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Updated: Jul 11, 2026
06:46
Automated, Long-term Behavioral Assay for Cognitive Functions in Multiple Genetic Models of Alzheimer's Disease, Using IntelliCage
Published on: August 4, 2018
Cognitive impairment in familial ALS
M W Wheaton1, A R Salamone, D M Mosnik
1Department of Neurology, NB-302, Baylor College of Medicine, 6501 Fannin, Houston, TX 77030, USA.
Neurology
|October 3, 2007
Summary
Cognitive impairment is common in both familial Amyotrophic Lateral Sclerosis (fALS) and sporadic ALS (sALS), with similar patterns observed. Age, not symptom onset, correlated with impairment in fALS patients.
Area of Science:
- Neuroscience
- Neurology
- Genetics
Background:
- Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease impacting motor neurons.
- Emerging evidence indicates cognitive deficits in sporadic ALS (sALS) patients.
Purpose of the Study:
- To investigate cognitive impairment in familial ALS (fALS) patients.
- To compare the type and degree of cognitive impairment in fALS with that in sALS.
Main Methods:
- Neuropsychological testing was administered to 37 fALS patients.
- Cognitive impairment was assessed using cluster analysis, cutoff scores, and clinical judgment.
Main Results:
- Cognitive impairment was observed in 62% of fALS patients and 48.5% of sALS patients (not statistically different).
- Motor scores and symptom onset location did not correlate with cognitive impairment.
- Age was the only significant factor differentiating affected and unaffected fALS groups.
Conclusions:
- The prevalence and characteristics of cognitive impairment in fALS are comparable to sALS.
- In fALS, age is a correlating factor, while symptom onset is not.
- Further research will explore genetic links to cognitive impairment in fALS.
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