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从临床医生角度看罕见神经系统疾病的分子诊断方法

Molecular diagnostic approach to rare neurological diseases from a clinician viewpoint.

作者信息

Lee Jin Sook

机构信息

Department of Pediatrics, Seoul National University Hospital Child Cancer and Rare Disease Administration, Seoul National University Children's Hospital, 101 Daehakro Jongno-gu, Seoul, 110-744, South Korea.

出版信息

Genomics Inform. 2024 Oct 10;22(1):18. doi: 10.1186/s44342-024-00025-0.

Abstract

Advancements in sequencing technology have significantly enhanced diagnostic capabilities for rare neurological diseases. This progress in molecular diagnostics can greatly impact clinical management and facilitate the development of personalized treatments for patients with rare neurological diseases. Neurologists with expertise should raise clinical awareness, as phenotyping remains crucial for making a clinical diagnosis, even in the genomics era. They should prioritize different types of genomic tests, considering both the benefits and the limitations inherent to each test. Notably, long-read sequencing is being utilized in cases suspected to involve repeat expansion disorders or complex structural variants. Repeat expansion disorders are highly prevalent in neurological diseases, particularly within the ataxia group. Significant efforts, including periodic reanalysis, data sharing, or integration of genomics with multi-omics studies, should be directed toward cases that remain undiagnosed after standard next-generation sequencing.

摘要

测序技术的进步显著提高了对罕见神经系统疾病的诊断能力。分子诊断方面的这一进展可极大地影响临床管理,并促进针对罕见神经系统疾病患者的个性化治疗的发展。具备专业知识的神经科医生应提高临床意识,因为即使在基因组学时代,表型分析对于做出临床诊断仍然至关重要。他们应根据每种检测固有的益处和局限性,对不同类型的基因组检测进行优先级排序。值得注意的是,长读长测序正被用于疑似涉及重复扩增疾病或复杂结构变异的病例。重复扩增疾病在神经系统疾病中非常普遍,尤其是在共济失调组中。对于标准二代测序后仍未确诊的病例,应做出重大努力,包括定期重新分析、数据共享或将基因组学与多组学研究相结合。

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