Abstract

The cingulate island sign (CIS) that reflects sparing of the posterior cingulate cortex (PCC) relative to the precuneus plus cuneus on FDG-PET and brain perfusion SPECT, has been proposed as a feature of dementia with Lewy bodies (DLB). As the CIS is influenced by concomitant Alzheimer’s disease (AD)-type neurofibrillary tangle (NFT) pathology, we postulated that the CIS gradually disappears as DLB progresses. To determine temporal changes in the CIS, 24 patients with mild DLB and 7 with prodromal DLB underwent 123I-IMP–SPECT and MMSE twice at an interval of two years. The CIS was evaluated as a ratio that was derived by dividing IMP accumulation in the PCC with that in the precuneus plus cuneus. We found that the CIS changed over time and that the relationship between CIS ratios and MMSE scores was inverted U-shaped. Thus, the CIS was most obvious in the vicinity of an MMSE score of 22 and it gradually diminished as the MMSE score decreased. Moreover, a lower CIS ratio in mild DLB was associated with a worse prognosis for cognitive decline, presumably due to concomitant AD-type NFT pathology. Our findings would provide a foundation for the appropriate usage of CIS as a biomarker.

Highlights

  • Dementia with Lewy bodies (DLB) shows various clinical manifestations

  • The cingulate island sign (CIS) is reportedly influenced by Alzheimer’s disease (AD)-type neurofibrillary tangle (NFT) pathology[19] and we reinforced the notion by showing relationship between CIS and medial temporal lobe (MTL) atrophy[18]

  • The CIS was evaluated by CIS ratio that was derived by the division of the IMP accumulation in the posterior cingulate cortex (PCC) with that in the precuneus plus cuneus (PpC)

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Summary

Introduction

Dementia with Lewy bodies (DLB) shows various clinical manifestations. Occipital hypometabolism on 18F-FDG-PET images is a useful biomarker that can discriminate DLB from AD5–8 and it is listed as a supportive biomarker in the revised criteria[1]. The cingulate island sign (CIS) on FDG-PET and brain perfusion SPECT has recently been proposed as a neuroimaging feature of DLB16,17. The term refers to sparing of the posterior cingulate cortex (PCC) relative to the precuneus plus cuneus (PpC). This sign is highly specific for an accurate diagnosis of DLB16,17 and was described as a supportive biomarker in the revised criteria[1].

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