Stage I ALS TDP-43 pathology has been located in the αMNs of the ventral spinal cord, a region discussed in this review, as well as in the agranular motor cortex and the bulbar MNs of cranial nerves V, VII, X, XI, XII (Brettschneider et al., 2013). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.