Objective:

To determine clinical predictors of survival from cognitive symptom onset in patients with clinically probable DLB.

Background:

Previous studies have shown a shorter survival in dementia with Lewy bodies (DLB) compared with other dementias. However, clinical predictors of survival in DLB are not well-understood.

Design/Methods:

We studied 488 patients who met criteria for probable DLB during their disease course and separately analyzed 175 of these individuals who had pathologically proven DLB at autopsy. Survival was estimated from time of cognitive symptom onset to death or last follow-up, with right censoring of those lost to follow-up. Predictors of survival were assessed with Cox-Proportional Hazards Models.

Results:

Average age of cognitive symptom onset was 69.6 ± 8.4 years, with a median survival of 8.6 years from cognitive symptom onset. Older age at cognitive symptom onset (HR 1.05, 95% CI, 1.04-1.06) and the presence of visual hallucinations (HR 1.6, 95% CI, 1.2-2.2) and cognitive fluctuations (HR 1.6, 95% CI, 1.3-2.1) at or before cognitive symptom onset was associated with shorter survival; sex, years of education, parkinsonism and REM sleep behavior disorder were not associated with survival. Further, a higher number of simultaneous core features at or before onset of cognitive symptoms predicted shorter survival (HR 1.2, 95% CI, 1.1-1.4). In the pathology-proven subgroup, only older age at cognitive symptom onset (HR 1.03. 95% CI, 1.00-1.05), cognitive fluctuations (HR 1.5, 95% CI, 1.01-2.1) and total number of core features (HR 1.3, 95% CI, 1.1-1.5) were associated with shorter survival. 

Conclusions:

Older age at cognitive symptom onset, visual hallucinations, cognitive fluctuations, and higher number of core features at the onset of cognitive symptoms in probable DLB were associated with worse prognosis.  Similar associations were seen in pathology-confirmed DLB, with one exception: visual hallucinations were no longer associated with survival.