Phenotypic presentation may have an effect on survival in amyotrophic lateral sclerosis–frontotemporal dementia (ALS-FTD). While overall cognitive deficits were similar in individuals with motor onset vs cognitive onset, initial presentations of motor symptoms led to faster disease progression.
Why this matters
Evidence from previous studies suggests that compared to individuals with pure ALS and those with behavioral variant FTD (bvFTD), individuals with ALS-FTD show a more rapid progression to death. Understanding the effect of initial motor and cognitive presentation on survival in individuals with ALS-FTD may help to identify specific neuroimaging markers to identify disease progression patterns.