Frontal assessment battery and frontal atrophy in amyotrophic lateral sclerosis

Objectives: To determine the potential utility of the frontal assessment battery (FAB)in assessing cognitive impairments in amyotrophic lateral sclerosis (ALS), we investigatedthe association between the FAB score and regional gray matter volume, andascertained whether the regional brain alterations related to cognitive impairmentsoccur in relatively mild stage of ALS.Materials and Methods: Twenty-fourALS patients with a Mini-MentalStateExamination score of >23, a normal score on the Self-RatingDepression Scale, little orno disturbance in speech and handling utensils on the ALS Functional Rating Scale(ALSFRS), and normal measures on respiratory tests (respiratory function test and arterialblood gas analysis), and two age-matchednormal control groups (one for FABassessment and the other for brain morphometry) underwent FAB testing and structuralmagnetic resonance imaging. We applied voxel-basedmorphometry to investigatethe relationship between the FAB score and regional brain alteration, andassessed the relationship between the altered regional brain volume and ALSFRS orrespiratory tests.Results: Frontal assessment battery scores were significantly lower in ALS patientsthan in normal controls. Volume reduction in the right orbitofrontal gyrus in ALS wascorrelated with a lower FAB score. There was no correlation between the right orbitofrontalgyrus volume and ALSFRS or respiratory tests.Conclusions: The results suggest that the FAB is an adequate tool for detecting cognitiveimpairments related to frontal lobe pathology in the relatively mild stage of ALS,independent of physical dysfunctions

Correlation of frontal atrophy with behavioral changes in amyotrophic lateral sclerosis

Background: Recent studies have linked cognitive impairment in amyotrophic lateral sclerosis (ALS) to frontotemporal pathology. Aim: We examined possible associations between behavior changes and regional gray matter volume in early ALS and assessed whether the volume changes were independent of physical impairments. Methods: Seventeen ALS patients with Mini-Mental State Examination ≥24, no need for assistance in daily life, normal respiratory tests (respiratory function test and arterial blood gas analytes), and eleven age-matched controls, underwent structural MRI. Behavioral changes were assessed with family-rating Frontal Systems Behavior Scale (FrSBe). We applied voxel-based morphometry to investigate the correlation between FrSBe and gray matter volume and assessed the correlation of volume with ALS Functional Rating Scale (ALSFRS) and respiratory tests. Results: Current FrSBe were significantly higher than retrospective scores assessing the status before onset, most notably in apathy. The volumes of right middle and left medial frontal gyri in ALS were negatively correlated with FrSBe, whereas they were not correlated with ALSFRS and respiratory tests. The volume of right frontal cluster, but not left medial frontal cluster, was significantly smaller than that of controls. Conclusions: Regional atrophy within frontal lobe was associated with behavioral dysfunction in early ALS, this association was independent of physical factors