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Aim To investigate the natural course of progression of ocular motor signs in patients with progressive supranuclear palsy (PSP). Methods 114 patients with a possible or probable PSP following NINDS-SPSP criteria seen at the Department of Neurology and the German Center for Vertigo and Balance Disorders (LMU, Munich) were included in this retrospective study. All patients underwent a structured neuro-ophthalmological testing by a orthoptist at initial diagnostic evaluation, 35 patients several times during their course of disease. The following ocular motor signs were extracted from the charts: Saccadic slowing (with normal range of movement) and saccadic paresis (loss of saccade initiation or severe saccadic hypometria) in vertical and horizontal direction, range of up- and downward as well as horizontal ocular motility during smooth pursuit in mm, presence of square wave jerks, function of the vestibulo-ocular reflex on head impulse testing. Clinical ocular motor signs were analysed in all patients in relation to the duration of disease at the time of examination (based on the medical history of the first sign indicative for PSP) or longitudinally during the course of disease in a subgroup of 35 patients. Results In the whole group of PSP patients saccadic abnormalities showed the following distribution over time: at 1 year of disease duration 10% showed only saccadic slowing on upgaze, 40% on up- and downgaze, 21% saccadic paresis on upgaze and 29% complete vertical saccade paresis; at 2 years the proportion of vertical saccade paresis was 32%, at 3 years 65% and at 4 years 82%. Progression of horizontal saccade paresis was slower (year 1: 6%, year 2: 9%, year 3: 21%, year 4: 41%). The subgroup of patients with longitudinal follow up showed a similar tendency. Ocular motility in this group decrease by a mean of 1.5 mm/a on upgaze, 1.6 mm/a on downgaze and 1.4 mm/a on horizontal gaze. The degree of motility loss on up- and downgaze over time showed a good correlation (R2 = 0.71). Conclusion Ocular motor examination can be used as a robust marker of disease progression. Variability between patients however is considerable. Prospective clinical and apparative quantification of ocular motor markers in well-characterized cohorts of PSP patients is needed. [ABSTRACT FROM AUTHOR]