Although family history was an exclusion criterion in previous MSA diagnostic criteria,4 they have been revised to be inclusive.7 Multiplex families have been described with mixed MSA and Lewy body disease presentations linked to rare COQ2 variants in Japanese families, although data supporting increased risk for MSA with common COQ2 variants remains equivocal.8,9 Recently, more risk loci in MSA were identified implicating GAB1, lnc-LRRC49-3, TENM2 and RABGEF1 in Europeans10 and PLA2G4C in meta-analysis of Asian and Caucasian MSA patients.11 This evidence concerns the gene TENM2 and multiple system atrophy.