The apolipoprotein E (apoE) gene plays an important role in forming predisposition and modulating the course of Alzheimer's disease, primary parkinsonism and some other human neurodegenerative disorders. In this study, for the first time in the Russian population, we performed the analysis of genetic association of apoE gene variants in 62 patients with a sporadic form of amyotrophic lateral sclerosis (ALS) aged from 20 to 75 years (49.5+/-14.2); age at disease onset was from 18 to 74 years (47.1+/-15.2). No statistically significant differences in the distribution of apoE genotypes between patients and controls were found. It was shown that the frequency of the apoE-e2 allele was significantly higher in the young group characterized by more benign course of ALS (spinal variant, slow rate of progression of ALS and longer survival) as well as in patients with a more "benign" spinal form of the disease. On the contrary, the chance of carrying the apoE-e4 allele is higher in the older age group in which a prognostically more severe bulbar form is also more prevalent. Therefore, the apoE-e2 allele may be regarded as a neuroprotective factor in the development of neurodegenerative process in patients with ALS.