A single, high-quality computed tomography angiography is the preferred diagnostic strategy. Short-term complications are rare and often transient. Long-term outcome is excellent with respect to disability and death, but high-quality studies focused at neuropsychological sequelae are needed.
Background and Purpose: In previous studies, women had a higher risk of rupture of intracranial aneurysms than men, but female sex was not an independent risk factor. This may be explained by a higher prevalence of patient- or aneurysm-related risk factors for rupture in women than in men or by insufficient power of previous studies. We assessed sex differences in rupture rate taking into account other patient- and aneurysm-related risk factors for aneurysmal rupture. Methods: We searched Embase and Pubmed for articles published until December 1, 2020. Cohorts with available individual patient data were included in our meta-analysis. We compared rupture rates of women versus men using a Cox proportional hazard regression model adjusted for the PHASES score (Population, Hypertension, Age, Size of Aneurysm, Earlier Subarachnoid Hemorrhage From Another Aneurysm, Site of Aneurysm), smoking, and a positive family history of aneurysmal subarachnoid hemorrhage. Results: We pooled individual patient data from 9 cohorts totaling 9940 patients (6555 women, 66%) with 12 193 unruptured intracranial aneurysms, and 24 357 person-years follow-up. Rupture occurred in 163 women (rupture rate 1.04%/person-years [95% CI, 0.89–1.21]) and 63 men (rupture rate 0.74%/person-years [95% CI, 0.58–0.94]). Women were older (61.9 versus 59.5 years), were less often smokers (20% versus 44%), more often had internal carotid artery aneurysms (24% versus 17%), and larger sized aneurysms (≥7 mm, 24% versus 23%) than men. The unadjusted women-to-men hazard ratio was 1.43 (95% CI, 1.07–1.93) and the adjusted women/men ratio was 1.39 (95% CI, 1.02–1.90). Conclusions: Women have a higher risk of aneurysmal rupture than men and this sex difference is not explained by differences in patient- and aneurysm-related risk factors for aneurysmal rupture. Future studies should focus on the factors explaining the higher risk of aneurysmal rupture in women.
Background and Purpose A much higher rupture rate for patients with familial intracranial aneurysms (IA) compared with patients with sporadic IA has been reported in a study with highly selected familial aneurysms using sporadic patients from other populations a controls. We aimed to validate these findings in a large independent series of Dutch patients with familial and sporadic IA. Methods We conducted a secondary analysis of our institutional cohort of patients who were screened for IAs between 1994 and 2016. We assessed the incidence of aneurysmal subarachnoid hemorrhage between familial, defined as ≥2 affected first-degree relatives with aneurysmal subarachnoid hemorrhage and unruptured IA (UIA), and sporadic patients with UIA with Cox regression analysis. Results We identified 62 familial IA patients with 91 UIA and 412 sporadic IA patients with 542 UIA. Despite familial aneurysms being smaller and more often located at low risk sites than sporadic IA, 3 familial patients had aneurysmal subarachnoid hemorrhage (0.77 ruptures per 100 aneurysm-years [95% CI, 0.20–2.09]) compared with 7 sporadic patients (0.51 ruptures per 100 aneurysm-years [95% CI, 0.22–1.01]). As compared to sporadic UIA, familial UIA seems to have a 3-fold higher risk of rupture (hazard ratio, 2.9 [95% CI, 0.6–14]). Conclusions Our results suggest a slightly increased risk of aneurysm rupture for familial compared with sporadic IA, although we were not able to demonstrate this with statistical significance. However, the rupture risk seems less strongly increased than found in a previous study. Based on our results, we recommend to treat familial UIA more aggressively.
Similar to aSAH, smoking is a risk factor for PMH and excessive alcohol consumption is not. In contrast to aSAH, hypertension is not a risk factor for PMH. This implies that the pathophysiological mechanisms causing PMH might be slightly different from those causing aSAH.
Background and objectives:Screening for unruptured intracranial aneurysms (UIAs) is effective for first degree relatives (FDRs) of aneurysmal subarachnoid hemorrhage (aSAH) patients. Whether screening is also effective for FDRs of UIA patients is unknown. We determined the yield of screening in such FDRs, assessed rupture risk and treatment decisions of aneurysms that were found, identified potential high-risk subgroups and studied effects of screening on quality of life (QoL).Methods:In this prospective cohort study we included FDRs, aged 20–70 years, of UIA patients without a family history of aSAH who visited the Neurology outpatient clinic in one of three participating tertiary referral centers in the Netherlands. FDRs were screened for UIA with magnetic resonance angiography (MRA) between 2017 and 2021. We determined UIA prevalence and developed a prediction model for UIA risk at screening using multivariable logistic regression. QoL was evaluated with questionnaires six times during the first year following screening and assessed with a linear mixed effects model.Results:We detected 24 UIAs in 23 of 461 screened FDRs, resulting in a 5.0% prevalence (95% CI: 3.2–7.4%). Median aneurysm size was 3mm (IQR: 2–4mm) and median 5-year rupture risk assessed with the PHASES score was 0.7% (IQR: 0.4–0.9%). All UIAs received follow-up imaging, none were treated preventively. After a median follow-up of 24 months (IQR: 13–38 months) no UIA had changed. Predicted UIA risk at screening ranged between 2.3–14.7% with the highest risk in FDRs who smoke and have excessive alcohol consumption (c-statistic: 0.76; 95% CI: 0.65–0.88). At all survey moments health-related QoL (HRQoL) and emotional functioning were comparable with those in a reference group from the general population. One FDR with a positive screen expressed regret about screening.Discussion:Based on the current data, we do not advise screening FDRs of UIA patients, since all identified UIAs had a low rupture risk. We observed no negative effect of screening on QoL. Longer follow-up should determine the risk of aneurysm growth requiring preventive treatment.
