Most patients with LRRK2 mutation exhibit neuropathological features consistent with typical Lewy body Parkinson's disease (Gilks et al., 2005; Khan et al., 2005; Zimprich et al., 2004); however, “pure” nigral degeneration, tau, α-synuclein, or ubiquitin pathologies resembling progressive supranuclear palsy (PSP), multiple system atrophy, and frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) have also been reported (Dachsel et al., 2007; Gaig et al., 2008; Giasson et al., 2006; Hasegawa et al., 2009; Rajput et al., 2006; Zimprich et al., 2004). This evidence concerns the gene MAPT and supranuclear palsy, progressive, 1.