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Dementia as the most common presentation of cortical-basal ganglionic degeneration
Grimes DA, Lang AE, Bergeron CB.. Neurology, 1999-12-01
Topic: Risk, prevention and care in population studies
Objective To evaluate the clinical presentations and dominant symptoms of patients with postmortem proven cortical-basal ganglionic degeneration (CBGD) from one neuropathology center. Background CBGD is a rare but increasingly recognized condition with clinical and pathologic features that continue to evolve. Attempts have been made to develop clinical criteria to enhance the specificity of diagnosis, but it is not clear what proportion of patients harboring CBGD disease present in the "classical" fashion versus other presentations. Previous large-case series that emphasize a parietal/perceptual-motor presentation may be biased because the cases mainly originate from movement disorder centers. Methods Thirteen cases of pathologically confirmed CBGD with sufficient clinical data were identified from a single neuropathology center between 1981 and 1996. Results Before death, only 4 of the 13 patients had a clinical diagnosis of CBGD, 6 had a clinical diagnosis of Alzheimer's disease (AD), 1 had AD and parkinsonism, and 2 had an atypical dementia of the frontotemporal type. Nine of 13 cases had early dementia. Conclusions Dementia was the most common presentation of CBGD in this study. Despite the best efforts to define criteria to enhance the specificity of a diagnosis of CBGD, it is becoming clear that the clinical syndrome that accompanies this disease is quite varied. Unfortunately, patients fulfilling classical diagnostic criteria may represent a minority of those with this pathologic diagnosis.
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
APA 7th
Grimes, D. A., Lang, A. E., & Bergeron, C. B. (1999). Dementia as the most common presentation of cortical-basal ganglionic degeneration. Neurology. https://doi.org/10.1212/wnl.53.9.1969
Vancouver
Grimes DA, Lang AE, Bergeron CB. Dementia as the most common presentation of cortical-basal ganglionic degeneration. Neurology. 1999. doi:10.1212/wnl.53.9.1969. PMID: 10599767.
BibTeX
@article{grimes1999-10599767,
author = {Grimes, D. A. and Lang, A. E. and Bergeron, C. B.},
title = {{Dementia as the most common presentation of cortical-basal ganglionic degeneration}},
journal = {Neurology},
year = {1999},
doi = {10.1212/wnl.53.9.1969},
pmid = {10599767},
url = {https://doi.org/10.1212/wnl.53.9.1969}
}
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Key findings
- Attempts have been made to develop clinical criteria to enhance the specificity of diagnosis, but it is not clear what proportion of patients harboring CBGD disease present in the "classical" fashion versus other presentations.
- Results Before death, only 4 of the 13 patients had a clinical diagnosis of CBGD, 6 had a clinical diagnosis of Alzheimer's disease (AD), 1 had AD and parkinsonism, and 2 had an atypical dementia of the frontotemporal type.
Why it matters
This study of 13 patients with pathologically confirmed cortical-basal ganglionic degeneration found that dementia was the most common presentation. It is a small case series and does not test any treatment, cure, or prevention for Alzheimer's disease.
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Study details
- Participants13
- Evidence strengthLow
How this study scored 47/100
Published in a leading specialty journal. Scored zero for study design, effect reported and randomised / blinded because the record does not state it. We have no data on institution's track record, so that was left out: it earned 46 of the 97 points we could judge it on, which is 47 out of 100.
| 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 | 1999 — older than 20 years, still counted |
| Sample size | 1 / 4 | 13 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 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 5 / 7 | Cited 218 times (OpenAlex, 2026-10-10) |
| Citations per year | 2 / 5 | 7.5 citations a year over 29 year(s) since 1999 |
| Impact for its field and year | 3 / 6 | Cited 4.7× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 2 / 6 | Cited by 6 other studies in this library |
| Still being cited | 1 / 4 | Cited 7 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 2 / 4 | Last-listed author Catherine Bergeron: h-index 35 (OpenAlex, whole career) |
| Institution's track record | — / 3 | No institution listed in OpenAlex — left out of this score |
| Multi-centre collaboration | 0 / 3 | OpenAlex lists 0 institution(s) in 0 countries — scores zero (affiliation lists can be incomplete) |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 10599767) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Points | 46 / 97 | Scored on 19 of 20 parts. We have no data for the rest, so they are left out: 46 of the 97 points we could judge it on is 47 out of 100. |
| Total | 47 / 100 | — |
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