The FIG4 p.Ile41Thr missense, originally described in compound heterozygous in demyelinating autosomal recessive CMT neuropathy type 4 juvenile (CMT4J) patients [23,24,25,26], was also subsequently reported in ALS patients, with autosomal dominant transmission [8,18,27,28]. This evidence concerns the gene FIG4 and amyotrophic lateral sclerosis.