Objective: To determine the background pathology of Japanese patients with ‘corticobasal degeneration (CBD) mimics’.

Background: Recent research has focused on the development of disease-modifying treatments for CBD, targeting mainly at tau dysfunction. Accurate diagnosis of CBD is increasingly important, but a major limitation has been the similarity of clinical phenotypes between CBD and ‘CBD mimics’ caused by other pathology.

Methods: Patients for whom detailed clinicopathological findings were available, among those who were diagnosed as clinically CBD or corticobasal syndrome (CBS) during central review. The evaluation items included age at onset, age at time of death, final clinical diagnosis and presence of clinical signs and symptoms both at presentation and during the entire clinical course.

Results: We identified 32 pathologically confirmed patients from 25 Japanese institutions. The age at onset was 65.5±8.9 (mean± SD) years. Disease duration was 7.7±3.1 years. Pathological analyses demonstrated that progressive supranuclear palsy (PSP) were the most common diagnosis (34%), followed by Alzheimer disease (AD; 19%), globular glial tauopathies (GGT; 7%) and frontotemporal lobar degeneration (FTLD)-TDP (7%), and other diseases including dementia with Lewy bodies (DLB), Pick disease, FTLD- FUS, prion disease and glioblastoma.

Conclusions: ‘CBD mimics’ include 4 repeat tauopathies (PSP and GGT; 41%), non-4 repeat tauopathies (AD, Pick disease; 22%) and other diseases (DLB, FTLD, prion disease and glioblastoma etc.). Because disease-modifying treatments for CBD may also be beneficial for 4 repeat tauopathies, exclusion of non-4 repeat tauopathies and other diseases are absolutely necessary.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/background-pathology-of-corticobasal-degeneration-cbd-mimics-japanese-validation-study-of-cbd-j-vac-study/