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溶酶体贮积病的神经学表现。

Neurological manifestations of lysosomal storage diseases.

作者信息

Elendu Chukwuka, Babawale Emmanuel A, Babarinde Festus O, Babatunde Olusola D, Chukwu Christopher, Chiegboka Sobechukwu F, Shode Omotola P, Ngozi-Ibeh Jide K, Njoku Anthonia, Ikokwu Mary N, Kaka Grace U, Hassan Jemilah I, Fatungase Oluwasunmisola O, Osifodunrin Tolulope, Udoeze Chidi A, Ikeji Victor I

机构信息

Federal University Teaching Hospital, Owerri, Nigeria.

Griffith College Dublin, Dublin, Ireland.

出版信息

Ann Med Surg (Lond). 2024 Sep 30;86(11):6619-6635. doi: 10.1097/MS9.0000000000002611. eCollection 2024 Nov.

Abstract

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.

摘要

溶酶体贮积症(LSDs)是一组罕见的遗传性代谢紊乱疾病,其特征是溶酶体内未降解底物的积累,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LSDs的神经系统表现,重点关注中枢神经系统(CNS)受累情况及治疗策略。虽然文中未详细阐述每种LSD亚型及其相关的CNS表现的复杂细节,但旨在总结主要发现及意义。LSDs的神经系统表现包括一系列症状,如认知障碍、运动功能障碍、癫痫发作和感觉缺陷,这些显著影响患者的生活质量,并带来治疗挑战。当前的治疗策略主要旨在缓解症状和减缓疾病进展,但在逆转已有的神经损伤方面成效有限。酶替代疗法、底物减少疗法和新兴的基因疗法有望解决LSDs中的CNS受累问题。然而,血脑屏障穿透和长期疗效等挑战依然存在。除了讨论治疗方式外,本文还强调了早期诊断、多学科护理以及患者宣传在优化受LSDs影响个体的治疗结果方面的重要性。同时也探讨了伦理考量,包括公平获得新兴治疗方法以及整合个性化医疗方法。总体而言,本文强调了遗传学、神经科学和临床护理之间在理解和管理LSDs的神经系统表现方面的复杂相互作用,同时强调需要持续开展研究与合作,以推进治疗干预措施并改善患者治疗结果。

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