An observational study of cognitive impairment in amyotrophic lateral sclerosis

Archives of Neurology
Gregory A RipponYaakov Stern

Abstract

Cognitive impairment is increasingly recognized in patients with amyotrophic lateral sclerosis (ALS). Clinical and pathologic features overlap in frontotemporal lobar dementia and ALS. Demographics, respiratory status, bulbar site of onset, and disease severity are potential risk factors for cognitive impairment in ALS. To further delineate the frequency, nature, and implications of cognitive impairment in ALS and to assess previously identified risk factors. Case-control and retrospective cohort study. Academic referral center. Forty consecutive patients with ALS underwent baseline neurologic and neuropsychologic examinations. Cognitive test performance was compared in patients with ALS and matched controls. An exploratory analysis of the relationship between cognitive performance and ALS survival was performed. Neuropsychologic test performance, ALS severity, and survival. Twelve patients (30%) showed evidence of cognitive impairment, including 9 (23%) who met the neuropsychologic criteria for dementia. No statistically significant differences were found between demented and nondemented ALS groups regarding demographics, family history, site of onset, bulbar dysfunction, or ALS severity. Only 1 patient with dementia had bulba...Continue Reading

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