Fig. 2.

Relationships between OCT measures and disease severity scales in NPC1-P (a, b) and in NPC1-MC (c). mDRS modified disability rating scale, SARA scale for the assessment and rating of ataxia. Motor score: one point each for presence of a reduced arm swing, intention tremor, increased muscle tone, ankle clonus, and gait abnormalities. Scores ≥ 1 were considered pathological. R² represents a goodness-of-fit measure for linear regression model. pRNFL peripapillary retinal nerve fiber layer thickness, GCIP volumes of combined ganglion cell and inner plexiform layer, OPNL combined outer plexiform layer and outer nuclear layer