Fig. 10

Distribution of the age at onset and disease duration in previously reported CWP-AD cases. a The age at onset in previously reported CWP-AD cases due to PSEN1 mutations. b The disease duration in previously reported CWP-AD cases due to PSEN1 mutations. c The relationship between the age at onset and disease duration in the present case and previously reported CWP-AD. The data of cases 1 to 28 were extracted from the references cited in Table 2. No significant correlation between the age at onset and disease duration was demonstrated by Spearman rank order correlation analysis when all cases whose disease duration was available were examined (ρ = − 0.101, p = 0.65) or when only previous cases were examined (ρ = 0.058, p = 0.80). These findings suggest that young age at onset is not necessarily a factor that predicts rapid progression or short disease duration in CWP-AD cases. Red solid circle: the present case, purple solid circle: previously reported CWP-AD cases with spastic paraparesis, purple open circle: previously reported CWP-AD cases without spastic paraparesis