背景:人囊性包虫病(CE)是由属于细粒棘球蚴(s.l.)复合体的物种的幼虫期引起的人畜共患寄生虫感染。引起人类CE的寄生虫性囊肿主要位于肝脏和肺中。在少数情况下,幼虫可以在任何器官或组织中建立,包括中枢神经系统(CNS)。脑CE(CCE)很少见,但构成了严重的临床挑战。
方法:本研究提供了一个居住在罗马(意大利)附近农村的儿童的CCE案例,以及对来自患者和当地农场绵羊的分离囊肿标本的比较分子分析。我们还系统地搜索了文献,以总结这种罕见定位的最相关的流行病学和临床方面。
结果:比较分子分析证实感染是由S.S.(G3基因型)引起的,很可能是在家庭农场获得的。文献检索确定了2,238例CCE。在80.51%的病例中,大脑是唯一的定位,84.07%的病例存在单个CCE囊肿。患者平均年龄为20岁,70.46%为儿童。据报道,囊肿破裂占12.96%,治疗后CCE复发占9.61%。7.86%的病例报告了永久残疾,而死亡发生率为6.21%。如果系列报告所有CE本地化,CCE占所有CE病例的1.5%。在分子水平确定CCE囊肿的少数报道中,在40%的病例中发现了E.granulosuss.s.,在60%的病例中发现了E.canadensis。
结论:我们报告了一例罕见的CCE病例,并证实了可能的局部感染来源。局部罕见且对患者生活影响较大的CE病例比例已被全球忽略,应纳入CE全球负担的计算中。
Human cystic echinococcosis (CE) is a zoonotic parasitic infection caused by the larval stage of the species belonging to the Echinococcus granulosus sensu lato (s.l.) complex. Parasitic cysts causing human CE are mainly localized in the liver and in the lungs. In a smaller number of cases, larvae may establish in any organ or tissue, including the central nervous system (CNS). Cerebral CE (CCE) is rare but poses serious clinical challenges.
This study presents a case of CCE in a child living in the countryside near Rome (Italy), along with a comparative molecular analysis of the isolated cyst specimens from the patient and sheep of local farms. We also systematically searched the literature to summarize the most relevant epidemiological and clinical aspects of this uncommon localization.
The comparative molecular analysis confirmed that the infection was caused by E. granulosus sensu stricto (s.s.) (G3 genotype), and most likely acquired in the family farm. The literature search identified 2,238 cases of CCE. In 80.51% of cases, brain was the only localization and single CCE cysts were present in 84.07% of cases. Mean patients\' age was 20 years and 70.46% were children. Cyst rupture was reported in 12.96% and recurrence of CCE after treatment in 9.61% of cases. Permanent disability was reported in 7.86% of cases, while death occurred in 6.21%. In case series reporting all CE localization, CCE represented 1.5% of all CE cases. In the few reports that identified at molecular level the CCE cyst, E. granulosus s.s. was found in 40% and E. canadensis in 60% of cases.
We report a rare case of CCE and evidenced the probable local origin of infection. The proportions of CE cases with uncommon localizations and with high impact on patients\' lives have been globally neglected and should be included in the computation of the global burden of CE.