Hanyang Med Rev.
2006 Feb;26(1):44-51.
Diagnosis and Therapeutic Strategies of Amyotrophic Lateral Sclerosis
- Affiliations
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- 1Department of Neurology, Institute of Biomedical Science, College of Medcine, Hanyang University, Seoul, Korea. kimsh1@hanyang.ac.kr
Abstract
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Amyotrophic lateral sclerosis (ALS) is a progressive fatal neurodegenerative disease involving both upper and lower motor neuron systems, and multiple pathogenic mechanisms are involved in death of motor neurons; including oxidative injury, excitotoxicity, mitochondrial dysfunction, immune-inflammatory process and abnormal protein aggregation. Clinically, the disease is characterized by muscle weakness and wasting, often beginning focally, and gradually progressing over 1 or more years to involve muscles of the limb and bulbar regions, diffusely. Diagnosis is established by documenting clinical and/or electrophysiological evidences of both upper and lower motor neuron dysfunction and excluding other diseases showing similar clinical manifestations. In this article, the issues on diagnostic criteria, differential diagnosis, and pathogenic mechanism-based potential therapeutic approaches of ALS are reviewed.