Dementia with Lewy bodies: Basis of cingulate island sign

Jonathan Graff-Radford, Melissa E. Murray, Val J. Lowe, Bradley F. Boeve, Tanis J. Ferman, Scott A. Przybelski, Timothy G. Lesnick, Matthew L. Senjem, Jeffrey L. Gunter, Glenn E. Smith, David S. Knopman, Clifford R. Jack, Dennis W. Dickson, Ronald C. Petersen, Kejal Kantarci

Research output: Contribution to journalArticlepeer-review

90 Scopus citations


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 18F-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). Results: Patients with a clinical diagnosis of DLB had a higher ratio of posterior cingulate to precuneus plus cuneus metabolism, cingulate island sign (CIS), on FDG-PET than patients with AD (p , 0.001), a finding independent of b-amyloid load on PiB-PET (p 5 0.56). Patients with CIS positivity on visual assessment of FDG-PET fit into the group of high- or intermediate-probability DLB pathology and received clinical diagnosis of DLB, not AD. Higher CIS ratio correlated with lower Braak NFT stage (r 5 20.96; p , 0.001). Conclusions: Our study found that CIS on FDG-PET is not associated with fibrillar b-amyloid deposition but indicates lower Braak NFT stage in patients with DLB. Identifying biomarkers that measure relative contributions of underlying pathologies to dementia is critical as neurotherapeutics move toward targeted treatments.

Original languageEnglish (US)
Pages (from-to)801-809
Number of pages9
Issue number9
StatePublished - 2014

ASJC Scopus subject areas

  • Clinical Neurology


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