The first mechanism is supported by the evolution of GM atrophy through time, as in neurodegenerative disorders,56 with Tau deposits involvement.5,6,57 Interestingly, neuropathological findings suggest tau aggregation, neurofibrillary tangles mostly in the hippocampus and the parahippocampus,5,6 both altered regions in our DM1 patients. Here, MAPT is linked to myotonic dystrophy type 1.