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The cognitive profile of ALS: a systematic review and meta-analysis update
Beeldman E, Raaphorst J, Klein Twennaar M, de Visser M, Schmand BA, de Haan RJ.. Journal of neurology, neurosurgery, and psychiatry, 2015-08-17
Topic: Risk, prevention and care in population studies
Cognitive impairment is present in approximately 30% of patients with amyotrophic lateral sclerosis (ALS) and, especially when severe, has a negative impact on survival and caregiver burden. Our 2010 meta-analysis of the cognitive profile of ALS showed impairment of fluency, executive function, language and memory. However, the limited number of studies resulted in large confidence intervals. To obtain a more valid assessment, we updated the meta-analysis and included methodological improvements (controlled data extraction, risk of bias analysis and effect size calculation of individual neuropsychological tests). Embase, Medline and PsycInfo were searched for neuropsychological studies of non-demented patients with ALS and age-matched and education-matched healthy controls. Neuropsychological tests were categorised in 13 cognitive domains and effect sizes (Hedges' g) were calculated for each domain and for individual tests administered in ≥5 studies. Subgroup analyses were performed to assess the influence of clinical and demographic variables. Forty-four studies were included comprising 1287 patients and 1130 healthy controls. All cognitive domains, except visuoperceptive functions, showed significant effect sizes compared to controls. Cognitive domains without bias due to motor impairment showed medium effect sizes (95% CI): fluency (0.56 (0.43 to 0.70)), language (0.56 (0.40 to 0.72)), social cognition (0.55 (0.34 to 0.76)), or small effect sizes: delayed verbal memory 0.47 (0.27 to 0.68)) and executive functions (0.41 (0.27 to 0.55)). Individual neuropsychological tests showed diverging effect sizes, which could be explained by bias due to motor impairment. Subgroup analyses showed no influence of bulbar disease onset and depression and anxiety on the cognitive outcomes. The cognitive profile of ALS consists of deficits in fluency, language, social cognition, executive functions and verbal memory. Social cognition is a new cognitive domain with a relatively large effect size, highlighting the overlap between ALS and frontotemporal dementia. The diverging effect sizes for individual neuropsychological tests show the importance of correction for motor impairment in patients with ALS. These findings have implications for bedside testing, the design of cognitive screening measures and full neuropsychological examinations.
Key findings
- All cognitive domains, except visuoperceptive functions, showed significant effect sizes compared to controls.
- These findings have implications for bedside testing, the design of cognitive screening measures and full neuropsychological examinations.
Why it matters
This meta-analysis of cognitive studies in amyotrophic lateral sclerosis found significant effect sizes in all cognitive domains except visuoperceptive functions compared with controls. It concerns cognitive impairment in ALS, not the treatment, cure, or prevention of Alzheimer's disease.
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Study details
- Study designmeta-analysis
- Participants1,287
- Evidence strengthHigh
How this study scored 69/100
Published in a peer-reviewed journal, and it is a meta-analysis pooling many studies. Scored zero for effect reported because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Journal of neurology, neurosurgery, and psychiatry) |
| Journal's citation record | 3 / 4 | Journal of Neurology Neurosurgery & Psychiatry: journal h-index 336 (OpenAlex, 2026-10-29) |
| Study design | 12 / 12 | A meta-analysis pooling many studies |
| Strength of evidence | 5 / 5 | Rated “high” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 2016 — within 20 year(s) of the newest study here |
| Sample size | 3 / 4 | 1,287 participants |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 3 / 3 | Studied in people (participant count stated) |
| Randomised / blinded | 0 / 2 | A review of other studies — randomisation is not credited here |
| Times cited | 6 / 7 | Cited 312 times (OpenAlex, 2026-10-10) |
| Citations per year | 4 / 5 | 26.0 citations a year over 12 year(s) since 2016 |
| Impact for its field and year | 3 / 6 | Cited 5.4× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 1 / 6 | Cited by 2 other studies in this library |
| Still being cited | 3 / 4 | Cited 64 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 3 / 4 | Last-listed author Rob J. de Haan: h-index 82 (OpenAlex, whole career) |
| Institution's track record | 1 / 3 | University of Amsterdam: institution h-index 1,209 (OpenAlex) |
| Multi-centre collaboration | 2 / 3 | OpenAlex lists 4 institution(s) in 1 country |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 26283685) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 69 / 100 | — |
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Read the original
- DOI: 10.1136/jnnp-2015-310734
- PubMed: 26283685
- Original source →
- Free full text: Pure Amsterdam UMC →
Full text: free to read — OpenAlex lists this copy as free to read: the link above (checked 6 Feb 1020).
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
Built from the record alzhub holds for this paper, not looked up. Volume, issue and page numbers are not held in the record. Check the citation against the original before you use it.
APA 7th
Beeldman, E., Raaphorst, J., Klein Twennaar, M., de Visser, M., Schmand, B. A., & de Haan, R. J. (2016). The cognitive profile of ALS: a systematic review and meta-analysis update. Journal of neurology, neurosurgery, and psychiatry. https://doi.org/10.1136/jnnp-2015-310734
Vancouver
Beeldman E, Raaphorst J, Klein Twennaar M, de Visser M, Schmand BA, de Haan RJ. The cognitive profile of ALS: a systematic review and meta-analysis update. Journal of neurology, neurosurgery, and psychiatry. 2016. doi:10.1136/jnnp-2015-310734. PMID: 26283685.
BibTeX
@article{beeldman2016-26283685,
author = {Beeldman, E. and Raaphorst, J. and Klein Twennaar, M. and de Visser, M. and Schmand, B. A. and de Haan, R. J.},
title = {{The cognitive profile of ALS: a systematic review and meta-analysis update}},
journal = {Journal of neurology, neurosurgery, and psychiatry},
year = {2016},
doi = {10.1136/jnnp-2015-310734},
pmid = {26283685},
url = {https://doi.org/10.1136/jnnp-2015-310734}
}
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