OR per additional score point=1.46, 95%CI [1.16 - 1.84], p=0.013). Conclusion: We present a large cohort of probable CAA patients without ICH and show that those first evaluated for TFNEs bear a higher burden of structural MRI SVD related damage compared to those first seen for cognitive symptoms. This study sheds light on CAA disease phenotypes, adds to the understanding of CAA clinical expression, and sets the basis for future works investigating CAA prognosis amongst phenotypes.

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