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Prevalence and patterns of cognitive impairment in sporadic ALS
Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE.. Neurology, 2005-08-01
Topic: Risk, prevention and care in population studies
Objective To investigate the prevalence and nature of cognitive changes associated with sporadic amyotrophic lateral sclerosis (ALS) using a large scale study. Methods Consecutive patients with sporadic ALS (n = 279) underwent comprehensive neurologic evaluation and neuropsychological testing. Testing data from normal controls (n = 129) were used for classification and comparison purposes. Results On non-motor, non-speed-dependent tasks, 51% of patients with ALS had evidence of cognitive impairment compared to 5% of controls. Cluster analysis suggested four patient subgroups: 49% intact, 32% with mild impairment, 13% with moderate impairment, and 6% with severe impairment. Forty-one patients (15%) met criteria for frontotemporal dementia (FTD). ALS patient subgroups, excluding the intact group, performed significantly lower on tests of executive function and memory than normal controls. Patients with more severe disease also had deficits in confrontation naming. Although memory function declined with increasing severity of overall cognitive impairment, only two patients had the severe memory loss typical of Alzheimer disease. Cognitive impairment was correlated with clinical measures of word-finding, phrase length, and motor programming. Cognitive impairment was not correlated with depression scores or severity or duration of motor or bulbar symptoms. Patients with bulbar vs limb-onset ALS were not different in either level of impairment or pattern of performance. Conclusions These data confirm the presence of cognitive impairment in 50% of patients with ALS and particularly implicate executive dysfunction and mild memory decline in the disease process. More severe impairment occurs in a subset of patients with ALS and has features consistent with FTD.
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
APA 7th
Ringholz, G. M., Appel, S. H., Bradshaw, M., Cooke, N. A., Mosnik, D. M., & Schulz, P. E. (2005). Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology. https://doi.org/10.1212/01.wnl.0000172911.39167.b6
Vancouver
Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE. Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology. 2005. doi:10.1212/01.wnl.0000172911.39167.b6. PMID: 16116120.
BibTeX
@article{ringholz2005-16116120,
author = {Ringholz, G. M. and Appel, S. H. and Bradshaw, M. and Cooke, N. A. and Mosnik, D. M. and Schulz, P. E.},
title = {{Prevalence and patterns of cognitive impairment in sporadic ALS}},
journal = {Neurology},
year = {2005},
doi = {10.1212/01.wnl.0000172911.39167.b6},
pmid = {16116120},
url = {https://doi.org/10.1212/01.wnl.0000172911.39167.b6}
}
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Key findings
- Objective To investigate the prevalence and nature of cognitive changes associated with sporadic amyotrophic lateral sclerosis (ALS) using a large scale study.
- Results On non-motor, non-speed-dependent tasks, 51% of patients with ALS had evidence of cognitive impairment compared to 5% of controls.
- ALS patient subgroups, excluding the intact group, performed significantly lower on tests of executive function and memory than normal controls.
- Although memory function declined with increasing severity of overall cognitive impairment, only two patients had the severe memory loss typical of Alzheimer disease.
Why it matters
This study examined cognitive impairment in people with sporadic ALS and found that many had cognitive changes, with some meeting criteria for frontotemporal dementia. It is about cognitive changes in ALS, not about treating, curing, or preventing Alzheimer's disease.
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Study details
- Participants279
- Evidence strengthLow
How this study scored 58/100
Published in a leading specialty journal. Scored zero for study design, effect reported and randomized / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 9 / 10 | Tier 2 — leading specialty journal (Neurology) |
| Journal's citation record | 4 / 4 | Neurology: journal h-index 525 (OpenAlex, 2026-10-29) |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 1 / 5 | Rated “low” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 2005 — older than 20 years, still counted |
| Sample size | 2 / 4 | 279 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) |
| Randomized / blinded | 0 / 2 | No randomization or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 6 / 7 | Cited 848 times (OpenAlex, 2026-10-10) |
| Citations per year | 4 / 5 | 36.9 citations a year over 23 year(s) since 2005 |
| Impact for its field and year | 4 / 6 | Cited 13.5× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 3 / 6 | Cited by 19 other studies in this library |
| Still being cited | 3 / 4 | Cited 78 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 2 / 4 | Last-listed author Paul Ernest Schulz: h-index 45 (OpenAlex, whole career) |
| Institution's track record | 2 / 3 | Baylor College of Medicine: institution h-index 1,607 (OpenAlex) |
| Multicenter collaboration | 2 / 3 | OpenAlex lists 4 institution(s) in 1 country |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 16116120) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 58 / 100 | — |
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