Progression of cognitive and behavioral disturbances in motor neuron diseases assessed using standard and computer-based batteries

Abstract

Objective: Detecting and monitoring cognitive and behavioral deficits in motor neuron diseases (MND) is critical due to their considerable clinical impact. In this scenario, computer-based batteries may play an important role. In this study, we investigated the progression of cognitive and behavioral deficits in MND patients using both standard and computer-based neuropsychological batteries. Methods: This is a retrospective study on 74 MND patients (52 amyotrophic lateral sclerosis [ALS], 12 primary lateral sclerosis [PLS], and 10 progressive muscular atrophy [PMA]) who were followed up for 12 months and underwent up to three cognitive/behavioral assessments, 6 months apart, including standard and/or computerized based (the Test of Attentional Performance [TAP]) batteries. Behavioral/cognitive changes were investigated over time using generalized linear model for longitudinal data accounting for time and revised-ALS Functional Rating Scale. Results: Over 12 months, ALS patients showed a global cognitive decline (Mini Mental State Examination) at the standard battery and reduced performance in the alertness, sustained and divided attention, go/nogo, cross-modal and incompatibility TAP tasks. Most of these findings remained significant when ALSFRS-R changes over time were included as covariate in the analyses. ALS patients did not show significant behavioral abnormalities over time. No cognitive and behavioral changes were found in PLS and PMA cases. Conclusions: Computer-based neuropsychological evaluations are able to identify subtle cognitive changes in ALS, unique to this condition. This study highlights the need of specific, accurate and well-tolerated tools for the monitoring of cognitive deficits in MND.

Keywords:

• Motor neuron disease
• amyotrophic lateral sclerosis
• cognitive decline
• computer-based neuropsychological evaluation
• test of attentional performance

Data availability statement

The data that support the findings of this study are available from the corresponding author, upon reasonable request.

Disclosure statement

V. Castelnovo, N. Riva, B. Poletti, C. Cividini, A. Fontana, and F. Solca report no conflict of interest. E. Canu has received research supports from the Italian Ministry of Health. V. Silani received compensation for consulting services and/or speaking activities from AveXis, Cytokinetics, Italfarmaco, and Zambon; and receives research supports from the Italian Ministry of Health (Grant RF-2013-02355764), Fondazione Regione per la Ricerca Biomedica Regione Lombardia (Project nr.2015-0023), and E-RARE JTC 2018 (Project Repetomics). M. Filippi is Editor-in-Chief of the Journal of Neurology; received compensation for consulting services and/or speaking activities from Bayer, Biogen Idec, Merck-Serono, Novartis, Roche, Sanofi Genzyme, Takeda, and Teva Pharmaceutical Industries; and receives research support from Biogen Idec, Merck-Serono, Novartis, Roche, Teva Pharmaceutical Industries, Italian Ministry of Health, Fondazione Italiana Sclerosi Multipla, and ARiSLA (Fondazione Italiana di Ricerca per la SLA). F. Agosta is Section Editor of NeuroImage: Clinical; has received speaker honoraria from Biogen Idec, Novartis and Philips; and receives or has received research supports from the Italian Ministry of Health, AriSLA (Fondazione Italiana di Ricerca per la SLA), and the European Research Council.

Additional information

Funding

This work was supported by the Italian Ministry of Health under Grants RF-2010-2313220 and RF-2011-02351193.