溶酶体贮积症的神经系统表现。

IF 1.7 Q2 MEDICINE, GENERAL & INTERNAL
Annals of Medicine and Surgery Pub Date : 2024-09-30 eCollection Date: 2024-11-01 DOI:10.1097/MS9.0000000000002611
Chukwuka Elendu, Emmanuel A Babawale, Festus O Babarinde, Olusola D Babatunde, Christopher Chukwu, Sobechukwu F Chiegboka, Omotola P Shode, Jide K Ngozi-Ibeh, Anthonia Njoku, Mary N Ikokwu, Grace U Kaka, Jemilah I Hassan, Oluwasunmisola O Fatungase, Tolulope Osifodunrin, Chidi A Udoeze, Victor I Ikeji
{"title":"溶酶体贮积症的神经系统表现。","authors":"Chukwuka Elendu, Emmanuel A Babawale, Festus O Babarinde, Olusola D Babatunde, Christopher Chukwu, Sobechukwu F Chiegboka, Omotola P Shode, Jide K Ngozi-Ibeh, Anthonia Njoku, Mary N Ikokwu, Grace U Kaka, Jemilah I Hassan, Oluwasunmisola O Fatungase, Tolulope Osifodunrin, Chidi A Udoeze, Victor I Ikeji","doi":"10.1097/MS9.0000000000002611","DOIUrl":null,"url":null,"abstract":"<p><p>Lysosomal storage diseases (LSDs) encompass a group of rare inherited metabolic disorders characterized by the accumulation of undegraded substrates within lysosomes, leading to multisystemic manifestations, including profound neurological involvement. This article provides a concise overview of the neurological manifestations of LSDs, with a focus on central nervous system (CNS) involvement and treatment strategies. While the paper intricacies of each LSD subtype and its associated CNS manifestations, it aims to provide a summary of the essential findings and implications. The neurological manifestations of LSDs encompass a spectrum of symptoms, including cognitive impairment, motor dysfunction, seizures, and sensory deficits, which significantly impact patients' quality of life and pose therapeutic challenges. Current treatment strategies primarily aim to alleviate symptoms and slow disease progression, with limited success in reversing established neurological damage. Enzyme replacement therapy, substrate reduction therapy, and emerging gene therapies hold promise for addressing CNS involvement in LSDs. However, challenges such as blood-brain barrier penetration and long-term efficacy remain. In addition to discussing treatment modalities, this article highlights the importance of early diagnosis, multidisciplinary care, and patient advocacy in optimizing outcomes for individuals affected by LSDs. Ethical considerations are also addressed, including equitable access to emerging treatments and integrating personalized medicine approaches. Overall, this article underscores the complex interplay between genetics, neuroscience, and clinical care in understanding and managing the neurological manifestations of LSDs while emphasizing the need for continued research and collaboration to advance therapeutic interventions and improve patient outcomes.</p>","PeriodicalId":8025,"journal":{"name":"Annals of Medicine and Surgery","volume":"86 11","pages":"6619-6635"},"PeriodicalIF":1.7000,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11543150/pdf/","citationCount":"0","resultStr":"{\"title\":\"Neurological manifestations of lysosomal storage diseases.\",\"authors\":\"Chukwuka Elendu, Emmanuel A Babawale, Festus O Babarinde, Olusola D Babatunde, Christopher Chukwu, Sobechukwu F Chiegboka, Omotola P Shode, Jide K Ngozi-Ibeh, Anthonia Njoku, Mary N Ikokwu, Grace U Kaka, Jemilah I Hassan, Oluwasunmisola O Fatungase, Tolulope Osifodunrin, Chidi A Udoeze, Victor I Ikeji\",\"doi\":\"10.1097/MS9.0000000000002611\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Lysosomal storage diseases (LSDs) encompass a group of rare inherited metabolic disorders characterized by the accumulation of undegraded substrates within lysosomes, leading to multisystemic manifestations, including profound neurological involvement. This article provides a concise overview of the neurological manifestations of LSDs, with a focus on central nervous system (CNS) involvement and treatment strategies. While the paper intricacies of each LSD subtype and its associated CNS manifestations, it aims to provide a summary of the essential findings and implications. The neurological manifestations of LSDs encompass a spectrum of symptoms, including cognitive impairment, motor dysfunction, seizures, and sensory deficits, which significantly impact patients' quality of life and pose therapeutic challenges. Current treatment strategies primarily aim to alleviate symptoms and slow disease progression, with limited success in reversing established neurological damage. Enzyme replacement therapy, substrate reduction therapy, and emerging gene therapies hold promise for addressing CNS involvement in LSDs. However, challenges such as blood-brain barrier penetration and long-term efficacy remain. In addition to discussing treatment modalities, this article highlights the importance of early diagnosis, multidisciplinary care, and patient advocacy in optimizing outcomes for individuals affected by LSDs. Ethical considerations are also addressed, including equitable access to emerging treatments and integrating personalized medicine approaches. Overall, this article underscores the complex interplay between genetics, neuroscience, and clinical care in understanding and managing the neurological manifestations of LSDs while emphasizing the need for continued research and collaboration to advance therapeutic interventions and improve patient outcomes.</p>\",\"PeriodicalId\":8025,\"journal\":{\"name\":\"Annals of Medicine and Surgery\",\"volume\":\"86 11\",\"pages\":\"6619-6635\"},\"PeriodicalIF\":1.7000,\"publicationDate\":\"2024-09-30\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11543150/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Annals of Medicine and Surgery\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1097/MS9.0000000000002611\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/11/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q2\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Annals of Medicine and Surgery","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1097/MS9.0000000000002611","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/11/1 0:00:00","PubModel":"eCollection","JCR":"Q2","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0

