An alternative pathogenetic mechanism for SCA10 is bidirectional transcription producing toxic antisense transcripts or repeat-associated non-ATG (RAN) translation, as observed in DM1, DM2, fragile X-associated tremor/ataxia syndrome (FXTAS), C9orf72 amyotrophic lateral sclerosis and frontotemporal dementia ALS/FTD, and SCA8 (Zu et al., 2011; Ash et al., 2013; Todd et al., 2013; Zu et al., 2013; Zu et al., 2017). Here, ATXN8OS is linked to frontotemporal dementia.