ABSTRACT

Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder. The highest incidence is in adults aged 50-70 years, with men marginally more affected than women, and lies between 2 and 3 per 100,000 adults. The disease is sporadic in 90%–95%, 5%–10% of ALS patients present with the familiar form, and 5%–10% of these have a mutation in the gene coding for superoxide 1 (SOD1). The etiology of the disease remains unclear, even though a variety of pathomechanisms such as glutamate excitotoxicity, oxidative stress, lack of neurotrophic factors, defects in axonal transport, accumulation of intracellular aggregates, and aberrant RNA metabolism have been identišed.