Figure 1 shows the history of Perry disease research. In 1975, Perry disease was initially reported and named by Perry et al. [4] in Canada. The identification of DCTN1 mutations in 2009 accelerated Perry disease research [5]. Just after the establishment of a new TAR DNA-binding protein 43 (TDP-43) proteinopathy [6,7], in 2018, international diagnostic criteria for Perry disease were created [8]. Perry disease generally produces uniform clinical features and characteristic pathological findings; therefore, in the same year, we proposed a name change from Perry syndrome to Perry disease [8]. This evidence concerns the gene TARDBP and proteostasis deficiencies.