【摘 要】
：
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which attacks the motor system.Current treatment of human ALS patients or transgenic Cu, Zn superoxide dimutase 1 (SOD 1) mice,
【机 构】
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Department of Neurology Concord Hospital Australia
【出 处】
：
BIT`s 3rd Annual World Congress of NeuroTalk-2012(2012第三届国际神
论文部分内容阅读
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which attacks the motor system.Current treatment of human ALS patients or transgenic Cu, Zn superoxide dimutase 1 (SOD 1) mice, most commonly produces only a modest increase in survival.At present, Riluzole remains the only disease-modifying therapy available for ALS and has been used since 1995.Using the statistical motor unit number estimation (MUNE) technique, we performed a longitudinal study of asymptomatic carriers of the SOD 1 gene to determine whether early institution of Riluzole can reduce that rate of motor unit loss in familial amyotrophic lateral sclerosis (fALS).Motor unit numbers were estimated from the right abductor pollicis brevis (APB) and right extensor digitorum brevis (EDB) muscles.During the study period, we were able to detect loss of motor neurones in the pre-symptomatic phase of the disease.At the time of symptom onset, they were treated with Riluzole and in all the cases, there was "symptomatic" improvement as well as a positive change in MUNE numbers.Riluzole is not a disease altering agent but if given in the pre-symptomatic phase of the disease, before significant motor neurone loss has occurred, it may have some therapeutic benefit.This effect may have implications for the management of asymptomatic carriers of the SOD 1 gene, as they are at risk of developing fALS.
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