Heterozygous missense mutations in the gene encoding the triggering receptor expressed on myeloid cells 2 (TREM2) have been recently described to significantly increase the risk of late onset Alzheimer's disease (AD) with an odds ratio similar to that of carrying an apolipoprotein E (APOE) ε4 allele (Guerreiro et al, 2013a; Jonsson et al, 2013). This evidence concerns the gene TREM2 and early-onset autosomal dominant Alzheimer disease.