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Motor function decline correlates with behavioral impairment in C9orf72 mutation carriers

Expansion mutations in the C9orf72 gene are associated with clinical presentations of amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), and mixed ALS-FTD features.1 Clinical phenotypes in neurodegenerative disorders have been attributed to the progression of degeneration along specific networks, implying that different networks produce symptoms of ALS and FTD. In a longitudinal study of C9orf72 expansion carriers, we found that the ALS Functional Rating Scale (ALSFRS-R) declined in patients meeting the criteria for ALS or ALS-FTD, and the Frontal Behavioral Inventory (FBI) worsened in patients meeting the criteria for FTD/dementia or ALS-FTD.2 To explore whether degeneration affects motor, cognitive, and behavioral domains differentially, we examined correlations between changes in ALSFRS-R scores, FBI scores, and cognitive measures over a 6-month period in C9orf72 carriers.