摘要

溶酶体贮积病(LSDs)是一组罕见的遗传性代谢性疾病,其特点是未降解的底物在溶酶体内积聚,导致多系统表现,包括严重的神经系统受累。本文简要概述了 LSD 的神经系统表现,重点是中枢神经系统(CNS)受累情况和治疗策略。虽然本文对每种 LSD 亚型及其相关的中枢神经系统表现进行了复杂的阐述,但其目的是对基本的研究结果和意义进行总结。LSD 的神经系统表现包含一系列症状,包括认知障碍、运动功能障碍、癫痫发作和感觉障碍,严重影响患者的生活质量,并给治疗带来挑战。目前的治疗策略主要旨在缓解症状和延缓疾病进展,但在逆转已形成的神经损伤方面成效有限。酶替代疗法、底物还原疗法和新兴的基因疗法有望解决中枢神经系统受 LSD 影响的问题。然而,血脑屏障渗透和长期疗效等挑战依然存在。除了讨论治疗方法外,本文还强调了早期诊断、多学科护理和患者权益维护对优化 LSD 患者预后的重要性。文章还讨论了伦理方面的考虑因素,包括公平获取新兴治疗方法和整合个性化医学方法。总之,这篇文章强调了遗传学、神经科学和临床护理在理解和管理 LSDs 神经系统表现方面的复杂相互作用,同时也强调了持续研究和合作的必要性,以推进治疗干预措施并改善患者预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Neurological manifestations of lysosomal storage diseases.

Lysosomal storage diseases (LSDs) encompass a group of rare inherited metabolic disorders characterized by the accumulation of undegraded substrates within lysosomes, leading to multisystemic manifestations, including profound neurological involvement. This article provides a concise overview of the neurological manifestations of LSDs, with a focus on central nervous system (CNS) involvement and treatment strategies. While the paper intricacies of each LSD subtype and its associated CNS manifestations, it aims to provide a summary of the essential findings and implications. The neurological manifestations of LSDs encompass a spectrum of symptoms, including cognitive impairment, motor dysfunction, seizures, and sensory deficits, which significantly impact patients' quality of life and pose therapeutic challenges. Current treatment strategies primarily aim to alleviate symptoms and slow disease progression, with limited success in reversing established neurological damage. Enzyme replacement therapy, substrate reduction therapy, and emerging gene therapies hold promise for addressing CNS involvement in LSDs. However, challenges such as blood-brain barrier penetration and long-term efficacy remain. In addition to discussing treatment modalities, this article highlights the importance of early diagnosis, multidisciplinary care, and patient advocacy in optimizing outcomes for individuals affected by LSDs. Ethical considerations are also addressed, including equitable access to emerging treatments and integrating personalized medicine approaches. Overall, this article underscores the complex interplay between genetics, neuroscience, and clinical care in understanding and managing the neurological manifestations of LSDs while emphasizing the need for continued research and collaboration to advance therapeutic interventions and improve patient outcomes.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Annals of Medicine and Surgery
Annals of Medicine and Surgery MEDICINE, GENERAL & INTERNAL-
自引率
5.90%
发文量
1665
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信