Abstract
BackgroundIt has reached a consensus that patients with amyotrophic lateral sclerosis (ALS) could display cognitive impairment characterized by executive dysfunction or even dementia, but cognitive spectrum of Chinese patients with ALS still waits to be documented.MethodsA total of 106 incident patients with sporadic ALS were enrolled and comprehensive neuropsychological tests covering memory, executive function, attention, language, and visuospatial function were administered to them. Neuropsychological performances of 76 age- and education- matched healthy controls were used for the purpose of classification and comparison.Results106 patients were categorized into 4 subtypes:84 (79.2%) ALS with normal cognition (ALS-NC), 12 (11.3%) ALS with executive cognitive impairment (ALS-ECI), 5 (4.7%) ALS with non-executive cognitive impairment (ALS-NECI), and 5 (4.7%) ALS with frontotemporal lobe degeneration (ALS-FTLD). Under the same criteria, 2 (2.6%) and 1 (1.3%) healthy controls were diagnosed as ECI and NECI, respectively. The proportion of ECI was significantly higher in non-demented ALS than that in healthy controls, but it was not for NECI. Patients with ALS-FTLD had significantly severer bulbar function and older age than those with ALS-NC.ConclusionComorbid FTLD occurred in around 5% of Chinese sporadic ALS cases. Different genetic background and unique age distribution of Chinese ALS patients might be the reasons for the relatively low rate of comorbid FTLD. Cognitive dysfunction, predominant but not exclusive in executive area, was present in around 16% of non-demented ALS patients.
Highlights
Amyotrophic lateral sclerosis (ALS) is a progressive neurological disorder pathologically characterized by loss of motor neurons in spinal cord, brain stem and motor cortex of brain, leading to clinically variable combinations of dysarthria, muscle atrophy, limb weakness and pyramidal signs
106 patients were categorized into 4 subtypes:84 (79.2%) amyotrophic lateral sclerosis (ALS) with normal cognition (ALSNC), 12 (11.3%) ALS with executive cognitive impairment (ALS-ECI), 5 (4.7%) ALS with non-executive cognitive impairment (ALS-NECI), and 5 (4.7%) ALS with frontotemporal lobe degeneration (ALS-frontotemproal lobe degeneration (FTLD))
2 (2.6%) and 1 (1.3%) healthy controls were diagnosed as ECI and NECI, respectively
Summary
Amyotrophic lateral sclerosis (ALS) is a progressive neurological disorder pathologically characterized by loss of motor neurons in spinal cord, brain stem and motor cortex of brain, leading to clinically variable combinations of dysarthria, muscle atrophy, limb weakness and pyramidal signs. The diagnostic axis for ALS based on cognitivestatushas been established [6], and several studies have described cognitive spectrum of ALS patients in their own country [7,8,9,10] Despite these attempts and advances in this field, problems remain to be solved: most relevant studies were carried out in Europe and North America, whereas the data of Chinese population turned to be rather limited. It has reached a consensus that patients with amyotrophic lateral sclerosis (ALS) could display cognitive impairment characterized by executive dysfunction or even dementia, but cognitive spectrum of Chinese patients with ALS still waits to be documented.
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