SETX and amyotrophic lateral sclerosis: Overexpression of ELP3 has been suggested to be neuroprotective and reduce toxicity associated with mutant SOD1 and C9orf72 repeat expansion.64 Mutation in ELP3 has been shown to cause decreased survival in ALS and FTD patients with C9orf72 repeat expansion.65 The co-occurrence of FIG4 with SETX (p.L158V) or C9orf72 repeat expansions has also been linked with poor prognosis.66 The co-presence of FIG4 and ELP3 variants in our patient might have resulted in shorter survival duration..