Progressive supranuclear palsy with action myoclonus, seizures.

A case of atypical progressive supranuclear palsy is presented that differs from the classical description of the disorder. The patient was a 50 year old woman who had a neurologic disturbance that progressed over eight years and that was studied clinically and pathologically. The case differs from classical progressive supranuclear palsy in that action myoclonus, seizures, and a late eye-movement disorder were present, while rigidity and dementia were relatively absent. An additional difference is that the most prominent pathologic findings were in the midbrain, dentate nuclei, and cerebral cortex. If the case is properly classified, it is unique in its clinical presentation and in the distribution of lesions.