Abstract
ALS is predominantly a disease of the motor system, but cognitive and behavioral symptoms also are observed. DT MR imaging is sensitive to microstructural changes occurring in WM tracts of patients with ALS. In this study, we investigated the association between cognitive functions and extramotor WM tract abnormalities in ALS patients. DT MR imaging was obtained from 16 nondemented patients with ALS and 15 healthy controls. Patients with ALS underwent a neuropsychologic and behavioral evaluation. DT tractography was used to asses the integrity of the CST, corpus callosum, and the major long-range association tracts. The relationship between DT MR imaging metrics and cognitive functions was tested by using linear model analyses, adjusting for age and clinical disability. Eleven patients (69%) scored below the fifth percentile in at least 1 cognitive test, and 2 of them had a mild executive impairment. Performances at tests assessing attention and executive functions correlated with DT MR imaging metrics of the corpus callosum, CST, and long association WM tracts bilaterally, including the cingulum, inferior longitudinal, inferior fronto-occipital, and uncinate fasciculi. Verbal learning and memory test scores were associated with fornix DT MR imaging values, whereas visual-spatial abilities correlated with left uncinate fractional anisotropy. WM tract degeneration is associated with neuropsychologic deficits in patients with ALS. DT tractography holds promise to gain insight into the role of the brain WM network abnormalities in the development of cognitive impairment in patients with ALS.
Highlights
MethodsDT MR imaging was obtained from 16 nondemented patients with ALS and 15 healthy controls
AND PURPOSE: ALS is predominantly a disease of the motor system, but cognitive and behavioral symptoms are observed
Performances at tests assessing attention and executive functions correlated with DT MR imaging metrics of the corpus callosum, CST, and long association WM tracts bilaterally, including the cingulum, inferior longitudinal, inferior fronto-occipital, and uncinate fasciculi
Summary
DT MR imaging was obtained from 16 nondemented patients with ALS and 15 healthy controls. Patients with ALS underwent a neuropsychologic and behavioral evaluation. Written informed consent was obtained from each participant before study enrollment. Subjects Patients with sporadic ALS were recruited consecutively. Patients were excluded if they had cerebrovascular disorders, history of traumatic brain injury, hydrocephalus, or intracranial mass; other neurologic diseases; psychiatric and major medical disorders; psychotropic medication; or history of substance abuse. ALS disease severity was assessed by the ALSFRS-R questionnaire.. Muscle strength was scored by using the Medical Research Council scale from 0 to 5 (higher score indicating greater muscle strength).. UMN involvement was assessed by totaling the number of pathologic UMN signs on examination (higher score indicating more UMN signs; maximum total score ϭ 16). ALS disease severity was assessed by the ALSFRS-R questionnaire. Muscle strength was scored by using the Medical Research Council scale from 0 to 5 (higher score indicating greater muscle strength). UMN involvement was assessed by totaling the number of pathologic UMN signs on examination (higher score indicating more UMN signs; maximum total score ϭ 16).
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