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Amyotrophic Lateral Sclerosis (ALS) is a clinically and genetically heterogeneous, late-onset, neurodegenerative disorder of the motor system. Five to ten percent of cases are familial and about 20% of these cases have point mutations in the Cu/Zn superoxide dismutase 1 (SOD-1) gene. Since its discovery, mutations in Cu/Zn superoxide dismutase (SOD-1) have stimulated a huge amount of interest, but the pathogenic mechanisms underlying disease’s induction in familiar cases are still elusive. The most accepted hypothesis is that familiar ALS, SOD-1 positive could be caused by a neuronal damage, due to a gradual accumulation of a toxic product SOD-1 this cumulative damage leads to a disruption of the cytoskeleton and organelle trafficking within motor neuron dendrites. Aggregates do not exclusively occur in neurons, but also in glial cells, raising the question of whether mutant SOD-1 expression in neurons is sufficient per se to induce pyramidal degeneration and sustain disease evolution over time. The familial form is clinically indistinguishable from the sporadic one and to date only few studies have tried to highlight electromyographic differences between sporadic and familiar ALS forms.
In Motor Neuron Diseases (MND), standard needle electromyography often reveals evidence of chronic reinnervation (increased motor unit action potential amplitudes and duration, with reduced recruitment), eventually associated with fasciculations and signs of denervation activity in progress, but provides little information about the extent of both motor neuron loss and axonal regeneration.