Motor Neurone Disease

  • Nages Nagaratnam
  • Kujan Nagaratnam
  • Gary Cheuk
Reference work entry


Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder. It affects both upper and lower motor neurons and is characterised by a progressive degeneration and death of the anterior horn cells, cortico-spinal tracts and/or bulbar motor nuclei. The cause is unknown. There is an association between frontotemporal dementia (FTD) and ALS. ALS commonly occurs between the ages of 40 and 60 years, and males are affected more than females. The disease is relentlessly progressive. The median life span is about 4 years. Most with ALS will die from respiratory failure. Symptoms of bulbar involvement usually imply a rapid course usually with survival of a year. There is no cure, and medical care is mainly palliation and involves a multidisciplinary approach.


Motor neurone disease Amyotrophic lateral sclerosis Frontotemporal dementia (FTD) Riluzole El Escorial criteria Awaji criteria 


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Nages Nagaratnam
    • 1
  • Kujan Nagaratnam
    • 1
  • Gary Cheuk
    • 2
  1. 1.The University of SydneyWestmead Clinical SchoolWestmeadAustralia
  2. 2.Rehabilitation and Aged Care ServiceBlacktown-Mt Druitt HospitalMount DruittAustralia

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