Loss of cellular ARH3 activity, recently described in patients with the autosomal recessive disorder stress-induced childhood-onset neurodegeneration with variable ataxia and seizures (CONDSIAS), was linked with episodic infection-/stress-associated neurological deterioration resulting in impaired or declining cognitive development, physical impairments including muscle weakness, seizures and gait ataxia, and in several cases childhood lethality (Danhauser et al., 2018; Ghosh et al., 2018). This evidence concerns the gene ADPRS and neurodegeneration, childhood-onset, stress-induced, with variable ataxia and seizures.