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与妊娠重叠的肌萎缩侧索硬化症的临床特征、病程和结局:38 例已发表病例的系统评价。

Clinical characteristics, course, and outcomes of amyotrophic lateral sclerosis overlapping with pregnancy: a systematic review of 38 published cases.

机构信息

Faculty of Medicine, Menoufia University, Shibin El-Kom, Menoufia, Egypt.

Medical Research Group of Egypt, Negida Academy, Arlington, MA, USA.

出版信息

Neurol Sci. 2023 Dec;44(12):4219-4231. doi: 10.1007/s10072-023-06994-4. Epub 2023 Aug 17.

DOI:10.1007/s10072-023-06994-4
PMID:37587387
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10641051/
Abstract

OBJECTIVES

Amyotrophic lateral sclerosis (ALS) is a rare and fatal neurodegenerative disease that can overlap with pregnancy, but little is known about its clinical characteristics, course, and outcomes in this context. This systematic review aimed to synthesize the current evidence on ALS overlapping with pregnancy.

METHODS

We comprehensively searched four databases on February 2, 2023, to identify case studies reporting cases of ALS overlapping with pregnancy. Joanna Brigs Institute tool was followed to assess the quality of the included studies.

RESULTS

Twenty-six articles reporting 38 cases were identified and included in our study. Out of the 38 cases, 18 were aged < 30 years. The onset of ALS was before pregnancy in 18 cases, during pregnancy in 16 cases, and directly after pregnancy in 4 cases. ALS progression course was rapid or severe in 55% of the cases during pregnancy, and this percentage reached 61% in cases with an onset of ALS before pregnancy. While ALS progression course after pregnancy was rapid or severe in 63% and stable in 37% of the cases. Most cases (95%) were able to complete the pregnancy and gave live birth. However, preterm delivery was common. For neonates, 86% were healthy without any complications.

CONCLUSION

While pregnancy with ALS is likely to survive and result in giving birth to healthy infants, it could be associated with rapid or severe progression of ALS and result in a worse prognosis, highlighting the importance of close monitoring and counselling for patients and healthcare providers.

摘要

目的

肌萎缩侧索硬化症(ALS)是一种罕见且致命的神经退行性疾病,可能与妊娠重叠,但在此背景下,人们对其临床特征、病程和结局知之甚少。本系统评价旨在综合目前关于与妊娠重叠的 ALS 的证据。

方法

我们于 2023 年 2 月 2 日全面检索了四个数据库,以确定报告 ALS 与妊娠重叠病例的病例研究。采用 Joanna Brigs 研究所工具评估纳入研究的质量。

结果

共确定并纳入了 26 篇报告 38 例的文章。在 38 例病例中,18 例年龄<30 岁。18 例 ALS 发病在妊娠前,16 例在妊娠期间,4 例在妊娠后直接发病。妊娠期间 ALS 进展迅速或严重的病例占 55%,妊娠前发病的病例这一比例达到 61%。而妊娠后 ALS 进展迅速或严重的病例占 63%,稳定的病例占 37%。大多数病例(95%)能够完成妊娠并分娩活产儿。然而,早产较为常见。对于新生儿,86%健康,无任何并发症。

结论

虽然妊娠合并 ALS 可能存活并分娩健康婴儿,但可能与 ALS 迅速或严重进展相关,并导致预后更差,这突显了对患者和医疗保健提供者进行密切监测和咨询的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2786/10641051/3c1486e10be4/10072_2023_6994_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2786/10641051/3c1486e10be4/10072_2023_6994_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2786/10641051/3c1486e10be4/10072_2023_6994_Fig1_HTML.jpg

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A case of successful pregnancy managed in a patient living with Motor Neurone Disease for more than 3 years.一名患有运动神经元病超过 3 年的患者成功妊娠并得到妥善管理。
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