Cognitive And Behavioral Impairment Screening In Patients With Amyotrophic Lateral Sclerosis

Background Amyotrophic lateral sclerosis(ALS)is a fatal motor neuron disorder that is,characterized by progressive loss of the upper and lower motor neurons.Although originally ALS was mainly described as a disorder that only affects motor system,the cognitive and behavioral impairment in ALS patients is increasingly recognized now.At present,ALS has become part of amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder(ALS-FTD spectrum disorder).Cognitive and behavioral impairment in ALS significantly increases caregiver burden and shorten survival of the patients.Therefore,it is crucial to detect cognitive and behavioral abnormalities in ALS patients as early as possible.Objective Explore the incidence rate and the probable risk factors of cognitive and behavioral impairment in ALS patients in the central China and thus to offer basis for predicting and managing these symptoms early.Methods We enrolled 97 patients with ALS(possible,probable,or definite ALS according to the El Escorial criteria)from the Department of Neurology,Tongji hospital affiliated to Tongji Medical College,Huazhong University of Science and Technology between December 2017 and December 2018.Primary caregivers of all the patients were interviewed for behavioral changes by Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire(ALS-FTD-Q).We compare and analyzed the relationship between different age,sex,onset of disease,body mass index(BMI),education,age,disease duration,severity and the incidence of behavioral impairment in ALS patients.74 inpatients were interviewed for cognitive changes by localization version of The Edinburgh Cognitive and Behavioral ALS Screen(ECAS).Meanwhile,we collected their serological results to analyze the probable risk factors of cognitive and behavioral impairment.Results Of the 97 patients who were assessed with the ALS-FTD-Q,59 were normal(60.8%)and 38(39.2%)were abnormal.The incidence rate of abnormal behaviors displayed no significant difference in ALS patients with regard to gender,age,site of onset and severity(P>0.05),while the distribution of behavioral impairment in different ALS patients grouping by BMI,disease duration,and education was statistically different(P < 0.05).Thirty one(41.9%)Of the 74 inpatients assessed with ECAS presented impairment of cognition.Bilirubin level between ALS patients with normal cognition and cognitive impairment was significant difference(P < 0.05),whereas no obvious difference in terms of the level of albumin,uric acid,creatinine and thyroid-stimulating hormone(P > 0.05).There is no difference between ALS patients with normal behavior and behavioral impairment in all serological results.Conclusion Approximately half of ALS patients exist cognitive and behavioral impairment.BMI,education level and disease duration may play a role in the occurrence of behavioral abnormalities.Bilirubin is of certain value in predicting the incidence of cognitive impairment.