Table 1

Demographic and clinical data of control subjects and patient groups

ControlSporadic amyotrophic lateral sclerosisAmyotrophic lateral sclerosis patients homozygous for aspartate-to-alanine mutations in codon 90
Gender (male:female)12:814:61:6
Age (years)53±1656±1150±11
Site of disease onsetNA16 limb7 limb
4 bulbar
Disease duration (months)NA28±1842±47
Amyotrophic lateral sclerosis functional rating scaleNA38±637±7
Ashworth spasticity scoreNA2±39±2
Upper motor neuron burden scoreNA8±69±2