Riluzole preserves motor function in a transgenic model of familial amyotrophic lateral sclerosis

ME Gurney, TJ Fleck, CS Himes, ED Hall - Neurology, 1998 - AAN Enterprises
ME Gurney, TJ Fleck, CS Himes, ED Hall
Neurology, 1998AAN Enterprises
Riluzole was tested in a dose-ranging study for preservation of motor function in a
transgenic mouse model of familial ALS. The model is based on expression of mutant
human Cu, Zn superoxide dismutase in mouse brain and spinal cord. In contrast with the
human ALS trials, in the mouse model, riluzole significantly preserved motor function as
assessed by nightly running in a wheel. The effect of riluzole on motor performance was
greater earlier in disease than later, suggesting that riluzole may have benefit for" quality-of …
Riluzole was tested in a dose-ranging study for preservation of motor function in a transgenic mouse model of familial ALS. The model is based on expression of mutant human Cu,Zn superoxide dismutase in mouse brain and spinal cord. In contrast with the human ALS trials, in the mouse model, riluzole significantly preserved motor function as assessed by nightly running in a wheel. The effect of riluzole on motor performance was greater earlier in disease than later, suggesting that riluzole may have benefit for"quality-of-life" measures in ALS. Treatment with riluzole was initiated earlier in the transgenic model than in the human ALS trials, which may account for the significantly better outcome.
American Academy of Neurology