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Neurology 2003;60:1005-1011
© 2003 American Academy of Neurology

Staging disease severity in pathologically confirmed cases of frontotemporal dementia

M. Broe, PhD, J.R. Hodges, MD FRCP, E. Schofield, BSc(Hons), C.E. Shepherd, PhD, J.J. Kril, PhD and G.M. Halliday, PhD

From the Centre for Education and Research on Ageing (Drs. Broe, Shepherd, and Kril), Department of Medicine, The University of Sydney; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; and Prince of Wales Medical Research Institute and the University of New South Wales (E. Schofield and Dr. Halliday), Sydney, Australia.

Address correspondence and reprint requests to Assoc. Prof. Glenda Halliday, Prince of Wales Medical Research Institute, Barker Street, Randwick, 2031 Australia; e-mail: G.Halliday{at}unsw.edu.au

Objective: To devise a staging scheme for addressing the severity of atrophy in patients with pathologically proven frontotemporal dementia (FTD) and determine any relationship with clinical indices.

Methods: Twenty-four cases with clinical and pathologic features of FTD were selected using standard inclusion and exclusion criteria from 125 dementia cases collected in Sydney, Australia, over an 8.5-year period. Patterns of gross atrophy were determined in two coronal brain slices. Reproducibility of a four-stage severity scheme was tested. Nonparametric statistics were used to determine relationships between the stage of atrophy and clinical indices (age at death, duration from diagnosis, and clinical severity at death).

Results: The FTD cases studied could be reliably grouped ({kappa} = 0.97) into four progressively severe stages of global atrophy. Initial mild atrophy occurred in the orbital and superior medial frontal cortices and hippocampus (stage 1), progressed to involve the other anterior frontal regions, temporal cortices, and basal ganglia (stage 2), then involved all remaining tissue in these coronal slices (stage 3), until very marked atrophy was observed in all areas (stage 4). These stages correlated with disease duration and clinical dementia severity, lending validity to the progressive nature of the staging scheme.

Conclusions: The authors have identified a reproducible staging system for the severity of gross atrophy in cases of FTD. This staging scheme provides the required framework to compare different research indices and determine correlates relating to time and disease progression in FTD—information necessary to determine core disease processes and etiologic factors.






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