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The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study
Background Despite considerable interest, the population-based frequency, clinical characteristics and natural history of cognitive impairment in amyotrophic lateral sclerosis (ALS) are not known.Expand
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Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study
Summary Background Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of upper and lower motor neurons, associated with frontotemporal dementia (FTD) in about 14% ofExpand
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Delineating the genetic heterogeneity of ALS using targeted high-throughput sequencing
Background Over 100 genes have been implicated in the aetiology of amyotrophic lateral sclerosis (ALS). A detailed understanding of their independent and cumulative contributions to disease burdenExpand
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Executive dysfunction is a negative prognostic indicator in patients with ALS without dementia
Background: The prognostic implications of cognitive impairment in amyotrophic lateral sclerosis (ALS) are not established. Objectives: To investigate the survival effect of the comorbidExpand
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Cognitive changes predict functional decline in ALS
Objective: To determine whether cognitive status in patients with amyotrophic lateral sclerosis (ALS) is a useful predictor of attrition and motor and cognitive decline. Methods: Cognitive testingExpand
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Multiparametric MRI study of ALS stratified for the C9orf72 genotype
Objective: To describe the patterns of cortical and subcortical changes in amyotrophic lateral sclerosis (ALS) stratified for the C9orf72 genotype. Methods: A prospective, single-center,Expand
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Grey matter correlates of clinical variables in amyotrophic lateral sclerosis (ALS): a neuroimaging study of ALS motor phenotype heterogeneity and cortical focality
Background Body region of onset and functional disability are key components of disease heterogeneity in amyotrophic lateral sclerosis (ALS). Objectives To evaluate patterns of grey matter pathologyExpand
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Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis
Background The population rate of familial amyotrophic lateral sclerosis (FALS) is frequently reported as 10%. However, a systematic review and meta-analysis of the true population based frequency ofExpand
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Basal ganglia involvement in amyotrophic lateral sclerosis
Objectives: To characterize the nature and extent of basal ganglia involvement in amyotrophic lateral sclerosis (ALS) genotypes in vivo. Methods: Forty-four healthy controls and 39 patients with ALSExpand
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Proposed criteria for familial amyotrophic lateral sclerosis
There is currently no consensus on the defi nition of familial ALS (FALS). We propose criteria for FALS that incorporate family history and genetic analysis. The aim is to increase the yield of genesExpand
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