A Cortical Substrate for Square-Wave Jerks in Progressive Supranuclear Palsy

Square-wave jerks (SWJs) are the most common saccadic intrusion in progressive supranuclear palsy (PSP), but their genesis is uncertain. We aimed to determine the characteristics of SWJs in PSP (the Richardson subtype) and Parkinson's disease (PD) and to map the brain structures responsible for abnormal SWJ parameters in PSP.

Eye movements in 12 patients with PSP, 12 patients with PD, and 12 age-matched healthy controls were recorded using an infrared corneal reflection device. The rate, mean amplitude, and velocity of SWJs were analyzed offline. Voxel-based morphometry using a 3-Tesla MRI scanner was performed to relate changes in brain volume to SWJ parameters.

The SWJ rate was more than threefold higher in PSP patients than in both PD patients and controls (mean rates: 33.5, 10.3, and 4.3 SWJs per minute, respectively). The volumes of neither the midbrain nor other infratentorial brain regions were correlated with the SWJ rate. Instead, highly significant associations were found for atrophy in the superior, middle, and inferior temporal gyri in the PSP group.

SWJs in PSP are not mediated by midbrain atrophy. Instead, supratentorial cortical structures located mainly in the temporal lobe appear to be deeply involved in the generation of abnormally high SWJ rates in these patients. Known anatomical connections of the temporal lobe to the superior colliculus and the cerebellum might play a role in SWJ genesis.