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From the Discipline of Pathology (S.L.F., D.R.C., A.S., C.V.M., J.J.K.), Central Clinical School (J.B.K., W.S.K., E.J., R.H.T., J.R.H., G.M.H.), Faculty of Medicine and Health, Brain and Mind Centre (J.B.K., W.S.K., E.J., R.H.T., O.P., J.R.H., G.M.H.), and School of Psychology (O.P.), The University of Sydney; Neuroscience Research Australia (H.M., C.E.S., A.B.M., A.J.A., F.C.-J., L.B., J.B.K., W.S.K., E.J., R.H.T., O.P., J.R.H., G.M.H.), Sydney; School of Medical Sciences (C.E.S., A.J.A., F.C.-J., J.B.K., W.S.K., R.H.T., G.M.H.), University of New South Wales; and ARC Centre of Excellence in Cognition and its Disorders (O.P., J.R.H.), Sydney, Australia.
Published: May 2019
AI Article Synopsis
Article Abstract
Objective: To investigate the prevalence of clinically relevant multiple system atrophy (MSA) and Lewy body disease (LBD) pathologies in a large frontotemporal lobar degeneration (FTLD) cohort to determine if concomitant pathologies underlie the heterogeneity of clinical features.
Methods: All prospectively followed FTLD-tau and FTLD-TDP cases held by the Sydney Brain Bank (n = 126) were screened for coexisting MSA and LBD (Braak ≥ stage IV) pathology. Relevant clinical (including family history) and genetic associations were determined.
Results: MSA pathology was not identified in this series. Of the FTLD cohort, 9 cases had coexisting LBD ≥ Braak stage IV and were associated with different FTLD subtypes including Pick disease (n = 2), corticobasal degeneration (n = 2), progressive supranuclear palsy (n = 2), and TDP type A (n = 3). All FTLD-TDP cases with coexisting LBD had mutations in (n = 2) or an abnormal repeat expansion in (n = 1). All FTLD-tau cases with coexisting LBD were sporadic. The haplotype was found in all cases that could be genotyped (n = 6 of 9). Seven cases presented with a predominant dementia disorder, 3 of which developed parkinsonism. Two cases presented with a movement disorder and developed dementia in their disease course. The age at symptom onset (62 ± 11 years) and disease duration (8 ± 5 years) in FTLD cases with coexisting LBD did not differ from pure FTLD or pure LBD cases in the brain bank.
Conclusion: Coexisting LBD in FTLD comprises a small proportion of cases but has implications for clinical and neuropathologic diagnoses and the identification of biomarkers.
