Amyotrophic lateral sclerosis

被引：1787

作者：

Kiernan, Matthew C. ^{[1
,2
]}

Vucic, Steve ^{[3
]}

Cheah, Benjamin C. ^{[1
,2
]}

Turner, Martin R. ^{[4
]}

Eisen, Andrew ^{[5
]}

Hardiman, Orla ^{[6
]}

Burrell, James R. ^{[1
,2
]}

Zoing, Margaret C. ^{[1
,2
]}

机构：

[1] Neurosci Res Australia, Sydney, NSW 2031, Australia

[2] Univ New S Wales, Prince Wales Clin Sch, Sydney, NSW, Australia

[3] Univ Sydney, Western Clin Sch, Sydney, NSW 2006, Australia

[4] Univ Oxford, Nuffield Dept Clin Neurosci, Oxford, England

[5] Univ British Columbia, Dept Med, Div Neurol, Vancouver, BC V6T 1W5, Canada

[6] Trinity Coll Dublin, Inst Neurosci, Dublin, Ireland

来源：

LANCET | 2011年 / 377卷 / 9769期

基金：

英国医学研究理事会;

关键词：

MOTOR-NEURON DISEASE; POSITRON-EMISSION-TOMOGRAPHY; ALS-LINKED SOD1; FRONTOTEMPORAL LOBAR DEGENERATION; TRANSGENIC MOUSE MODEL; GOOD PRACTICE POINTS; QUALITY-OF-LIFE; EL-ESCORIAL; SPINAL-CORD; IN-VIVO;

D O I：

10.1016/S0140-6736(10)61156-7

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Amyotrophic lateral sclerosis (ALS) is an idiopathic, fatal neurodegenerative disease of the human motor system. In this Seminar, we summarise current concepts about the origin of the disease, what predisposes patients to develop the disorder, and discuss why all cases of ALS are not the same. In the 150 years since Charcot originally described ALS, painfully slow progress has been made towards answering these questions. We focus on what is known about ALS and where research is heading-from the small steps of extending longevity, improving therapies, undertaking clinical trials, and compiling population registries to the overarching goals of establishing the measures that guard against onset and finding the triggers for this neurodegenerative disorder.

引用

页码：942 / 955

页数：14

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