Abstract
Atrophic lateral sclerosis (ALS) is a disease resulting from degeneration of motor neurons in the CNS and spinal cord. In 5 to 20% of cases it is an autosomal dominating hereditary disease. About 20% of patients suffering from the familial atrophic lateral sclerosis (FALS) have a point mutation in the gene coding copper-zinc superoxide dismutase (CuZnSOD). The authors suggest that the major role both in the etiology and progression of the ALS falls to reactive forms of oxygen, while the CuZnSOD dysfunction is a factor predestinating to this disease development.