Amyotrophic lateral sclerosis

被引：408

作者：

Feldman, Eva L. ^{[1
]}

Goutman, Stephen A. ^{[1
]}

Petri, Susanne ^{[2
]}

Mazzini, Letizia ^{[3
,4
]}

Savelieff, Masha G. ^{[1
]}

Shaw, Pamela J. ^{[5
]}

Sobue, Gen ^{[6
]}

机构：

[1] Univ Michigan, Dept Neurol, Michigan Med, Ann Arbor, MI 48109 USA

[2] Hannover Med Sch, Dept Neurol, Hannover, Germany

[3] Azienda Osped Univ Maggiore Carita, ALS Ctr, Novara, Italy

[4] Univ Piemonte Orientale, Dept Neurol, Novara, Italy

[5] Univ Sheffield, Sheffield Inst Translat Neurosci SITraN, Sheffield, S Yorkshire, England

[6] Aichi Med Univ, Dept Neurol, Nagakute, Aichi, Japan

来源：

LANCET | 2022年 / 400卷 / 10360期

基金：

欧盟地平线“2020”; 英国医学研究理事会;

关键词：

QUALITY STANDARDS SUBCOMMITTEE; PRACTICE PARAMETER UPDATE; AMERICAN ACADEMY; COGNITIVE IMPAIRMENT; SYMPTOM MANAGEMENT; STAGING SYSTEM; ALS; PREVALENCE; DISEASE; TDP-43;

D O I：

10.1016/S0140-6736(22)01272-7

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Amyotrophic lateral sclerosis is a fatal CNS neurodegenerative disease. Despite intensive research, current management of amyotrophic lateral sclerosis remains suboptimal from diagnosis to prognosis. Recognition of the phenotypic heterogeneity of amyotrophic lateral sclerosis, global CNS dysfunction, genetic architecture, and development of novel diagnostic criteria is clarifying the spectrum of clinical presentation and facilitating diagnosis. Insights into the pathophysiology of amyotrophic lateral sclerosis, identification of disease biomarkers and modifiable risks, along with new predictive models, scales, and scoring systems, and a clinical trial pipeline of mechanism-based therapies, are changing the prognostic landscape. Although most recent advances have yet to translate into patient benefit, the idea of amyotrophic lateral sclerosis as a complex syndrome is already having tangible effects in the clinic. This Seminar will outline these insights and discuss the status of the management of amyotrophic lateral sclerosis for the general neurologist, along with future prospects that could improve care and outcomes for patients with amyotrophic lateral sclerosis.

引用

页码：1363 / 1380

页数：18

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