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Cognitive disorders (CD) are observed in more than half of patients with amyotrophic lateral sclerosis (ALS), but rarely reach the degree of dementia. Currently, a classification of ALS has been proposed depending on the presence of cognitive and/or behavioral disorders. CD in ALS can be represented by speech fluency disorders, various aphasic disorders, disorders of regulatory functions, social cognitive functions, and verbal memory. The most diagnostically sensitive are special scales, such as the Edinburgh Cognitive and Behavioral Impairment Screening Scale for ALS. CD in ALS have a negative impact on the outcome of the disease and the quality of life of patients and their families. This issue requires further study.

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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 cognitive impairment, which was mostly presented with executive and social cognitive dysfunction. ALS with behavioral impairment was observed at 9% of patients, which mostly presented with apathy and disinhibition. A combination of cognitive and behavioral impairment was registered in 7% of patients. In another 7% of patients severity of cognitive and behavioral impairment reached a degree of dementia and corresponded to frontotemporal degeneration. CONCLUSION. This cognitive and behavioral impairment is observed in a substantial number of patients with ALS and mostly presented with moderate executive and social cognitive dysfunction, as well as apathy and less often disinhibition.