In keeping with previous observations (Gregory et al., 2008) a striking aspect of our cohort of NBIA type 2 cases with PLA2G6 mutations and consisting of young individuals (age at death ranging between 8 and 36 years), is the presence of alpha-synuclein pathology, which was severe in 3 cases and in 2 of the postmortem cases it corresponded to that seen in end stage Parkinson's disease and dementia with Lewy bodies (Halliday et al., 2008; McKeith et al., 2005). This evidence concerns the gene PLA2G6 and dementia.