title: The range and clinical impact of cognitive impairment in French patients with ALS: a cross-sectional study of neuropsychological test performance.
abstract: Our objective was to assess the spectrum and clinical associations of cognitive impairment in French patients with ALS, and determine the effect of cognitive impairment on survival in this population. One hundred and thirty-one patients were enrolled in a cross-sectional cohort study of neuropsychological test performance. ANOVA and χ(2) tests assessed differences in clinical characteristics between impaired and unimpaired patients; multiple regression determined which features contributed most strongly to cognitive status, and Cox models compared survival. Fifty-three patients (40%) were categorized as cognitively impaired based on test performance. Thirteen (10%) patients had frontotemporal dementia (FTD) clinically; all scored in the moderate to severely impaired range on testing. Impaired patients had less education (p = 0.001), and severely impaired patients were more likely to have bulbar onset than unimpaired patients (p < 0.001). Severe cognitive impairment predicted shorter survival (p = 0.007), even when controlled for motor severity (p = 0.001). In summary, 10% of a consecutive series of French ALS patients had overt dementia and 40% were cognitively impaired by neuropsychological testing. We conclude that lower education level and possibly bulbar-onset ALS were associated with impairment. As in other causes of dementia, higher education attainment may protect against clinical cognitive deterioration in ALS. French patients with severe cognitive impairment have shorter survival time.
authors.affiliation: AP-HP, Centre référent maladies rares SLA, Département de Neurologie. paul.gordon@psl.aphp.fr
authors.forename: Paul H
authors.initials: PH
authors.lastname: Gordon
authors.forename: Daniel
authors.initials: D
authors.lastname: Delgadillo
authors.forename: Ambre
authors.initials: A
authors.lastname: Piquard
authors.forename: Gaelle
authors.initials: G
authors.lastname: Bruneteau
authors.forename: Pierre-François
authors.initials: PF
authors.lastname: Pradat
authors.forename: François
authors.initials: F
authors.lastname: Salachas
authors.forename: Christine
authors.initials: C
authors.lastname: Payan
authors.forename: Vincent
authors.initials: V
authors.lastname: Meininger
authors.forename: Lucette
authors.initials: L
authors.lastname: Lacomblez
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000453:epidemiology* / Q000523:psychology*; D003072:Cognition Disorders / Q000175:diagnosis / Q000453:epidemiology* / Q000523:psychology*; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D005260:Female; D005500:Follow-Up Studies; D005602:France / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D015996:Survival Rate / Q000639:trends