Amyotrophic lateral sclerosis is characterised by the progressive loss of motor neurons in the brain and spinal cord.This neurodegenerative syndrome shares pathobiological features with frontotemporal dementia and, indeed, manypatients show features of both diseases. Many different genes and pathophysiological processes contribute to thedisease, and it will be necessary to understand this heterogeneity to find effective treatments. In this Seminar, wediscuss clinical and diagnostic approaches as well as scientific advances in the research fields of genetics, diseasemodelling, biomarkers, and therapeutic strategies.
Amyotrophic lateral sclerosis
van Es, Michael A;Hardiman, Orla;Chio, Adriano;Al-Chalabi, Ammar;Pasterkamp, R Jeroen;Veldink, Jan H;van den Berg, Leonard H
2017-01-01
Abstract
Amyotrophic lateral sclerosis is characterised by the progressive loss of motor neurons in the brain and spinal cord.This neurodegenerative syndrome shares pathobiological features with frontotemporal dementia and, indeed, manypatients show features of both diseases. Many different genes and pathophysiological processes contribute to thedisease, and it will be necessary to understand this heterogeneity to find effective treatments. In this Seminar, wediscuss clinical and diagnostic approaches as well as scientific advances in the research fields of genetics, diseasemodelling, biomarkers, and therapeutic strategies.
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