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Background and Purpose: Data on health‐related quality of life (HRQoL) and mood in cerebral amyloid angiopathy (CAA), a disease characterized by stroke and cognitive decline, are limited. We aimed to investigate the impacted domains of life, value‐based HRQoL and the prevalence of depression and anxiety in patients with CAA. Methods: We conducted a cross‐sectional study of patients with sporadic (s)CAA, lobar dominant mixed CAA and hypertensive arteriopathy (mixed CAA‐HTA), or Dutch‐type hereditary (D‐)CAA, from prospective outpatient clinic cohorts. Participants completed four questionnaires: the EuroQoL 5 dimensions 5‐level questionnaire (EQ‐5D‐5L; EQ‐VAS for visual analogue scale; EQ‐Index for index rating), the Short‐Form 36 questionnaire (SF‐36), the Center for Epidemiologic Studies—Depression scale (CES‐D), and the Hospital Anxiety and Depression Scale (HADS; ‐D for depression and ‐A for anxiety subscales). The EQ‐5D‐5L assesses the domains mobility, self‐care, usual activities, pain/discomfort and anxiety/depression. The SF‐36 domains are physical functioning, social functioning, physical role limitations, emotional role limitations, mental health, vitality, bodily pain, and general health perceptions. We compared age‐ and sex‐ adjusted HRQoL (SF‐36 domain scores; EQ‐VAS; EQ‐Index) to the Dutch normative population, and estimated the prevalences of current depression (either: history of depression or current use of antidepressants, with high score on CES‐D [≥16] and/or HADS‐D [≥8]; or high score on both depression questionnaires) and anxiety (HADS‐A ≥ 8). Results: We included 179 patients: 77 with sCAA (mean age: 72 years, women: 36%), 31 with mixed CAA‐HTA (68 years, women: 29%), and 71 with D‐CAA (56 years, women: 52%, symptomatic: 35 [49%]). The SF‐36 profiles of all patient groups were similar, negatively differing from the norm in emotional role functioning, social functioning and vitality. The EQ‐VAS score of patients (mean [SD] sCAA: 76 [16], D‐CAA: 77 [15]) was similar to the norm, as was the EQ‐Index score. Fifteen patients with sCAA (23%; 95% confidence interval [CI] 13%–33%), seven with mixed CAA‐HTA (27%; 95% CI 10%–44%) and eight with D‐CAA (14%; 95% CI 5%–22%) were noted as having depression. The prevalences of anxiety and depression were equivalent. Conclusions: We found that CAA influenced emotional role functioning and aspects linked to social engagement consistently across its subtypes. One quarter of patients exhibited depressive or anxiety symptoms. Recognizing these impacted domains could enhance overall well‐being. [ABSTRACT FROM AUTHOR]

Introduction: Cerebral amyloid angiopathy (CAA) has a remarkably variable disease course, even in monogenetic hereditary forms. Our aim was to investigate the prevalence of vascular risk factors and their effect on disease onset and course in Dutch-type hereditary (D-)CAA and sporadic CAA. Methods: We performed a cohort study in D-CAA to investigate the association between vascular risk factors (hypertension, hypercholesterolemia, smoking, and alcohol use) and age of intracerebral hemorrhage (ICH) onset and time of ICH recurrence with survival analyses. In addition, we performed a systematic review to assess the prevalence of vascular risk factors and their effect on clinical outcome in sporadic CAA. We searched PubMed, Embase, Web of Science, and Cochrane Library from 1987 to 2022 and included cohorts with ≥10 patients. We created forest plots, calculated pooled estimates, and reported variability (heterogeneity plus sampling variability) and risk of bias. Results: We included 70 participants with D-CAA (47% women, mean age 53 years). Sixteen (23%) had hypertension, 15 (21%) had hypercholesterolemia, 45 (64%) were smokers, and 61 (87%) used alcohol. We found no clear effect of vascular risk factors on age of first ICH (log-rank test hypertension: p = 0.35, hypercholesterolemia: p = 0.41, smoking: p = 0.61, and alcohol use: p = 0.55) or time until ICH recurrence (log-rank test hypertension: p = 0.71, hypercholesterolemia: p = 0.20, and smoking: p = 0.71). We identified 25 out of 1,234 screened papers that assessed the prevalence of risk factors in CAA and 6 that reported clinical outcomes. The pooled prevalence estimates of hypertension was 62% (95% CI: 55–69%), diabetes was 17% (95% CI: 14–20%), dyslipidemia was 32% (95% CI: 23–41%), and tobacco use was 27% (95% CI: 18–36%). One study reported study diabetes and hypertension to be associated with a lower risk of recurrent ICH, whereas another study reported hypertension to be associated with an increased risk. All other studies showed no association between vascular risk factors and clinical outcome. High-quality studies focusing on vascular risk factors were lacking. Conclusion: In patients with D-CAA and sporadic CAA, the prevalence of vascular risk factors is high. Although this suggests an opportunity for prevention, there is no clear association between these risk factors and CAA-related ICH onset and recurrence. [ABSTRACT FROM AUTHOR]

Peak width of skeletonized mean diffusivity (PSMD) is an emerging diffusion-MRI based marker to study subtle early alterations to white matter microstructure. We assessed PSMD over the clinical continuum in Dutch-type hereditary CAA (D-CAA) and its association with other CAA-related MRI-markers and cognitive symptoms. We included (pre)symptomatic D-CAA mutation-carriers and calculated PSMD from diffusion-MRI data. Associations between PSMD-levels, cognitive performance and CAA-related MRI-markers were assessed with linear regression models. We included 59 participants (25/34 presymptomatic/symptomatic; mean age 39/58 y). PSMD-levels increased with disease severity and were higher in symptomatic D-CAA mutation-carriers (median [range] 4.90 [2.77–9.50]mm2/s × 10−4) compared with presymptomatic mutation-carriers (2.62 [1.96–3.43]mm2/s × 10−4) p = <0.001. PSMD was positively correlated with age, CAA-SVD burden on MRI (adj.B [confidence interval] = 0.42 [0.16–0.67], p = 0.002), with number of cerebral microbleeds (adj.B = 0.30 [0.08–0.53], p = 0.009), and with both deep (adj.B = 0.46 [0.22–0.69], p = <0.001) and periventricular (adj.B = 0.38 [0.13–0.62], p = 0.004) white matter hyperintensities. Increasing PSMD was associated with decreasing Trail Making Test (TMT)-A performance (B = −0.42 [−0.69–0.14], p = 0.04. In D-CAA mutation-carriers microstructural white matter damage is associated with disease phase, CAA burden on MRI and cognitive impairment as reflected by a decrease in information processing speed. PSMD, as a global measure of alterations to the white matter microstructure, may be a useful tool to monitor disease progression in CAA. [ABSTRACT FROM AUTHOR]