Neuropsychological performance, disease severity, and depression in progressive supranuclear palsy.

To investigate the relationship between disease severity, cognitive impairment and depression in progressive supranuclear palsy (PSP) we studied a group of 25 patients who fulfilled strict research criteria and 25 matched controls. Disease severity was judged from the duration of symptoms, level of physical disability using the Columbia Rating Scale (CRS), and the degree of eye movement abnormality. The neuropsychological battery was designed to assess attention and executive function, visual and auditory perception, semantic memory and language production. Although the PSP group were significantly impaired on almost all of these measures, the most profound deficits were on tests of sustained and divided attention. There was no correlation between cognitive impairment and either disease duration or scores on the CRS, but performance on tests of attention correlated significantly with the degree of ocular motor impairment. Depression was found to be common in PSP but did not correlate with any other parameters. It is concluded that the cognitive deficit in PSP is widespread and independent of depression. The association between the severity of eye movement disorder and deficits in sustained and divided attention leads us to postulate that pathology involving the midbrain periaqueductal region may be critical for breakdown in these fundamental processes.