Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan

Amyotrophic Lateral Sclerosis

  • Alexander I. TrösterEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_514


Lou Gehrig’s disease

Short Description or Definition

The features of amyotrophic lateral sclerosis (ALS) were first described by Charcot in the nineteenth century. ALS is a progressive, fatal neurodegenerative disease affecting upper and lower motor neurons, although increasingly ALS is recognized as a multisystem disorder whose manifestations may also include cognitive and behavioral changes. Most patients present with motor neuron symptoms at disease onset, and as the disease progresses, persons with ALS demonstrate impairments in speech, swallowing, breathing, and use of upper and lower limbs, with eventual paralysis. The prevalence of cognitive changes, which is not well studied, is estimated to range from about 20% to 50% and most often involve executive dysfunction. Deficits in visuospatial, language, and memory functions are more inconsistently observed. When dementia is seen, it resembles a frontotemporal lobar degeneration or frontotemporal dementia characterized by...

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References and Readings

  1. Averill, A. J., Kasarskis, E. J., & Segerstrom, S. C. (2007). Psychological health in patients with amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis, 8, 243–254.PubMedCrossRefGoogle Scholar
  2. Beeldman, E., Raaphorst, J., Twennaar, M. K., de Visser, M., Schmand, B. A., & de Haan, R. J. (2015). The cognitive profile of ALS: A systematic review and meta-analysis update. Journal of Neurology, Neurosurgery, and Psychiatry.  https://doi.org/10.1136/jnnp-2015-310734. online first, 17 Aug 2015.CrossRefGoogle Scholar
  3. Brownlee, A., & Palovcak, M. (2007). The role of augmentative communication devices in the medical management of ALS. NeuroRehabilitation, 22, 445–450.PubMedGoogle Scholar
  4. Kiernan, M. C. (2015). Palliative care in amyotrophic lateral sclerosis. Lancet Neurology, 14, 347–348.PubMedCrossRefGoogle Scholar
  5. Lewis, M., & Rushanan, S. (2007). The role of physical therapy and occupational therapy in the treatment of amyotrophic lateral sclerosis. NeuroRehabilitation, 22, 451–461.PubMedGoogle Scholar
  6. Logroscino, G., Traynor, B. J., Hardiman, O., Chio, A., Couratier, P., Mitchell, J. D., et al. (2008). Descriptive epidemiology of amyotrophic lateral sclerosis: New evidence and unsolved issues. Journal of Neurology, Neurosurgery and Psychiatry, 79, 6–11.PubMedCrossRefGoogle Scholar
  7. McCluskey, L. (2007). Palliative rehabilitation and amyotrophic lateral sclerosis: A perfect match. NeuroRehabilitation, 22, 407–408.PubMedGoogle Scholar
  8. Murphy, J., et al. (2016). Cognitive-behavioral screening reveals prevalent impairment in a large multicenter ALS cohort. Neurology, 86, 813–820.PubMedPubMedCentralCrossRefGoogle Scholar
  9. Phukan, J., Pender, N. P., & Hardiman, O. (2007). Cognitive impairment in amyotrophic lateral sclerosis. Lancet Neurology, 6, 994–1003.PubMedCrossRefGoogle Scholar
  10. Radunovic, A., Mitsumoto, H., & Leigh, P. N. (2007). Clinical care of patients with amyotrophic lateral sclerosis. Lancet Neurology, 6, 913–925.PubMedCrossRefGoogle Scholar
  11. Strong, M. J., Grace, G. M., Orange, J. B., & Leeper, H. A. (1996). Cognition, language, and speech in amyotrophic lateral sclerosis: A review. Journal of Clinical and Experimental Neuropsychology, 18, 291–303.PubMedCrossRefGoogle Scholar
  12. Trojsi, F., Santangelo, G., Caiazzo, G., Siciliano, M., Ferrantino, T., Piccirillo, G., Femiano, C., Cristillo, V., Monsurro, M., Espositio, F., & Tedeschi, G. (2016). Neuropsychological assessment at different King’s clinical stages of amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 17, 1–7.CrossRefGoogle Scholar
  13. Van der Hulst, E.-J., Bak, T. H., & Abrahams, S. (2015). Impaired affective and cognitive theory of mind and behavioural change in amyotrophic lateral sclerosis. Journal of Neurology, Neurosurgery, and Psychiatry, 86, 1208–1215.PubMedCrossRefGoogle Scholar
  14. Woolley, S. C., & Rush, B. K. (2017). Considerations for clinical neuropsychoplogical evaluation in amyotrophic lateral sclerosis. Archives of Clinical Neuropsychology, 32, 906–916.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Clinical Neuropsychology and Center for NeuromodulationBarrow Neurological InstitutePhoenixUSA