Since the neuropathological confirmation of cerebral TDP43 pathology within the complete spectrum of ALS phenotypes is limited by the lack of autopsies, the MRI-based staging technique has been applied to patients with progressive lower motor neuron disease [15, 16], with PLS [17, 18], and with progressive bulbar palsy [19]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.