Etiologies of Rapidly Progressive Dementias: A Systematic Review and Meta-analysis of Causes in Worldwide and Latin America

Overview

Journal J Alzheimers Dis Rep

Publisher IOS Press

Date 2025 Mar 4

PMID 40034520

Authors

Jonathan Fernando Cubas Guillen

Carolina Agata Ardohain Cristalli

Waleska Berrios

Florencia Deschle

Guido Dorman

Julian Fernandez Boccazzi

Ignacio Flores

Hector Gaston Graviotto

Cristian Flavio Isaac

Galeno Rojas

Karen Daniela Roman

Maria de la Paz Scribano Parada

Marcos Sorbara

Ricardo F Allegri

Ismael Luis Calandri

Affiliations

Soon will be listed here.

Abstract

Background: Rapidly progressive dementia (RPD) is a group of neurological diseases, where three etiologies are particularly relevant: neurodegenerative, prion and autoimmune encephalitis (AIE) diseases.

Objective: The aim of this study is to conduct a systematic review and meta-analysis of the frequency of these etiologies causing RPD in worldwide and Latin America (LatAm).

Methods: A systematic review and meta-analysis were conducted. A bibliographic search of publications related to the etiologies of RPD was done. The etiologies, the timeframe definition (<1 year versus <2 years) and the study's place of origin were analyzed.

Results: A total of 10 articles were selected for the analysis in this study (n = 1006 patients). Three studies were originated in LatAm cohorts (two from Argentina and one from Brazil). The global prevalence of RPD due to neurodegenerative disease was 23% CI95% [11%; 42%]; prion diseases, 16% CI95% [9%; 28%]; and AIE, 12% CI95% [6%; 22%]. Comparing each overall proportion of etiologies of LatAm versus non-LatAm there were statistically significant differences for AIE (25% versus 8%, respectively, < 0.01). In the case of timeframe definitions, the comparison of the etiological percentage did not show statistically significant differences.

Conclusions: From our results, approximately a half of the causes of RPD were due to neurodegenerative, prion, and AIE diseases. Future studies will be needed to analyze this issue both globally and regionally.

References

Jack Jr C, Bennett D, Blennow K, Carrillo M, Dunn B, Budd Haeberlein S . NIA-AA Research Framework: Toward a biological definition of Alzheimer's disease. Alzheimers Dement. 2018; 14(4):535-562. PMC: 5958625. DOI: 10.1016/j.jalz.2018.02.018. View

Stamatelos P, Kontokostas K, Liantinioti C, Giavasi C, Ioakeimidis M, Antonelou R . Evolving Causes of Rapidly Progressive Dementia: A 5-Year Comparative Study. Alzheimer Dis Assoc Disord. 2021; 35(4):315-320. DOI: 10.1097/WAD.0000000000000472. View

Hermann P, Zerr I . Rapidly progressive dementias - aetiologies, diagnosis and management. Nat Rev Neurol. 2022; 18(6):363-376. PMC: 9067549. DOI: 10.1038/s41582-022-00659-0. View

Flanagan E, Geschwind M, Lopez-Chiriboga A, Blackburn K, Turaga S, Binks S . Autoimmune Encephalitis Misdiagnosis in Adults. JAMA Neurol. 2022; 80(1):30-39. PMC: 9706400. DOI: 10.1001/jamaneurol.2022.4251. View

Studart Neto A, Soares Neto H, Simabukuro M, Solla D, Goncalves M, Fortini I . Rapidly Progressive Dementia: Prevalence and Causes in a Neurologic Unit of a Tertiary Hospital in Brazil. Alzheimer Dis Assoc Disord. 2016; 31(3):239-243. DOI: 10.1097/WAD.0000000000000170. View

Abboud H, Probasco J, Irani S, Ances B, Benavides D, Bradshaw M . Autoimmune encephalitis: proposed recommendations for symptomatic and long-term management. J Neurol Neurosurg Psychiatry. 2021; . PMC: 8292591. DOI: 10.1136/jnnp-2020-325302. View

