RILUZOLE PRESERVES MOTOR FUNCTION IN A TRANSGENIC MODEL OF FAMILIAL AMYOTROPHIC-LATERAL-SCLEROSIS

Riluzole was tested in a dose-ranging study for preservation of motor function in a transgenic mouse model of familial ALS. The model is bas ed on expression of mutant human Cu,Zn superoxide dismutase in mouse b rain and spinal cord. In contrast with the human ALS trials, in the mo use model, riluzole significantly preserved motor function as assessed by nightly running in a wheel. The effect of riluzole on motor perfor mance was greater earlier in disease than later, suggesting that riluz ole may have benefit for ''quality-of-life'' measures in ALS. Treatmen t with riluzole was initiated earlier in the transgenic model than in the human ALS trials, which may account for the significantly better o utcome.