Dementia and Rapid Mortality: Who is at Risk?

Abstract

Abstract.Background: Dementia is typically known for its insidious onset and slowly progressive course, but a subgroup deteriorates fast and dies within years or even months. Objective: The purpose of this study was to characterize dementia patients with a rapidly progressive course to death and evaluate their cause of death. Methods: We retrospectively included all patients from the Amsterdam Dementia Cohort who died within two years after diagnosis. We evaluated the characteristics of these rapid progressors and compared them to patients known to be alive two years after diagnosis ('non-rapid mortality'). Results: We included 129 dementia patients (13% of our total cohort with known follow-up) with rapid mortality (age 72 ± 10 y [29% <65 y], 70[55%]M, MMSE 20 ± 5). Mean(SD) survival was 12 ± 7 months. Compared to non-rapid mortality patients (n = 892; age 68 ± 9, 503(56%)M, MMSE 22 ± 5), patients with rapid mortality were slightly older at time of diagnosis, had lower MMSE scores, more depressive symptoms and higher prevalence of a cardiovascular history (all p < 0.05). Alzheimer's disease (AD, 43%) was most frequent in patients with rapid mortality, but the occurrence was much lower compared to non-rapid mortality patients (71%), while all other dementia diagnoses, especially Creutzfeldt-Jakob disease (CJD), vascular dementia (VaD), and frontotemporal dementia (FTD), were more frequent (p < 0.001). There were no specific characteristics for AD patients with rapid versus non-rapid mortality, especially APOE genotypes and CSF-profiles were comparable (p > 0.70). Cause of death was highly variable without a clear relation to dementia diagnosis, with exception of dementia itself in CJD, intracerebral hematoma in VaD, and motor neuron disease in FTD. Conclusions: Short survival is relatively common (∼13% in our cohort) and occurs in all different types of dementia, with overrepresentation of non-AD dementias like CJD, VaD, and FTD.

Typical and atypical pathology in primary progressive aphasia variants

“…In our study, conventional neuroimaging was also reviewed at the time of clinical diagnosis, allowing for an “imaging-supported” diagnosis, which is likely to be more consistent with pathology. The fact that our cohort of pathologically-proven lvPPA is smaller than the other two main groups may be partially explained by the longer survival we found in lvPPA patients, in keeping with studies showing a more aggressive clinical course of FTLD-related disorders compared with AD 50 . Although we do not expect that larger cohorts will show a 100% association between lvPPA and AD pathology, large biomarker studies do indicate that AD is the most typical pathology in lvPPA 18–20 .…”

“…That our cohort of pathologically proven lvPPA is smaller than the other 2 main groups may be partially explained by the longer survival we found in lvPPA patients, in keeping with studies showing a more aggressive clinical course of FTLD-related disorders compared with AD. 50 Although we do not expect that larger cohorts will show a 100% association between lvPPA and AD pathology, large biomarker studies do indicate that AD is the most typical pathology in lvPPA. [18][19][20] In summary, our findings, together with previous evidence, suggest that the clinical and neuroimaging diagnostic features of lvPPA are highly predictive of underlying AD.…”

Amsterdam Dementia Cohort: Performing Research to Optimize Care

“…Looking at MRI, both WMH and microbleeds were associated with increased risk of mortality, while after age adjustment, the effect of atrophy lost significance [ 131 ]. When we took an opposite approach, taking short survival (<2 years after diagnosis) as a starting point, we found that short survival is relatively common (approximately 13% of our cohort) and occurs in all different types of dementia, with overrepresentation of non-AD dementia’s like Creutzfeldt-Jakob disease, VaD, and FTD [ 132 ].…”

Strategy of Cardiovascular Surgery for Patients With Dementia as Evaluated by Mini-Mental State Examination

“…Staekenborg et al have reported that a lower MMSE score (20±5) and the complication of cardiovascular disease were risk factors for death within 2 years after a diagnosis of dementia. 24 Thus, the surgical indications for patients with moderate dementia (MMSE score <20) might still be controversial with high surgical risk and poor prognosis. And if a surgical strategy is selected, minimally invasive strategy represented by off-pump CABG, TAVR, and endovascular aneurysm repair, should be considered.…”