Figure 1

Exemplary SD-OCT data sets in individual pure (top) and complex (bottom) HSP patients. Left panels (a,d) demonstrate anatomy of the optic nerve head and indicate placement of the peripapillary ring scan (green circle) in relation to the point of fixation (blue line) recorded by simultaneous scanning laser ophthalmoscopy. In the patient with pure HSP (SPG4), the acquired virtual cross section (b) of the peripapillary retina demonstrates a normal RNFL thickness (delineated by the red lines). Quantitative spatial analysis (c) of this individuals RNFL thickness is displayed as black line in front of data from the normative data set provided by the software (green line = mean, green area = 95% confidence intervall, yellow area = 99% CI, red area = outside 99% CI). Conversely, recordings from a SPG7 patient with complex HSP (d-f) indicate severely decreased RNFL thickness. This was most pronounced in the temporal sector (43 μm, norm: 81 μm). Both temporal inferior (113 μm, norm: 152 μm) and nasal inferior (72 μm, norm: 109 μm) sectors were borderline to pathologic levels, whereas nasal (66 μm, norm: 72 μm), nasal superior (97 μm, norm: 102 μm) and temporal superior (121 μm, norm: 141 μm) sectors did not show significant reduction.