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Inflammation in ALS/FTD pathogenesis
McCauley ME, Baloh RH.. Acta neuropathologica, 2018-11-21
Topic: Brain proteins, microglia and lab models
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are neurodegenerative diseases that overlap in their clinical presentation, pathology and genetics, and likely represent a spectrum of one underlying disease. In ALS/FTD patients, neuroinflammation characterized by innate immune responses of tissue-resident glial cells is uniformly present on end-stage pathology, and human imaging studies and rodent models support that neuroinflammation begins early in disease pathogenesis. Additionally, changes in circulating immune cell populations and cytokines are found in ALS/FTD patients, and there is evidence for an autoinflammatory state.
Key findings
- In ALS/FTD patients, neuroinflammation characterized by innate immune responses of tissue-resident glial cells is uniformly present on end-stage pathology, and human imaging studies and rodent models support that neuroinflammation begins early in disease pathogenesis.
- Additionally, changes in circulating immune cell populations and cytokines are found in ALS/FTD patients, and there is evidence for an autoinflammatory state.
- However, despite the prominent role of neuro- and systemic inflammation in ALS/FTD, and experimental evidence in rodents that altering microglial function can mitigate pathology, therapeutic approaches to decrease inflammation have thus far failed to alter disease course in humans.
- Here, we review the characteristics of inflammation in ALS/FTD in both the nervous and peripheral immune systems.
Why it matters
This review describes neuroinflammation in ALS and FTD, which overlap as a disease spectrum, and notes that inflammation appears early. It reports no treatment that cures or prevents these diseases.
Study details
- Evidence strengthLow
How this study scored 52/100
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 (Acta neuropathologica) |
| Journal's citation record | 3 / 4 | Acta Neuropathologica: journal h-index 287 (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 | 2 / 5 | 2019 — within 10 year(s) of the newest study here |
| 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 322 times (OpenAlex, 2026-10-10) |
| Citations per year | 4 / 5 | 35.8 citations a year over 9 year(s) since 2019 |
| Impact for its field and year | 4 / 6 | Cited 14.9× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 2 / 6 | Cited by 3 other studies in this library |
| Still being cited | 3 / 4 | Cited 123 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 3 / 4 | Last-listed author Robert H. Baloh: h-index 63 (OpenAlex, whole career) |
| Institution's track record | 1 / 3 | Cedars-Sinai Medical Center: institution h-index 866 (OpenAlex) |
| Multi-centre collaboration | 0 / 3 | OpenAlex lists 1 institution(s) in 1 country — scores zero (affiliation lists can be incomplete) |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 30465257) |
| Free to read | 2 / 2 | Free to read |
| Total | 52 / 100 | — |
What each of these 20 components measures, and what this score is not →
Read the original
- DOI: 10.1007/s00401-018-1933-9
- PubMed: 30465257
- Original source →
- Free full text: Acta Neuropathologica →
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
McCauley, M. E., & Baloh, R. H. (2019). Inflammation in ALS/FTD pathogenesis. Acta neuropathologica. https://doi.org/10.1007/s00401-018-1933-9
Vancouver
McCauley ME, Baloh RH. Inflammation in ALS/FTD pathogenesis. Acta neuropathologica. 2019. doi:10.1007/s00401-018-1933-9. PMID: 30465257.
BibTeX
@article{mccauley2019-30465257,
author = {McCauley, M. E. and Baloh, R. H.},
title = {{Inflammation in ALS/FTD pathogenesis}},
journal = {Acta neuropathologica},
year = {2019},
doi = {10.1007/s00401-018-1933-9},
pmid = {30465257},
url = {https://doi.org/10.1007/s00401-018-1933-9}
}
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