Genotype-phenotype relationship in hereditary amyotrophic lateral sclerosis

Abstract excerpt

Amyotrophic lateral sclerosis (ALS) is the most common adult-onset motor neuron disease. It is characterized by neuronal loss and degeneration of the upper motor neurons (UMNs) and lower motor neurons (LMNs), and is usually fatal due to respiratory failure within 3-5 years of onset. Although approximately 5-10 % of patients with ALS have an inherited form of the disease, the distinction between hereditary and...

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