Together, these results show quantitatively that sporadic ALS-TDP exhibits high intraindividual pathological homogeneity across the cortico-spinal neuraxis, and that non-TDP-43 proteinopathies are more likely to display significant lower motor neuron predominance than TDP-43 disease, which is in broad concordance with clinical presentations commonly seen for these genotypes. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.