Abstract
BackgroundCognitive impairment is associated with a negative prognosis in amyotrophic lateral sclerosis (ALS), as well as with clinical specificity. We investigate neuropsychological function in ALS patients without known genetic mutations in a Korean tertiary clinic.MethodsThree hundred and eighteen patients were enrolled in a prospective longitudinal cohort from September 2008 to February 2012. At the time of diagnosis of sporadic ALS, we carried out genetic and comprehensive neuropsychological tests on all patients, and collected demographic and clinical characteristics. Six cognitive domains, namely executive function, attention, language, calculation, visuospatial function and memory were evaluated. ANOVA and t-tests were used to assess differences in clinical characteristics and neuropsychological parameters between sporadic ALS patients. The Kaplan-Meier method and Cox proportional hazard model were used for survival analysis.ResultsOne hundred and sixty-six patients were categorized into five subtypes: normal cognition (ALS pure), cognitive impairment (ALSci), behavioral impairment (ALSbi), frontotemporal dementia (ALS-FTD), and other types of dementia. Seventy patients (70/166, 42.2%) were cognitively or behaviorally impaired. Among the impaired patients, eight (8/166, 4.8%) had FTD-type dementia and one (1/166, 0.6%) was Alzheimer's disease-type. The ALS patients with cognitive impairment (ALSci) and with FTD (ALS-FTD) were more severely impaired in executive function, attention, language and memory than the cognitively intact ALS patients (ALS pure). In a survival analysis, ALSci (β = 1.925, p = 0.025) and ALS-FTD groups (β = 4.150, p = 0.019) tended to have shorter survival than the ALS pure group.ConclusionsAbout half of ALS patients without known genetic variation have cognitive or behavioral impairment. ALS patients with cognitive abnormalities, especially FTD, have a poorer prognosis than those without cognitive impairment. In neuropsychological profiling, executive tasks were effective in identifying cognitive impairment in the ALS patients. It would be useful for clinicians to classify ALS according to neuropsychological profiles, and screen for subtle cognitive impairment.
Highlights
Amyotrophic lateral sclerosis (ALS) involves primarily motor system degeneration [1,2,3,4]
Most patients with amyotrophic lateral sclerosis (ALS) have mild cognitive impairment with subtle executive deficits, and 5% have a clinical subtype of frontotemporal lobar degeneration (FTLD) called frontotemporal dementia (FTD) [10,11]
The majority of ALSFTD patients presented with behavioral changes typical of behavioral variant FTD (n = 4), and language difficulties typical of semantic dementia (SD) (n = 3)
Summary
Amyotrophic lateral sclerosis (ALS) involves primarily motor system degeneration [1,2,3,4]. Dominated by motor dysfunction, there is increasing evidence that ALS is a multisystem disorder which affects the extra-motor cortex, cognition, behavior and extrapyramidal system [5,6,7,8]. Most patients with ALS have mild cognitive impairment with subtle executive deficits, and 5% have a clinical subtype of frontotemporal lobar degeneration (FTLD) called frontotemporal dementia (FTD) [10,11]. Several reports have suggested that ALS patients with cognitive and behavioral impairment have a poorer prognosis than those without cognitive and behavioral impairment [5,6]. Cognitive impairment is associated with a negative prognosis in amyotrophic lateral sclerosis (ALS), as well as with clinical specificity. We investigate neuropsychological function in ALS patients without known genetic mutations in a Korean tertiary clinic
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