Recently, recessive ADPRHL2 (ADPRS) mutations, encoding the ARH3 protein, were shown to cause a congenital-onset neurodegenerative stress-induced (epileptic) ataxia syndrome with early pediatric onset (CONDSIAS) (Danhauser et al, 2018; Ghosh et al, 2018; Aryan et al, 2020). The gene discussed is ADPRS; the disease is neurodegeneration, childhood-onset, stress-induced, with variable ataxia and seizures.