Amyotrophic Lateral Sclerosis: An Overview

摘要

Amyotrophic Lateral Sclerosis is an adult-onset neurodegenerative disease that causes paralysis. Presently it is incurable and rapidly progressive with a survival of 4-5 years from onset. It is degeneration of upper and lower motor neurons which results in weakness and wasting of muscles in arms, legs, trunk and bulbar region. There are two types sporadic and familial amyotrophic lateral sclerosis. The age of onset of people with familial is widely reported as being about 10 years younger than for those with apparently sporadic amyotrophic lateral sclerosis. Epidemiological studies show a worldwide incidence of 2-3 per year per 100-000 population over age of 15 years. About 10% of individuals have a family history of amyotrophic lateral sclerosis. The cause is unknown. Genes linked to diseases have been identified including one (C9ORF72) that seems to be particulary important. Researchers are studying several causes of ALS like gene mutation, chemical imbalance, disorganized immune response and protein mishandling. Riluzole and Radicava were approved for the treatment by the Food and Drug administration. The diagnosis of ALS is devastating for patient and family members. People with ALS should be delivered with multidisciplinary team. The current hope is that stem cells of neural or extraneural origin might be modified in vitro to neurons that may migrate to the sites of motor neuron loss. The quality of life is important not just the duration of survival.