Prevalence and outcomes of rapidly progressive dementia: a retrospective cohort study in a neurologic unit in China

Abstract

Background Rapidly progressive dementia (RPD) is a syndrome originating from various diseases. Recent advances have allowed a better understanding of its categories and spectrum; however, it remains challenging to make an accurate differential diagnosis and prognosis prediction. Methods This study was a retrospective evaluation of all participants admitted to the neurology department of a single center in China from January 2015 to December 2019. The screened patients met the RPD criteria and their characteristics were collected to explore a diagnostic pattern of RPD. In addition, outcomes of RPD were evaluated with the Glasgow Outcome Scale (GOS), activities of daily living scale (ADL), and simplified Mini-Mental State Examination (MMSE), and different prognostic analysis methods were performed to determine the prognostic factors of RPD. Results A total of 149 RPD patients among 15,731 inpatients were identified with an average MMSE value of 13.0 ± 4.6 at baseline. Etiological epidemiology revealed infectious, neurodegenerative and toxic/metabolic diseases as the three largest groups, accounting for 26.2%, 20.8% and 16.8% of all cases, respectively. In particular, prevalence rates of Creutzfeldt–Jakob disease (13.4%), Alzheimer’s disease (11.4%), carbon monoxide poisoning (8.1%), neurosyphilis (5.4%) and dementia with Lewy bodies (5.4%) were highest in this series. A recommended diagnostic framework for RPD etiology was thus established. Follow-up evaluations showed a negative correlation between age and GOS scores (r=-0.421, P < 0.001), as well as age and simplified MMSE scores (rs =- 0.393, P < 0.001), and a positive correlation between age and ADL scores (rs =0.503, P < 0.001), and significantly different GOS, ADL and simplified MMSE scores across various etiologies (P = 0.003; F = 9.463, P < 0.001; F = 6.117, P < 0.001). Conclusion Infectious, neurodegenerative and toxic-metabolic entities were the most common RPD categories, and establishing a practical approach to RPD etiology would allow better disease management.

Improving Early Recognition of Treatment‐Responsive Causes of Rapidly Progressive Dementia: The STAM3P Score

“…This cohort included a high proportion of non‐Hispanic White participants, and all patients were enrolled within North America. Common causes of RPD change with population characteristics and practice environment, with cohorts outside of North America and Europe reporting a greater contribution of infectious disease and post‐infectious sequelae to RPD than reported here 6,12,48 …”

“…Common causes of RPD change with population characteristics and practice environment, with cohorts outside of North America and Europe reporting a greater contribution of infectious disease and post-infectious sequelae to RPD than reported here. 6,12,48 The lack of neuropathological confirmation in many patients in this series also leaves open the possibility of misdiagnoses in subsets of cases. Although the high concordance (100%) between clinical and neuropathological diagnoses (n = 24 autopsies) in the subset of patients who died and came to autopsy is reassuring, 49 validation of study findings and methods in comparable cohorts followed until death is needed.…”

“…The application of uniform measures of cognitive function in this prospective observational study enrolling patients with RPD represents a substantial advance over prior retrospective series leveraging comprehensive chart reviews. [1][2][3]6,12,17,18,48 Yet, study follow-up and treatment decisions were dictated by clinical indications, contributing to variations in management and outcomes, and necessitating empiric assignment of treatment responsiveness. Although empiric assignment was reasonably aligned with observed outcomes (Fig.…”

Levels of plasma brain‐derived tau and p‐tau181 in Alzheimer's disease and rapidly progressive dementias

“…The distinct underlying etiologies of RPDs underscore the importance of accurate diagnosis for both patient care and research 3 . An accurate diagnosis of RPD and the initial categorization between neurodegenerative or other etiologies aid in providing appropriate and timely medical interventions, as treatment strategies may differ substantially, and misdiagnosis can lead to ineffective treatments and disease progression 2,3,6,7 …”

“…5 Accurate epidemiological data on RPD can be challenging to obtain due to underdiagnosis, misdiagnosis, and limited surveillance systems. 6,7 Creutzfeldt-Jakob disease (CJD), the prototype of human prion disease, is the most common cause of neurodegenerative RPD with an estimated annual incidence of approximately 1 to 2 cases per million population. 8,9 It encompasses several subtypes, including sporadic, familial, iatrogenic, and variant CJD.…”