关键词: Fahr’s disease basal ganglia calcification infection primary familial brain calcification systematic review

来  源:   DOI:10.3390/jcm13082365   PDF(Pubmed)

Abstract:
Background: It is unclear whether patients with basal ganglia calcifications (BGC) should undergo infectious disease testing as part of their diagnostic work-up. We investigated the occurrence of possibly associated infections in patients with BGC diagnosed with Fahr\'s disease or syndrome and consecutively performed a systematic review of published infectious diseases associated with BGC. Methods: In a cross-sectional study, we evaluated infections in non-immunocompromised patients aged ≥ 18 years with BGC in the Netherlands, who were diagnosed with Fahr\'s disease or syndrome after an extensive multidisciplinary diagnostic work-up. Pathogens that were assessed included the following: Brucella sp., cytomegalovirus, human herpesvirus type 6/8, human immunodeficiency virus (HIV), Mycobacterium tuberculosis, rubella virus, and Toxoplasma gondii. Next, a systematic review was performed using MEDLINE and Embase (2002-2023). Results: The cross-sectional study included 54 patients (median age 65 years). We did not observe any possible related infections to the BGC in this population. Prior infection with Toxoplasma gondii occurred in 28%, and in 94%, IgG rubella antibodies were present. The positive tests were considered to be incidental findings by the multidisciplinary team since these infections are only associated with BGC when congenitally contracted and all patients presented with adult-onset symptoms. The systematic search yielded 47 articles, including 24 narrative reviews/textbooks and 23 original studies (11 case series, 6 cross-sectional and 4 cohort studies, and 2 systematic reviews). Most studies reported congenital infections associated with BGC (cytomegalovirus, HIV, rubella virus, Zika virus). Only two studies reported acquired pathogens (chronic active Epstein-Barr virus and Mycobacterium tuberculosis). The quality of evidence was low. Conclusions: In our cross-sectional study and systematic review, we found no convincing evidence that acquired infections are causing BGC in adults. Therefore, we argue against routine testing for infections in non-immunocompromised adults with BGC in Western countries.
摘要:
背景:目前尚不清楚基底神经节钙化(BGC)患者是否应接受感染性疾病检测作为其诊断工作的一部分。我们调查了诊断为Fahr病或综合征的BGC患者可能相关感染的发生情况,并连续对已发表的与BGC相关的传染病进行了系统评价。方法:在一项横断面研究中,我们评估了荷兰年龄≥18岁的BGC非免疫功能低下患者的感染,经过广泛的多学科诊断工作后,他们被诊断出患有Fahr病或综合征。评估的病原体包括:布鲁氏菌。,巨细胞病毒,人类疱疹病毒6/8型,人类免疫缺陷病毒(HIV),结核分枝杆菌,风疹病毒,和弓形虫.接下来,我们使用MEDLINE和Embase(2002-2023年)进行了系统评价.结果:横断面研究包括54例患者(中位年龄65岁)。我们在该人群中没有观察到任何可能的与BGC相关的感染。先前感染弓形虫的发生率为28%,在94%中,存在IgG风疹抗体。阳性测试被认为是多学科团队的偶然发现,因为这些感染仅在先天性感染时与BGC相关,并且所有患者均出现成人发作症状。系统搜索产生了47篇文章,包括24个叙述性评论/教科书和23个原创研究(11个案例系列,6项横断面研究和4项队列研究,和2个系统综述)。大多数研究报告了与BGC(巨细胞病毒,艾滋病毒,风疹病毒,寨卡病毒)。只有两项研究报告了获得性病原体(慢性活动性EB病毒和结核分枝杆菌)。证据质量较低。结论:在我们的横断面研究和系统评价中,我们没有发现令人信服的证据表明获得性感染会导致成人BGC.因此,我们反对在西方国家对未免疫功能低下的成人BGC进行常规感染检测.
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