Abstract

ObjectivesTo determine the potential utility of the frontal assessment battery (FAB) in assessing cognitive impairments in amyotrophic lateral sclerosis (ALS), we investigated the association between the FAB score and regional gray matter volume, and ascertained whether the regional brain alterations related to cognitive impairments occur in relatively mild stage of ALS.Materials and MethodsTwenty‐four ALS patients with a Mini‐Mental State Examination score of >23, a normal score on the Self‐Rating Depression Scale, little or no disturbance in speech and handling utensils on the ALS Functional Rating Scale (ALSFRS), and normal measures on respiratory tests (respiratory function test and arterial blood gas analysis), and two age‐matched normal control groups (one for FAB assessment and the other for brain morphometry) underwent FAB testing and structural magnetic resonance imaging. We applied voxel‐based morphometry to investigate the relationship between the FAB score and regional brain alteration, and assessed the relationship between the altered regional brain volume and ALSFRS or respiratory tests.ResultsFrontal assessment battery scores were significantly lower in ALS patients than in normal controls. Volume reduction in the right orbitofrontal gyrus in ALS was correlated with a lower FAB score. There was no correlation between the right orbitofrontal gyrus volume and ALSFRS or respiratory tests.ConclusionsThe results suggest that the FAB is an adequate tool for detecting cognitive impairments related to frontal lobe pathology in the relatively mild stage of ALS, independent of physical dysfunctions.

Highlights

  • Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the progressive loss of upper and lower motor neurons leading to limb paralysis, dysphagia, dysarthria, and respiratory failure

  • A recent study proposed the following classification for frontotemporal syndromes in amyotrophic lateral sclerosis (ALS): ALS-­ frontotemporal dementia (FTD), which meets the Neary criteria for FTD (Neary et al, 1998); ALS with behavioral impairment (ALSbi), in which behavioral features are associated with frontotemporal dysfunction; and ALS with cognitive impairment (ALSci), in which impairment is seen in scores on neuropsychological tests sensitive to executive dysfunction (Goldstein & Abrahams, 2013; Murphy et al, 2007)

  • The aim of this study was to investigate the relationship between frontal cognitive dysfunctions detected by the frontal assessment battery (FAB) and brain volumes in ALS patients without dementia or depression, and to determine whether the anatomical changes associated with frontal cognitive dysfunctions are influenced by physical and emotional changes related to ALS

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Summary

| INTRODUCTION

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the progressive loss of upper and lower motor neurons leading to limb paralysis, dysphagia, dysarthria, and respiratory failure. A recent study proposed the following classification for frontotemporal syndromes in ALS: ALS-­ FTD, which meets the Neary criteria for FTD (Neary et al, 1998); ALS with behavioral impairment (ALSbi), in which behavioral features are associated with frontotemporal dysfunction; and ALS with cognitive impairment (ALSci), in which impairment is seen in scores on neuropsychological tests sensitive to executive dysfunction (Goldstein & Abrahams, 2013; Murphy et al, 2007). Cognitive impairments as assessed by neuropsychological tests of frontal function in ALS patients may reflect task difficulty due to motor impairment (Goldstein & Abrahams, 2013). The aim of this study was to investigate the relationship between frontal cognitive dysfunctions detected by the FAB and brain volumes in ALS patients without dementia or depression, and to determine whether the anatomical changes associated with frontal cognitive dysfunctions are influenced by physical and emotional changes related to ALS

| MATERIALS AND METHODS
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| DISCUSSION
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