Abstract
The goal of this study was to identify neurostructural frontal lobe correlates of cognitive and speaking rate changes in amyotrophic lateral sclerosis (ALS). 17 patients diagnosed with ALS and 12 matched controls underwent clinical, bulbar, and neuropsychological assessment and structural neuroimaging. Neuropsychological testing was performed via a novel computerized frontal battery (ALS-CFB), based on a validated theoretical model of frontal lobe functions, and focused on testing energization, executive function, emotion processing, theory of mind, and behavioral inhibition via antisaccades. The measure of speaking rate represented bulbar motor changes. Neuroanatomical assessment was performed using volumetric analyses focused on frontal lobe regions, postcentral gyrus, and occipital lobes as controls. Partial least square regressions (PLS) were used to predict behavioral (cognitive and speech rate) outcomes using volumetric measures. The data supported the overall hypothesis that distinct behavioral changes in cognition and speaking rate in ALS were related to specific regional neurostructural brain changes. These changes did not support a notion of a general dysexecutive syndrome in ALS. The observed specificity of behavior-brain changes can begin to provide a framework for subtyping of ALS. The data also support a more integrative framework for clinical assessment of frontal lobe functioning in ALS, which requires both behavioral testing and neuroimaging.
Highlights
Amyotrophic lateral sclerosis (ALS) is a multisystem disorder characterized by neurodegeneration of upper and lower motor neurons as well as extramotor pathways [1]
The measures that statistically distinguished ALS from the control group are marked with an asterisk
Superior medial and superior frontal regions approximated the regions that are associated with the specific cognitive processes in the Stuss model
Summary
Amyotrophic lateral sclerosis (ALS) is a multisystem disorder characterized by neurodegeneration of upper and lower motor neurons as well as extramotor pathways [1]. ALS is a rapidly progressing and highly debilitating condition, with motor neurodegeneration impacting both spinal (i.e., arm, trunk, and leg) and bulbar (i.e., speech and swallowing) musculatures. Bulbar motor signs and symptoms are associated with a disease phenotype with shorter survival and an overall more debilitating course, including higher association with upper motor neuron dysfunction and extramotor deficits [2,3,4]. Extramotor impairments in ALS impact cognitive functions across multiple domains including executive functions, social cognition, language, and memory. The profile of behavioral changes in ALS has been labelled as the “frontal dysexecutive syndrome” [5,6,7,8]. Limited knowledge exists systematically linking clinical symptomology to the underlying neurostructural changes
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