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Amyotrophic lateral sclerosis: a new diagnostic paradigm
  1. Matthew C Kiernan1,2,
  2. Jeremy M Shefner3,
  3. Ryuji Kaji4,
  4. David Burke5
  1. 1 Bushell Chair of Neurology, Brain and Mind Centre, University of Sydney, Sydney, New South Wales, Australia
  2. 2 Neurology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
  3. 3 Barrow Neurological Institute, Phoenix, Arizona, USA
  4. 4 Department of Neurology, Tokushima University Graduate School of Medicine, Tokushima, Japan
  5. 5 Institute of Clinical Neurosciences, University of Sydney, Sydney, New South Wales, Australia
  1. Correspondence to Professor Matthew C Kiernan, Bushell Chair of Neurology, Brain and Mind Centre, University of Sydney, Sydney, NSW 2040, Australia; matthew.kiernan{at}

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New consensus guidelines designed to facilitate diagnosis and improve patient access to clinical trials

In the absence of a definitive laboratory test that provides confirmation of a diagnosis of amyotrophic lateral sclerosis (ALS, also known as motor neuron disease), neurologists have relied on clinical signs.1 In turn, these signs are taken to reflect disease involvement of the upper and lower motor neuron compartments of the motor system,2–4 as initially identified by Charcot.5 Over more recent decades, diagnostic criteria have been developed and then refined to provide a degree of certainty to patients and their clinicians, and to guarantee that patients involved in clinical trials truly have ALS.6 7 With these criteria, degrees of certainty have been employed, from possible to probable and definite. This nomenclature, however, implies a level of diagnostic confidence that is confusing to patients, their families and clinicians, as all three levels of diagnosis indicate unambiguous ALS. …

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  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Commissioned; internally peer reviewed.