ObjectPatients with familial intracranial aneurysms (IA) have a higher risk of rupture than patients with sporadic IA. We compared geometric and morphological risk factors for aneurysmal rupture between patients with familial and sporadic aneurysmal subarachnoid hemorrhage (aSAH) to analyse if these risk factors contribute to the increased rupture rate of familial IA.MethodsGeometric and morphological aneurysm characteristics were studied on CT-angiography in a prospectively collected series of patients with familial and sporadic aSAH, admitted between September 2006 and September 2009, and additional patients with familial aSAH retrieved from the prospectively collected database of familial IA patients of our center. Odds ratios (OR) with corresponding 95% confidence intervals (95% CI) were calculated to compare the aneurysm characteristics between patients with familial and sporadic aSAH.ResultsWe studied 67 patients with familial and 184 with sporadic aSAH. OR’s for familial compared with sporadic aSAH were for oval shape 1.16(95%CI:0.65–2.09), oblong shape 0.26(95%CI:0.03–2.13), irregular shape 0.83(95%CI:0.47–1.49), aspect ratio ≥ 1.6 0.94(95%CI:0.54–1.66), contact with the perianeurysmal environment (PAE) 1.15(95%CI:0.56–2.40), deformation by the PAE 1.05(95%CI:0.47–2.35) and for dominance of the posterior communicating artery (PCoA) in case of PCoA aneurysms 1.97(95% CI:0.50–7.83).ConclusionsThe geometric and morphological risk factors for aneurysm rupture do not have a higher prevalence in familial than in sporadic aSAH and thus do not explain the increased risk of IA rupture in patients with familial IA. We recommend further search for other potential risk factors for rupture of familial IA, such as genetic factors.
Background and Purpose— Intracranial aneurysm (IA) size and location are important determinants of aneurysm rupture risk. In familial IAs there is concordance of location; however, if such concordance exists for size is unknown. We analyzed the concordance of aneurysm size at time of rupture in familial IAs. Methods— In pairs of affected relatives with aneurysmal subarachnoid hemorrhage, the ratio between the largest and the smallest aneurysm size at time of rupture was calculated. We also compared the proportion of families in which both IAs ruptured at a size < or ≥7 mm with the proportion of families in which one IA ruptured at <7 mm and another ≥7 mm. We calculated the repeatability with corresponding 95% CI for aneurysm size at time of rupture. Results— About 130 patients from 64 families were included. Of the 68 affected pairs 18 (26%) had a ratio ≤1.2, 38 (57%) had a ratio >1.2, and 12 (17%) had a ratio ≥3. We found no difference between the proportion of families (n=31; 49%) who both had IA at time of rupture <7 mm (n=20; 31%) or both ≥7 mm (n=11; 18%) and the proportion of those families with one patient with an IA <7 mm and another with an IA ≥7 mm (n=33; 51%; P =0.86). Overall, the repeatability in aneurysm size at rupture within familial IAs was 0.10 (95% CI, 0–0.35). Conclusions— There is no good concordance in aneurysm size at rupture within familial IAs. These data suggest that size of a ruptured IA in a family member should not significantly impact on the management of a familial unruptured IA in a relative.
Objective:We combined individual patient data (IPD) from prospective cohorts of patients with unruptured intracranial aneurysms (UIA) to assess to what extent patients with familial UIA have a higher rupture risk than those with sporadic UIA.Methods:For this IPD meta-analysis we performed an Embase and Pubmed search for studies published up to December 1, 2020. We included studies that 1) had a prospective study design; 2) included 50 or more patients with UIA; 3) studied the natural course of UIA and risk factors for aneurysm rupture including family history for aneurysmal subarachnoid haemorrhage and UIA; and 4) had aneurysm rupture as an outcome. Cohorts with available IPD were included. All studies included patients with newly diagnosed UIA visiting one of the study centers. The primary outcome was aneurysmal rupture. Patients with polycystic kidney disease and moyamoya disease were excluded. We compared rupture rates of familial versus sporadic UIA using a Cox proportional hazard regression model adjusted for the PHASES score and smoking. We performed two analyses: 1. only studies defining first-degree relatives as parents, children, and siblings and 2. all studies, including those in which first-degree relatives are defined as only parents and children, but not siblings.Results:We pooled IPD from eight cohorts with a low and moderate risk of bias. First-degree relatives were defined as parents, siblings and children in six cohorts (29% Dutch, 55% Finnish, 15% Japanese), totalling 2,297 patients (17% familial, 399 patients) with 3,089 UIA and 7,301 person-years follow-up. Rupture occurred in 10 familial patients (rupture rate: 0·89%/person-year; 95% CI:0·45-1·59) and 41 sporadic patients (0·66%/person-year; 95% CI:0·48-0·89); adjusted HR for familial patients 2·56 (95% CI: 1·18–5·56). After adding also the two cohorts excluding siblings as first-degree relatives resulting in 9,511 patients the adjusted HR was 1·44 (95% CI: 0·86–2·40).Conclusion:The risk of rupture of UIA is two and a half times higher, with a range from a 1.2 to 5 times higher risk, in familial than in sporadic UIA. When assessing the risk of rupture in UIA, family history should be taken into account.
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