TARDBP and motor neuron disorder: Consistent with the anatomical distribution of the neuropathological findings with prominent involvement of the anterior horns of the spinal cord, the patient at “stage 1 and brainstem ± spinal TDP-43 pathology” (patient #6, Tables 1, 2) and the patient with “stage 2 without TDP-43 pathology” (patient #11) showed, in addition to bulbar dysfunction, the clinical picture of motor neuron disease with marked muscle atrophy, paresis, and fasciculations.