Amyotrophic Lateral Sclerosis
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...
References and Readings
- 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
- 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