Clinical diagnosis of probable DLB depend on when each core feature emerges and may be related to pathology.

All patients (n=176) met clinical criteria for probable DLB, had transitional or diffuse Lewy body disease (TLBD, DLBD), and were subdivided based on a low (L) Braak NFT stage of 0-III or a high (H) Braak NFT stage of IV-VI. Probable DLB onset age and latency from cognitive symptom onset were compared for TLBD-L (n=30), DLBD-L (n=47), DLBD-H (n=78) and TLBD-H (n=21).

The TLBD-H group met criteria for probable DLB at a median age of 76 years which was older than the medians of 70 years for TLBD-L, DLBD-L or DLBD-H (p<0.001). The latency to probable DLB from cognitive onset was a median < 1 year for TLBD-L and DLBD-L which was shorter than the median of 2 years for DLBD-H (p=0.016), which was shorter than the median of 7 years for TLBD-H (p<0.001).  Examination of Braak NFT stage revealed a median latency ≤ 1.3 years to probable DLB for TLBD and DLBD without neocortical tangles (stages 0 to III) and for DLBD with tangles in the temporal isocortex or association cortices (stages IV and V). In contrast, the latency to diagnosis was a median of 3 years in DLBD with tangles extending to visual cortex (stage VI) (p<0.01), and a median of 7 years for TLBD with neocortical tangles (stages IV to VI) (p<0.001).

Patients with TLBD and neocortical tangles were older and met criteria for probable DLB at the later stages of the disease. A diagnosis of probable DLB was delayed a few years in patients with DLBD with Braak NFT stage VI.