Indeed, NFTs have been observed in both DM1 and DM2 brains in the amygdala, CA1, hippocampus, entorhinal cortex, and temporal cortex, then with a topographic distribution similar to that reported for moderate Alzheimer disease although the topographic progression of Tau pathology during DM1 has not yet been clearly established (Figure 1) (Yoshimura et al., 1990; Vermersch et al., 1996; Delacourte et al., 1999; Maurage et al., 2005). The gene discussed is MAPT; the disease is myotonic dystrophy type 1.