Systematic assessment of square-wave jerks in progressive supranuclear palsy: a video-oculographic study

Abstract Background The presence of frequent macro-square-wave jerks (SWJs) has been recently included in the diagnostic criteria for progressive supranuclear palsy (PSP). The aim of the current video-oculographic study was to systematically assess the presence and features of SWJs during a brief fixation task in PSP, in comparison with Parkinson’s disease (PD) patients and healthy controls (HC). Methods Thirty-eight PSP patients, 55 PD patients and 40 HC were enrolled in the study. All patients underwent a video-oculographic (VOG) examination including a 5-s fixation task, and the number, duration and amplitude of SWJs were recorded. The diagnostic performance of several SWJs parameters were then compared in distinguishing PSP from PD patients and controls. Results PSP patients showed a higher number and amplitude of SWJs compared to PD patients and controls. At least two SWJs within the 5-s fixation task were observed in 81.6% of PSP patients, 52.7% of PD patients and 25% of HC. The SWJs amplitude was the parameter showing the highest performances in distinguishing PSP from PD (AUC: 0.78) and HC (AUC: 0.88), outperforming the SWJ number and duration. The SWJ amplitude was larger in PSP-Richardson’s syndrome than in PSP-Parkinsonism patients, while no difference was found between PSP patients with different degrees of vertical ocular motor dysfunction. Conclusions This video-oculographic study provides robust evidence of larger SWJs number and amplitude in PSP than in PD patients, with some potential for differential diagnosis, supporting the inclusion of this ocular sign in PSP criteria.