Amiortrophic Lateral Sclerosis

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Seminar

Amyotrophic lateral sclerosis
Matthew C Kiernan, Steve Vucic, Benjamin C Cheah, Martin R Turner, Andrew Eisen, Orla Hardiman, James R Burrell, Margaret C Zoing Lancet 2011; 377: 942–55
Published Online
February 7, 2011
DOI:10.1016/S01406736(10)61156-7
Neuroscience Research
Australia and Prince of Wales
Clinical School, University of
New South Wales, Sydney,
Australia (Prof M C Kiernan DSc,
B C Cheah MBiostat,
J Burrell MBBS, M C Zoing BNurs);
Western Clinical School,
University of Sydney, Sydney,
Australia (S Vucic PhD); Nuffield
Department of Clinical
Neurosciences, University
of Oxford, Oxford, UK
(M R Turner PhD); Division of
Neurology, Department of
Medicine, University of British
Columbia Vancouver,
Vancouver, Canada
(Prof A Eisen MD); and
Trinity College Institute of
Neuroscience, Dublin, Ireland
(Prof O Hardiman MD)
Correspondence to:
Prof Matthew C Kiernan,
Neuroscience Research Australia,
Barker Street, Randwick, Sydney,
NSW 2031 Australia
m.kiernan@unsw.edu.au

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.

Introduction
Since the 1990s, there has been growing scientific and
clinical interest in amyotrophic lateral sclerosis (ALS).
Advances in our understanding of the glutamate
neurotransmitter system and the discovery of causal genes
linked to the development of familial ALS have stimulated
research interest, problems associated with clinical
heterogeneity have been identified, and survival in ALS is
now understood to be dependent on several factors,
including clinical presentation (phenotype), rate of disease progression, early presence of respiratory failure, and the
nutritional status of patients.
Extending life expectancy in ALS seems to be dependent
on improving our understanding of its pathogenesis,
which will lead to the development of early and specific
diagnostic methods. There is a crucial need to formulate
therapies that not only slow disease progression, but also
deal with the secondary consequences of malnutrition
and respiratory failure. At present, no definitive diagnostic test or biomarker for ALS exist, and neurologists rely on
only clinical criteria for diagnosis. The development of
novel biomarkers to objectively assess disease progression
holds the promise of greatly refining therapeutic trial
design and reducing trial costs. Furthermore, the power
of population registries is being increasingly recognised
as an essential adjunct to improved clinical assessment
techniques. These collaborative endeavours will inevitably
lead to a better understanding of ALS and its often

Search strategy and selection criteria
We searched Medline (1966, to December, 2009), EmBase
(1980, to December, 2009), and the Cochrane Library using
the search terms “amyotrophic lateral sclerosis” or “motor neurone disease” in combination with “diagnosis”,
“epidemiology”, “fronto-temporal dementia”, “imaging”, “neurophysiology”, “management”, and “neuroprotection”. Further articles were included from reference lists, review
articles, and major textbook chapters. Abstracts and reports from relevant meetings were also included. The final reference list was generated on the basis of originality and relevance to the topics covered in this Seminar. Emphasis...
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