Objectives: To investigate clinical, imaging, and pathologic associations of the cingulate island sign (CIS) in dementia with Lewy bodies (DLB).Methods: We retrospectively identified and compared patients with a clinical diagnosis of DLB (n 5 39); patients with Alzheimer disease (AD) matched by age, sex, and education (n 5 39); and cognitively normal controls (n 5 78) who underwent 18 F-fluorodeoxyglucose (FDG) and C11 Pittsburgh compound B (PiB)-PET scans. Among these patients, we studied those who came to autopsy and underwent Braak neurofibrillary tangle (NFT) staging (n 5 10). Dementia with Lewy bodies (DLB) is the second most common cause of dementia.1 Despite revised criteria, 2 clinically distinguishing DLB from other neurodegenerative diseases continues to be problematic. In fact, the National Alzheimer's Coordinating Center registry reported the sensitivity of clinical diagnoses of DLB compared with subsequent pathologic examination to be 32.1%.
3Complicating the inaccuracies of clinical diagnosis, the majority of dementia cases have multiple pathologies at autopsy. 4,5 Coexisting Alzheimer disease (AD) pathology occurs in 51% to 66% of those with pathologically confirmed DLB. 1,6 Accurate antemortem prediction of underlying causes of dementia is important for determining a prognosis, 7,8 predicting treatment response, 9 and planning for clinical trials. Additional biomarkers are required to better distinguish DLB from AD and to predict relative contributions of different underlying pathologies to DLB. By measuring b-amyloid (Ab) deposition with C11 Pittsburgh compound B (PiB) binding, investigators have demonstrated that more than half of patients with DLB have Ab deposition in the brain.
10,11From the Departments of Neurology