PROGRESSIVE SUPRANUCLEAR PALSY, SUBCORTICAL DEMENTIA, AND OTHER DEGENERATIVE DISEASES
The first change that Marie noticed in her husband William's behavior that gave her cause for concern was his uncharacteristic impulsiveness and occasional rudeness to other people. Some 5 months after this apparent alteration in his personality, William found that he was having difficulty running or walking. He sometimes felt unsteady and dizzy on his feet and had the occasional fall when he moved too quickly. At about the same time, Marie noticed that his memory was beginning to deteriorate. William could remember things that had happened in the past, but his recall of events or conversations in his every day life was unreliable. He also developed a slight tremor in his right hand.
A year later, his symptoms have progressed. His gait is clumsy and unsteady, and he looks stiff and uncertain as he walks into the room. He has trouble moving his eyes in the lateral plane, and is unable to move them upwards at all, even when following the physician's finger. His WAIS-R FSIQ is 85, however, his vocabulary skills and education suggest he was once functioning well above average. On the Rey Auditory Verbal Learning Test he learns 7 words after 5 trials. Neuropsychological testing provides clear evidence of cognitive dysfunction amounting to a mild dementia. The neurologist confirms that William has progressive supranuclear palsy.
The neurological conditions that have been reviewed so far are those disorders that have been most subjected to neuropsychological or psychosocial investigations. There are, however, a number of other degenerative diseases that have received less attention in this literature, some of which are introduced in this chapter. The first of these is progressive supranuclear palsy (PSP). There is neuropsychological research interest in this disorder because it is often regarded as the best instance of subcor-