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Are Amyotrophic Lateral Sclerosis patients cognitively normal?
Traditionally, cognition was felt to be spared in patients with Amyotrophic Lateral Sclerosis.
However, up to 50% of patients who have Amyotrophic Lateral Sclerosis have some cognitive impairment when detailed neuropsychologic evaluations are performed.
Frontal executive dysfunction (i.e., verbal fluency and attention) is the most common finding.
Other symptoms may range from mild behavioral impairment to up to 15% to 20% having severe cognitive impairment fulfilling the diagnostic criteria for frontotemporal dementia, especially given the genetic association with frontotemporal dementia.
Sources
- Brooks BR, Miller RG, Swash M, et al: El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1(5):293-299, 2000.
- Genetics Home Reference. ghr.nlm.nih.gov/condition/amyotrophic-lateral-sclerosis .
- DeJesus-Hernandez M, Mackenzie IR, Boeve BF, et al: Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 72(2):245-256, 2011.
