Abstract
(1) Background: Cognitive features of patients with amyotrophic lateral sclerosis (ALS) have never been specifically analyzed according to the lateralization of motor impairment. In the present study we investigated the cognitive performances of ALS patients to describe the relationship between motor and cognitive dysfunction, according to site and side of disease onset. (2) Methods: Six-hundred and nine ALS patients underwent a comprehensive neuropsychological evaluation at diagnosis in Turin ALS Centre Tests included—mini-mental state examination (MMSE), frontal assessment battery (FAB), trail-making test A/B (TMT A-B), digit span forward and backward (digit span FW/digit span BW), letter fluency test (FAS), category fluency test (CAT), Rey auditory verbal learning test (RAVLT), Babcock story recall test (BSRT), Rey-Osterrieth complex figure test (ROCFT), Wisconsin card sorting test (WCST), Raven’s coloured progressive matrices (CPM47). Cognitive performances of patients, grouped by side and site of onset, were statistically compared using z-scores, as appropriate. (3) Results: Bulbar patients and bilateral spinal onset patients (Sbil) were generally characterized by lower cognitive performances in most neuropsychological tests, when compared to patients with lateralized onset (right-side spinal onset, Sri and left-side spinal onset, Sle). Digit span backward and visual memory task (ROCFT) median z-scores were significantly higher, reflecting a better cognitive performance, in Sri patients when compared to bulbar/Sbil patients, while verbal memory tasks (RAVLT and BRST) resulted in significantly higher scores in Sle patients. Our results are in keeping with hemispheric functional lateralization of language and visuospatial abilities. (4) Conclusions: In ALS patients, as in other neurodegenerative diseases, we found a direct relationship between lateralized motor and cognitive features.
Highlights
IntroductionAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that primarily involves all voluntary motor functions, through progressive degeneration of upper and lower motor neurons [1]
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that primarily involves all voluntary motor functions, through progressive degeneration of upper and lower motor neurons [1].It is characterized usually by a focal onset in limbs or bulbar muscles, where upper and lower motor signs are maximal [2], spreading through contiguous or network pathways [3]
The aim of the present study was to analyze the performance of a cohort of amyotrophic lateral sclerosis (ALS) patients in different cognitive domains according to the site and side of disease onset, and to identify the presence of specific cognitive patterns related to the lateralization of motor impairment
Summary
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that primarily involves all voluntary motor functions, through progressive degeneration of upper and lower motor neurons [1]. It is characterized usually by a focal onset in limbs or bulbar muscles, where upper and lower motor signs are maximal [2], spreading through contiguous or network pathways [3]. There are indications that cognitive function may worsen during disease progression [7], with a correlation to motor dysfunction and clinical stages [8] Despite this general correlation between motor and cognitive impairment, the patterns followed by neurodegeneration to spread across are not fully understood but seem to maintain a certain degree of lateralization [3]. An area that has been generally neglected in ALS is the notion that some cognitive functions, which can be impaired in the disease, are functionally lateralized, in particular language and visuospatial abilities [9]
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