SHORT REPORT

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

K M Robinson¹, S C Lacey², P Grugan³, G Glosser⁴, M Grossman⁴ and L F McCluskey⁵

¹ Department of Rehabilitation Medicine, University of Pennsylvania Health System, Pennsylvania Hospital, Philadelphia, Pennsylvania, USA
² Department of Psychology, University of Michigan, Ann Arbor, Michigan, USA
³ Department of Medicine, Division of Geriatric Medicine, University of Pennsylvania Health System, Philadelphia
⁴ Department of Neurology, University of Pennsylvania Health System, Hospital of the University of Pennsylvania, Philadelphia
⁵ Department of Neurology, Penn Neurological Institute at Pennsylvania Hospital, University of Pennsylvania Health System, Philadelphia

Correspondence to:
Correspondence to:
Dr Keith M Robinson
University of Pennsylvania Health System, Department of Rehabilitation Medicine, Pennsylvania Hospital, 801 Spruce Street, 3rd Floor, Philadelphia, PA 19107, USA; kerobi{at}pahosp.com

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.

Abbreviations: ALS, amyotrophic lateral sclerosis; ALSA, Amyotrophic Lateral Sclerosis Association; FTD, frontotemporal dementia

Keywords: amyotrophic lateral sclerosis; cognition

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