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BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a multisystem neurodegenerative disease encompassing cognitive and behavioral impairments. The Revised Diagnostic Criteria for ALS-frontotemporal spectrum disorder (ALS-FTDS), while widely adopted, may overlook subtle impairments such as memory and visuospatial deficits, limiting their prognostic value. OBJECTIVES: This study aimed to apply the Mild Behavioral and Neurocognitive Impairment (MBNI) approach, adapted from other neurodegenerative diseases, to ALS patients and assess its prognostic utility for survival and disease progression. METHODS: A prospective cohort of 201 ALS patients was evaluated between January 2018 and July 2024. Participants underwent comprehensive cognitive and behavioral assessments. The MBNI approach identified patients with mild cognitive impairment (MCI), mild behavioral impairment (MBI), or combined cognitive-behavioral impairment (MCBI). Prognostic value was analyzed using Kaplan-Meier survival curves, Cox proportional hazards models, and logistic regression for disease progression. RESULTS: Mild cognitive and/or behavioral impairments were detected in 67% of patients classified as cognitively normal by ALS-FTDS criteria. At a median follow-up of 15 months, these patients showed shorter tracheostomy-free survival (all p < 0.005). MCI (HR5.3; CI 1.10-25.41; p = 0.038) and frontotemporal dementia (HR6.2; Confidence Interval: 1.34-28.40; p = 0.019) independently predicted poor outcomes. Logistic regression confirmed that MCBI and frontotemporal dementia were associated with rapid progression (both p < 0.019). CONCLUSION: The MBNI approach enhances the detection of mild cognitive and behavioral impairments in ALS, providing prognostic insights and improving stratification over the Revised Diagnostic Criteria for ALS-FTDS. This framework supports personalized care and the design of clinical trials targeting early disease stages.
Spisto et al. (Thu,) studied this question.