BACKGROUND. Amyotrophic lateral sclerosis (ALS) is not limited only to motor impairment, and can also manifest with cognitive and behavioral disorders. Currently these presentations of ALS are unrecognized. Hence the research of ALS variants with cognitive and behavioral impairments is necessary. AIM. To study the frequency and clinical characteristics of ALS variants with cognitive and behavioral impairments. MATERIAL AND METHODS. The patients were recruited in 20202021. All patients with definite and possible ALS according to El Escorial criteria were included. The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) was used to assess cognitive and behavioral changes. 44 patients, 26 males and 18 females, aged 39 to 77 years (median of age 61 years, interquartile range 53.574.5). Statistical analysis was performed using IBM SPSS Statistics 21. Continuous data were analyzed using the MannWhitney test, categorical data was analyzed by exact Fisher criteria. A p-value less than 0.05 was statistically significant. RESULTS. In 43.2% of patients cognitive and/or behavioral changes were observed. The majority of patients (approximately 20%) suffered from a variant of ALS with cognit
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