Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Observational Study
. 2024 Feb;95(2):237-248.
doi: 10.1002/ana.26812. Epub 2023 Oct 19.

Improving Early Recognition of Treatment-Responsive Causes of Rapidly Progressive Dementia: The STAM3 P Score

Nihal Satyadev  1   2 , Philip W Tipton  1 , Yuka Martens  3 , S Richard Dunham  4 , Michael D Geschwind  5 , John C Morris  4 , Matthew R Brier  4 , Neill R Graff-Radford  1 , Gregory S Day  1
Affiliations
Observational Study

Improving Early Recognition of Treatment-Responsive Causes of Rapidly Progressive Dementia: The STAM3 P Score

Nihal Satyadev et al. Ann Neurol. 2024 Feb.

Abstract

Objective: To improve the timely recognition of patients with treatment-responsive causes of rapidly progressive dementia (RPD).

Methods: A total of 226 adult patients with suspected RPD were enrolled in a prospective observational study and followed for up to 2 years. Diseases associated with RPD were characterized as potentially treatment-responsive or non-responsive, referencing clinical literature. Disease progression was measured using Clinical Dementia Rating® Sum-of-Box scores. Clinical and paraclinical features associated with treatment responsiveness were assessed using multivariable logistic regression. Findings informed the development of a clinical criterion optimized to recognize patients with potentially treatment-responsive causes of RPD early in the diagnostic evaluation.

Results: A total of 155 patients met defined RPD criteria, of whom 86 patients (55.5%) had potentially treatment-responsive causes. The median (range) age-at-symptom onset in patients with RPD was 68.9 years (range 22.0-90.7 years), with a similar number of men and women. Seizures, tumor (disease-associated), magnetic resonance imaging suggestive of autoimmune encephalitis, mania, movement abnormalities, and pleocytosis (≥10 cells/mm3 ) in cerebrospinal fluid at presentation were independently associated with treatment-responsive causes of RPD after controlling for age and sex. Those features at presentation, as well as age-at-symptom onset <50 years (ie, STAM3 P), captured 82 of 86 (95.3%) cases of treatment-responsive RPD. The presence of ≥3 STAM3 P features had a positive predictive value of 100%.

Interpretation: Selected features at presentation reliably identified patients with potentially treatment-responsive causes of RPD. Adaptation of the STAM3 P screening score in clinical practice may minimize diagnostic delays and missed opportunities for treatment in patients with suspected RPD. ANN NEUROL 2024;95:237-248.

PubMed Disclaimer

Conflict of interest statement

Potential conflicts of interest

Nothing to report.

Similar articles

See all similar articles

Cited by

References

    1. Papageorgiou SG, Kontaxis T, Bonakis A, Karahalios G, Kalfakis N, Vassilopoulos D. Rapidly progressive dementia: causes found in a Greek tertiary referral center in Athens. Alzheimer Dis Assoc Disord. 2009/October 2009;23(4):337–346. doi:10.1097/WAD.0b013e31819e099b - DOI - PubMed
    1. Stamatelos P, Kontokostas K, Liantinioti C, et al. Evolving causes of Rapidly Progressive Dementia: a 5-Year comparative study. Alzheimer Disease & Associated Disorders. 2021;35(4):315–320. - PubMed
    1. Day GS, Musiek ES, Morris JC. Rapidly Progressive Dementia in the Outpatient Clinic: More Than Prions. Alzheimer Dis Assoc Disord. 2018/October 2018;32(4):291–297. doi:10.1097/WAD.0000000000000276 - DOI - PMC - PubMed
    1. Josephs KA, Ahlskog JE, Parisi JE, et al. Rapidly progressive neurodegenerative dementias. Arch Neurol. 2009/February 2009;66(2):201–207. doi:10.1001/archneurol.2008.534 - DOI - PMC - PubMed
    1. Hermann P, Zerr I. Rapidly progressive dementias - aetiologies, diagnosis and management. Nat Rev Neurol. 2022/June 2022;18(6):363–376. doi:10.1038/s41582-022-00659-0 - DOI - PMC - PubMed

Publication types

LinkOut - more resources