The main findings of the present study were: (i) NF protein 70 kD subunit decreased dramatically in both nerve axons and NMJs in limb muscles but not in EOMs with ALS; (ii) synaptophysin decreased significantly at NMJs of ALS limb muscles but not of EOMs; (iii) S100B declined significantly in both limb and EOMs of ALS donors, in nerves as well as at NMJs; (iv) p75NTR decreased only at NMJs in ALS limb muscles but not in their nerves. Here, S100B is linked to amyotrophic lateral sclerosis.