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Pathophysiology and Treatment of Non-motor Dysfunction in Amyotrophic Lateral Sclerosis.

Publication ,  Journal Article
Mahoney, CJ; Ahmed, RM; Huynh, W; Tu, S; Rohrer, JD; Bedlack, RS; Hardiman, O; Kiernan, MC
Published in: CNS Drugs
May 2021

Amyotrophic lateral sclerosis is a progressive and fatal neurodegenerative disease typically presenting with bulbar or limb weakness. There is increasing evidence that amyotrophic lateral sclerosis is a multisystem disease with early and frequent impacts on cognition, behaviour, sleep, pain and fatigue. Dysfunction of normal physiological and metabolic processes also appears common. Evidence from pre-symptomatic studies and large epidemiological cohorts examining risk factors for the future development of amyotrophic lateral sclerosis have reported a high prevalence of changes in behaviour and mental health before the emergence of motor weakness. This suggests that changes beyond the motor system are underway at an early stage with dysfunction across brain networks regulating a variety of cognitive, behavioural and other homeostatic processes. The full impact of non-motor dysfunction continues to be established but there is now sufficient evidence that the presence of non-motor symptoms impacts overall survival in amyotrophic lateral sclerosis, and with up to 80% reporting non-motor symptoms, there is an urgent need to develop more robust therapeutic approaches. This review provides a contemporary overview of the pathobiology of non-motor dysfunction, offering readers a practical approach with regard to assessment and management. We review the current evidence for pharmacological and non-pharmacological treatment of non-motor dysfunction in amyotrophic lateral sclerosis and highlight the need to further integrate non-motor dysfunction as an important outcome measure for future clinical trial design.

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Published In

CNS Drugs

DOI

EISSN

1179-1934

Publication Date

May 2021

Volume

35

Issue

5

Start / End Page

483 / 505

Location

New Zealand

Related Subject Headings

  • Risk Factors
  • Research Design
  • Psychiatry
  • Outcome Assessment, Health Care
  • Humans
  • Disease Progression
  • Cognitive Dysfunction
  • Amyotrophic Lateral Sclerosis
  • 3214 Pharmacology and pharmaceutical sciences
  • 3209 Neurosciences
 

Citation

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Chicago
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Mahoney, C. J., Ahmed, R. M., Huynh, W., Tu, S., Rohrer, J. D., Bedlack, R. S., … Kiernan, M. C. (2021). Pathophysiology and Treatment of Non-motor Dysfunction in Amyotrophic Lateral Sclerosis. CNS Drugs, 35(5), 483–505. https://doi.org/10.1007/s40263-021-00820-1
Mahoney, Colin J., Rebekah M. Ahmed, William Huynh, Sicong Tu, Jonathan D. Rohrer, Richard S. Bedlack, Orla Hardiman, and Matthew C. Kiernan. “Pathophysiology and Treatment of Non-motor Dysfunction in Amyotrophic Lateral Sclerosis.CNS Drugs 35, no. 5 (May 2021): 483–505. https://doi.org/10.1007/s40263-021-00820-1.
Mahoney CJ, Ahmed RM, Huynh W, Tu S, Rohrer JD, Bedlack RS, et al. Pathophysiology and Treatment of Non-motor Dysfunction in Amyotrophic Lateral Sclerosis. CNS Drugs. 2021 May;35(5):483–505.
Mahoney, Colin J., et al. “Pathophysiology and Treatment of Non-motor Dysfunction in Amyotrophic Lateral Sclerosis.CNS Drugs, vol. 35, no. 5, May 2021, pp. 483–505. Pubmed, doi:10.1007/s40263-021-00820-1.
Mahoney CJ, Ahmed RM, Huynh W, Tu S, Rohrer JD, Bedlack RS, Hardiman O, Kiernan MC. Pathophysiology and Treatment of Non-motor Dysfunction in Amyotrophic Lateral Sclerosis. CNS Drugs. 2021 May;35(5):483–505.
Journal cover image

Published In

CNS Drugs

DOI

EISSN

1179-1934

Publication Date

May 2021

Volume

35

Issue

5

Start / End Page

483 / 505

Location

New Zealand

Related Subject Headings

  • Risk Factors
  • Research Design
  • Psychiatry
  • Outcome Assessment, Health Care
  • Humans
  • Disease Progression
  • Cognitive Dysfunction
  • Amyotrophic Lateral Sclerosis
  • 3214 Pharmacology and pharmaceutical sciences
  • 3209 Neurosciences