The Role of the Statistical Method of Motor Unit Number Estimation (MUNE) to Assess the Potential Therapeutic Benefits of Riluzole on Patients with Pre-symptomatic Familial Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which attacks the motor system. There is a family history in approximately 10% percent of cases and 20% of such families have point mutations in the Cu, Zn superoxide dimutase 1 (SOD1) gene. Pre-symp‐ tomatic loss of motor neurons has been identified prior to the onset of symptoms in SOD1 mice. This loss was biphasic with initial loss in the pre-symptomatic phase followed by a period of stabilisation and then gradual loss at time of weakness to death. (Kong & Xu, 1998).