RESUMO
BACKGROUND: The frontotemporal lobar degeneration-specific clinical dementia rating (FTLD-CDR), which was recently developed to measure frontotemporal dementia (FTD) severity, includes 2 items that assess language and behavior in addition to the 6 items of the conventional CDR. METHODS: To investigate which of the 3 ratings, i.e. the global score of the CDR (GCDR), the behavioral domain score of the FTLD-CDR (BCDR), or the language domain score of the FTLD-CDR (LCDR), is most suitable for monitoring the progression of semantic dementia (SD), the number of hypometabolic voxels was calculated by comparing 28 SD patients in each stage of the 3 ratings with 63 age/sex-matched controls using voxel-based statistical parametric mapping. RESULTS: The hypometabolic areas increased as a function of the LCDR score in SD patients. However, hypometabolic areas associated with the GCDR did not increase gradually as the stage increased. Furthermore, those associated with the BCDR showed the reverse pattern. CONCLUSION: Our findings suggest that the severity and patterning of glucose hypometabolism measured by the LCDR correspond well with the natural course of SD reported in previous clinical and neuroimaging studies, whereas the BCDR and GCDR did not reflect disease progression in SD.