The Progression Of Medical Treatment In Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is an adult onset neurodegenerative disorder characterized by the death of upper and lower motor neurons. The mechanisms underlying selective motor neuron degeneration in ALS remain unknown. Approximately 20% of familial ALS cases are caused by mutations in the superoxide dismutase 1 (SOD1) gene. There is no curative treatment for ALS. Nevertheless, there is an increasing volume of published studies. There have been several important clinical trials on the treatment of ALS and treatment efficacy studies using mouse (SOD1) models of ALS. This review assesses the current evidence for treatment of ALS,which provide reference to investigator and clinician. Recent studies revealed that diminished mutant SOD1 expression in the astrocytes delayed microglial activation and slowed disease progression. It can be target of the gene therapy. Current evidence has implicated a 43-kDa TAR DNA-binding protein (TDP-43) in the pathologenesis of ALS. Several mutations in TDP-43 were discovered in families with inherited motor neuron disease. It is a complementarity for the mechanism of ALS. Antiglutamate toxicity has been reported to have a protective effect on ALS patients,which attribute to multiple glutamate receptors. For example riluzole, which is the only drug for the treatment of ALS approved by the Food and Drug Administration,but with only modest increase in survival. Although phase III trials revealed that IGF-1 was not beneficial for patients with ALS, favorable outcomes in SOD1 mice were reported with hepatocyte growth factor(hrHGF),recombinant human erythropoietin (rhEPO),the granulocyte colony stimulating factor (G-CSF). Administrations of antioxidant,amending mitochondrial function have been investigated. A conditioning lesion provides selective protection in a rat model of ALS. This years, transplantation of neural stem cells is hotspot of investigation.In a study, ALS-human mesenchymal stem cells were transplanted into cisterna magna in SOD1-G93A ALS mice,it is a potential route for stem cell therapy in ALS patients.