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Amyotrophic lateral sclerosis: pathogenesis, differential diagnoses, and potential interventions
Sarju A Patel1, Nicholas J Maragakis
1Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
Background:
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative motor neuron disease. The etiology is likely multifactorial, involving both genetic and environmental factors.
Method:
Literature review.
Findings:
Motor neuron death is believed to arise from mutations in superoxide dismutase 1. Abnormal neurofilament metabolism, glutamate transporter dysfunction, and altered responses to growth factors may play a role. These discoveries have highlighted possible interventions, several of which have subsequently undergone human clinical trials. These include studies of antioxidants, antiglutamatergic agents, cyclooxygenase inhibitors, and various growth factors.
Conclusion:
Findings are inconclusive, and further investigations are underway. At present, nonpharmacologic interventions such as nutritional support; physical, speech, and occupational therapy; and respiratory management offer the best means of improving quality of life for patients with ALS.