Though this may be confused with ALS due to the age of onset, muscle weakness, and even evidence of neuropathic changes usually consistent with motor neuron degeneration, electromyography shows myopathic changes in affected individuals, and histological analysis of affected muscles by biopsy shows myonuclear and sarcoplasmic inclusion bodies reactive with ubiquitin and TDP-43 (Nalbandian et al., 2011). Here, TARDBP is linked to amyotrophic lateral sclerosis.