Neuropsychologic Findings in Frontotemporal Dementia with Genetic Mutations

1. A B C

2. Supplemental Figure 5. Representative examples of initial neuropsychologic findings in subjects with behavioral variant frontotemporal dementia presentations at a comparable degree of functional impairment – a 59 year old male with a C9ORF72 mutation (A), a 66 year old male with a MAPT mutation (B), and a 62 year old male with a PGRN mutation (C). Note the variable performance across tests and domains in the C9ORF72 case, poor performance on delayed recall and language measures in the MAPT case, and poor performance on language measures with slightly more variable performance on delayed recall measures in the PGRN case. Interestingly, performance on measures of attention/executive functioning was normal or minimally impaired despite all exhibiting classic frontotemporal dementia features. See Supplemental Figure 6 for their corresponding MRI and FDG-PET scans. Abbreviations for each measure are described in the text and associated with Figure 1. Performance on each measure is presented as Mayo Older American Normative Studies (MOANS) Standard Score, in which 10 represents 50th percentile and 3 represents 1 standard deviation (ie, 7 reflects 1 standard deviation below the mean).