Individuals with VCP mutations present with proximal and distal muscle paresis, similar to limb girdle muscular dystrophy, and muscle biopsy showing myopathic features included a change in muscle fiber size and rimmed vacuoles [9]; however, mutations in VCP can also lead to ALS, including two of the same mutations that had been previously linked to IBMPFD [6, 23]. This evidence concerns the gene VCP and amyotrophic lateral sclerosis.