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肌萎缩侧索硬化及其模仿综合征/变异型:全面综述

Amyotrophic Lateral Sclerosis and its Mimics/Variants: A Comprehensive Review.

作者信息

Yedavalli Vivek S, Patil Abhijit, Shah Parinda

机构信息

Department of Neuroradiology and Neurointervention, Stanford University, Palo Alto, California, USA.

Department of Radiology, Advocate Illinois Masonic Medical Center, Chicago, Illinois, USA.

出版信息

J Clin Imaging Sci. 2018 Dec 6;8:53. doi: 10.4103/jcis.JCIS_40_18. eCollection 2018.

Abstract

Motor neuron diseases (MNDs) are a debilitating subset of diseases, which result in progressive neuronal destruction and eventual loss of voluntary muscular function. These entities are often challenging to distinguish and accurately diagnose given overlapping clinical pictures and overall rarity. This group of diseases has a high morbidity and mortality rate overall and delineating each type of disease can help guide appropriate clinical management and improve quality of life for patients. Of all MNDs, amyotrophic lateral sclerosis (ALS) is by far the most common comprising 80%-90% of cases. However, other mimics and variants of ALS can appear similar both clinically and radiographically. In this review, we delve into the epidemiological, physiological, neuroimaging, and prognostic characteristics and management of ALS and its most common MND mimics/variants. In doing so, we hope to improve accuracy in diagnosis and potential management for this rare group of diseases.

摘要

运动神经元病(MNDs)是一组使人衰弱的疾病,会导致神经元进行性破坏,最终丧失自主肌肉功能。鉴于临床表现重叠且总体发病率低,这些疾病往往难以区分和准确诊断。这组疾病总体发病率和死亡率较高,明确每种疾病类型有助于指导适当的临床管理并提高患者生活质量。在所有运动神经元病中,肌萎缩侧索硬化症(ALS)最为常见,占病例的80%-90%。然而,ALS的其他类似疾病和变异型在临床和影像学上可能表现相似。在本综述中,我们深入探讨了ALS及其最常见的运动神经元病类似疾病/变异型的流行病学、生理学、神经影像学、预后特征及管理。通过这样做,我们希望提高对这一罕见疾病组诊断和潜在管理的准确性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7140/6302559/e30575df1220/JCIS-8-53-g003.jpg

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