ALS: Measuring disease progression

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This video aims to educate undergraduate university students with at least one year of science background about how to disease progression and development is measured for ALS (Amyotrophic Lateral Sclerosis, also known as Lou Gehrig’s disease or motor neuron disease). Despite recent advances in research, there is currently no cure for ALS and much is still unknown about the disease. The number of individuals who die from ALS has increased to three per day in Canada. This video discusses ALS research relating to the measurement of disease progression.

Video by students from McMaster University's Demystifying Medicine Seminar Series

References

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Armon, C. & Brandstater, M.E. (1999). Motor unit number estimate-based rates of progression of ALS predict patient survival. Muscle & Nerve, 22, 1571-1575.

Czaplinski, A., Yen, A.A. & Appel, S.H. (2006). Forced vital capacity (FVC) as an indicator of survival and disease progression in an ALS clinic population. Journal of Neurology, Neurosurgery, and Psychiatry, 77, 390-392.

DeJesus-Hernandez, M., Mackenzie, I.R., Boeve, B.F., Boxer, A.L., Baker, M., Rutherford, N.J., […] & Rademakers, R. (2011). Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-Linked FTD and ALS. Neuron, 72, 245-256.

Freischmidt, A., Wieland, T., Richter, B., Ruf, W., Schaeffer, V., Muller, K., […] & Weishaupt, J.H. (2015). Haploinsufficiency of TBK1 causes familial ALS and front-temporal dementia. Nature Neuroscience, 18, 631-636.

Simon, N.G., Turner, M. R., Vucic, S., Al-Chalabi, A., Shefner, J., Lomen-Hoerth, C. & Kiernan, M.C. (2014). Quantifying disease progression amyotrophic lateral sclerosis. Annals of Neurology, 76, 643-657.

Wong, P.C., Pardo, C.A., Borchelt, D.R., Lee, M.K., Copeland, N.G., Jenkins, J.A., […] & Price, D.L. (1995). An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron, 14, 1105-1116.