Acosta J, Ricciardi M, Alessandro L, Carnevale M, Farez M, Nagel V . Diagnosis of Rapidly Progressive Dementia in a Referral Center in Argentina. Alzheimer Dis Assoc Disord. 2019; 34(1):54-58. DOI: 10.1097/WAD.0000000000000337. View

Geschwind M . Rapidly Progressive Dementia. Continuum (Minneap Minn). 2016; 22(2 Dementia):510-37. PMC: 4879977. DOI: 10.1212/CON.0000000000000319. View

Grau-Rivera O, Gelpi E, Nos C, Gaig C, Ferrer I, Saiz A . Clinicopathological Correlations and Concomitant Pathologies in Rapidly Progressive Dementia: A Brain Bank Series. Neurodegener Dis. 2015; 15(6):350-60. DOI: 10.1159/000439251. View

10.

Ribeiro F, Teixeira-Santos A, Caramelli P, Leist A . Prevalence of dementia in Latin America and Caribbean countries: Systematic review and meta-analyses exploring age, sex, rurality, and education as possible determinants. Ageing Res Rev. 2022; 81:101703. PMC: 9582196. DOI: 10.1016/j.arr.2022.101703. View

11.

Lang K, Pruss H . Frequencies of neuronal autoantibodies in healthy controls: Estimation of disease specificity. Neurol Neuroimmunol Neuroinflamm. 2017; 4(5):e386. PMC: 5515597. DOI: 10.1212/NXI.0000000000000386. View

12.

Day G, Musiek E, Morris J . Rapidly Progressive Dementia in the Outpatient Clinic: More Than Prions. Alzheimer Dis Assoc Disord. 2018; 32(4):291-297. PMC: 6249048. DOI: 10.1097/WAD.0000000000000276. View

13.

Anuja P, Venugopalan V, Darakhshan N, Awadh P, Wilson V, Manoj G . Rapidly progressive dementia: An eight year (2008-2016) retrospective study. PLoS One. 2018; 13(1):e0189832. PMC: 5773088. DOI: 10.1371/journal.pone.0189832. View

14.

Liu X, Sun Y, Zhang X, Liu P, Zhang K, Yu L . Prevalence and outcomes of rapidly progressive dementia: a retrospective cohort study in a neurologic unit in China. BMC Geriatr. 2023; 23(1):142. PMC: 10012734. DOI: 10.1186/s12877-023-03841-1. View

15.

Valencia-Sanchez C, Pittock S, Mead-Harvey C, Dubey D, Flanagan E, Lopez-Chiriboga S . Brain dysfunction and thyroid antibodies: autoimmune diagnosis and misdiagnosis. Brain Commun. 2021; 3(2):fcaa233. PMC: 8152924. DOI: 10.1093/braincomms/fcaa233. View

16.

van Dyck C, Swanson C, Aisen P, Bateman R, Chen C, Gee M . Lecanemab in Early Alzheimer's Disease. N Engl J Med. 2022; 388(1):9-21. DOI: 10.1056/NEJMoa2212948. View

17.

Abboud H, Probasco J, Irani S, Ances B, Benavides D, Bradshaw M . Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management. J Neurol Neurosurg Psychiatry. 2021; 92(7):757-768. PMC: 8223680. DOI: 10.1136/jnnp-2020-325300. View

18.

Mead S, Rudge P . CJD mimics and chameleons. Pract Neurol. 2017; 17(2):113-121. PMC: 5520355. DOI: 10.1136/practneurol-2016-001571. View

19.

Tagliapietra M, Zanusso G, Fiorini M, Bonetto N, Zarantonello G, Zambon A . Accuracy of diagnostic criteria for sporadic creutzfeldt-jakob disease among rapidly progressive dementia. J Alzheimers Dis. 2012; 34(1):231-8. DOI: 10.3233/JAD-121873. View

20.

Graus F, Titulaer M, Balu R, Benseler S, Bien C, Cellucci T . A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 2016; 15(4):391-404. PMC: 5066574. DOI: 10.1016/S1474-4422(15)00401-9. View