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Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP
Josephs KA, Petersen RC, Knopman DS, Boeve BF, Whitwell JL, Duffy JR, Parisi JE, Dickson DW.. Neurology, 2006-01-01
Topic: Brain proteins, microglia and lab models
Objective To examine the relationship between early clinical features, pathologies, and biochemistry of the frontotemporal lobar degenerations (FTLDs), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). Methods The authors conducted pathologic reexamination with the most recent immunohistochemistry of all cases diagnosed with FTLD, PSP, and CBD between 1970 and 2004. The authors also reviewed the early clinical features for clinical diagnosis and application of published research criteria. Results Of 127 cases analyzed, 57 had a pathologic diagnosis of FTLD, 49 PSP, and 21 CBD. Of these, 38 were clinically reclassified as frontal variant frontotemporal dementia (FTD), 13 as progressive non-fluent aphasia (PNFA), 21 as CBD-like, 33 as PSP-like, and 13 with frontotemporal dementia with coexisting motor neuron disease (FTD-MND). The authors were unable to classify nine cases. All cases of FTD-MND were tau-negative and had pathologic evidence of motor neuron degeneration. All cases classified as PSP-like or CBD-like had tau-positive pathology. Of the 13 cases with PNFA, PSP and CBD accounted for almost 70% of the cases, while FTD was almost equally divided between tau-positive and tau-negative diseases. Conclusion Frontotemporal lobar degeneration, corticobasal degeneration (CBD), and progressive supranuclear palsy (PSP) have overlapping clinical features. The prediction of tau-positive pathology from a CBD or PSP-like presentation is good, while the frontotemporal dementia (FTD)-motor neuron disease syndrome almost certainly predicts motor neuron degeneration. Surprisingly, PSP and CBD accounted for most cases classified as progressive non-fluent aphasia. Frontal variant FTD is an unpredictable disease in terms of its biochemistry.
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APA 7th
Josephs, K. A., Petersen, R. C., Knopman, D. S., Boeve, B. F., Whitwell, J. L., Duffy, JR., et al. (2006). Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology. https://doi.org/10.1212/01.wnl.0000191307.69661.c3
Vancouver
Josephs KA, Petersen RC, Knopman DS, Boeve BF, Whitwell JL, Duffy JR, et al. Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. Neurology. 2006. doi:10.1212/01.wnl.0000191307.69661.c3. PMID: 16401843.
BibTeX
@article{josephs2006-16401843,
author = {Josephs, K. A. and Petersen, R. C. and Knopman, D. S. and Boeve, B. F. and Whitwell, J. L. and Duffy, JR. and Parisi, J. E. and Dickson, D. W.},
title = {{Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP}},
journal = {Neurology},
year = {2006},
doi = {10.1212/01.wnl.0000191307.69661.c3},
pmid = {16401843},
url = {https://doi.org/10.1212/01.wnl.0000191307.69661.c3}
}
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Key findings
- Results Of 127 cases analyzed, 57 had a pathologic diagnosis of FTLD, 49 PSP, and 21 CBD.
Why it matters
In 127 autopsy-confirmed cases of frontotemporal lobar degeneration, progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD), the clinical pictures overlapped. Some presentations still pointed to the underlying disease: every PSP-like or CBD-like case had tau-positive pathology, and every case of frontotemporal dementia with motor neuron disease was tau-negative with motor neuron degeneration. Frontal-variant FTD was split almost evenly between tau-positive and tau-negative disease, and PSP or CBD accounted for almost 70% of the 13 progressive non-fluent aphasia cases. This is a retrospective autopsy series about diagnosis, not treatment.
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Study details
- Evidence strengthLow
How this study scored 54/100
Published in a leading specialty journal. Scored zero for study design, sample size, effect reported, studied in people and randomised / 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 | 2006 — older than 20 years, still counted |
| Sample size | 0 / 4 | Participant count not stated — scores zero (not stated in the record — scored 0) |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 0 / 3 | Not stated whether this was studied in people — scores zero (not stated in the record — scored 0) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 6 / 7 | Cited 458 times (OpenAlex, 2026-10-10) |
| Citations per year | 4 / 5 | 20.8 citations a year over 22 year(s) since 2006 |
| Impact for its field and year | 5 / 6 | Cited 24.5× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 3 / 6 | Cited by 29 other studies in this library |
| Still being cited | 2 / 4 | Cited 22 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 4 / 4 | Last-listed author Dennis W. Dickson: h-index 218 (OpenAlex, whole career) |
| Institution's track record | 2 / 3 | Mayo Clinic: institution h-index 1,606 (OpenAlex) |
| Multi-centre collaboration | 1 / 3 | OpenAlex lists 2 institution(s) in 1 country |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 16401843) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 54 / 100 | — |
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