Staging of Lewy-related pathology in dementia.

OBJECTIVE

Lewy-related pathology is the characteristic feature of Parkinson's disease with and without dementia and dementia with Lewy bodies (DLB). There are two neuropathological staging systems for Lewy-related pathology commonly employed today: the staging system for Parkinson-related pathology by Braak et al., and the staging system by the Consortium on DLB. There are also several modified systems based on these two scales.

METHODS

We applied a total of eight different staging systems for Lewy-related pathology to 36 consecutive demented patients with various dementia disorders.

RESULTS

The staging systems varied considerably in number of unclassifiable cases (range 0 – 16 out of 36 cases), while the diagnostic agreement between the systems that were able to classify all or the very majority of cases varied only slightly (weighted κ 0.86 – 0.92 and Spearman's σ 0.80 – 1.0).

CONCLUSION

The different staging systems for Lewy-related pathology that exist today vary in staging procedure and proportion of unclassifiable cases. The choice of system may affect the stage of Lewy-related pathology and ultimately final diagnosis.