Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease, which is characterised by a loss of cerebral and spinal motor neurons (with saving of extraocular and sphincter muscles). It usually manifests itself in the 6th or 7th decade of life, but can also occur much earlier. The disease has several clinical forms, a traditional form of amyotrophic lateral sclerosis (ALS) with the involvement of central and peripheral motor neurons, progressive bulbar paralysis, and progressive (spinal) muscle atrophy (PSA) and primary lateral sclerosis. The traditional treatment is ineffective in most cases and fails to result in an improvement of the health, or relief from symptoms. Amino acid compounds may stop neurodegenerative processes of the neurons and temporarily reverse the course of the disease. They are active in the synaptic ending, regulate glutamate levels and have an inhibitory effect on the postsynaptic phase of the transfer. They help to improve the overall condition and reduce symptoms of the amyotrophic lateral sclerosis (ALS) according to the individual forms. Amino acid compounds improve the quality of life of patients and prolong their life expectations.