Cognitive functioning in sporadic amyotrophic lateral sclerosis: a six month longitudinal study

• Published in: Journal of neurology, neurosurgery and psychiatry Vol. 77; no. 5; pp. 668 - 670
• Main Authors: Robinson, K M, Lacey, S C, Grugan, P, Glosser, G, Grossman, M, McCluskey, L F
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd 01.05.2006; BMJ; BMJ Publishing Group LTD; BMJ Group
• Subjects: Age; Aged; ALS; ALSA; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis Association; Biological and medical sciences; cognition; Cognition & reasoning; Cognition Disorders - diagnosis; Cognition Disorders - psychology; Cognitive ability; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dementia; Disease Progression; Education; Female; frontotemporal dementia; FTD; Humans; Longitudinal Studies; Male; Medical sciences; Memory; Middle Aged; Motor Neuron Disease - diagnosis; Motor Neuron Disease - psychology; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; Neurology; Neuropsychological Tests; Recruitment; Reference Values; Short Report; Studies; Amyotrophic lateral sclerosis; Nervous system diseases; Degenerative disease; Sporadic; Spinal cord disease; Central nervous system disease
• ISSN: 0022-3050; 1468-330X
• DOI: 10.1136/jnnp.2005.073403

Objective: To observe changes in cognition over six months in subjects with recently diagnosed sporadic amyotrophic lateral sclerosis (ALS). Methods: The study used a between-group and within-group longitudinal design. Nineteen ALS subjects and eight matched caregivers were recruited to participate in baseline neuropsychological assessments that were repeated six months later. Between group comparisons for these variables were undertaken at baseline and six months later. Within group/across time comparisons for these variables were carried out for both groups. Individual analyses for the neuropsychological variables using z scores were done for the ALS subjects using their baseline performance as the basis for comparison with their six month performance. Results: The between-group and within-group comparisons did not show significant differences in cognitive function over time. In individual analyses, however, seven of 19 ALS subjects (36.84%) developed abnormal neuropsychological performance over six months. Conclusions: Early in the disease course, over one third of the ALS subjects developed cognitive deficits over six months. These findings support the hypothesis that cognitive deficits in ALS become more prominent over time.