Graduation Year


Document Type




Degree Name

Doctor of Philosophy (Ph.D.)

Degree Granting Department


Major Professor

Cynthia R. Cimino, Ph.D.

Committee Member

Paul Jacobsen, Ph.D.

Committee Member

Robert Schlauch, Ph.D.

Committee Member

Sandra Schneider, Ph.D.

Committee Member

Brent Small, Ph.D.


neuropsychology, subcortical dementia, awareness, executive functioning, memory, apathy


Huntington's disease (HD) is a genetic, neurodegenerative disorder that is characterized by motor, cognitive and psychiatric disturbances. Anosognosia, or lack of awareness of symptoms, is commonly observed in neurodegenerative disorders, including HD. Most theories suggest that emotion, executive functioning, and memory play important roles in self-awareness. There is limited research of anosognosia in HD and no theoretical model of how it manifests in the disease. The purpose of this study was to examine Metacognitive Knowledge, or overall beliefs about the self, and Online Awareness, or the ability to predict (Anticipatory Awareness) and evaluate (Emergent Awareness) task performance, in HD. Fifty-six symptomatic HD patients and fifty informants completed the study. Results revealed that those with the best executive functioning and lowest apathy were also better able to report on their symptoms. Those with the best executive functioning and memory and lowest apathy were the best at predicting and evaluating their performance on cognitive tasks. Patient self-report of memory was associated with cognitive performance while self-report of executive functioning and apathy was not. Only informant report of apathy and executive functioning was related to cognitive performance. For both Metacognitive Knowledge and Online Awareness, HD patients tended to have a better awareness of memory than executive functioning. These results suggest that awareness in HD is governed by local monitoring systems rather than a single metacognitive mechanism. It is also consistent with literature that suggests that individuals are least able to evaluate performance on tasks for which they are poorest in skill level, as HD patients tend to have impaired executive functioning and increased apathy with relative sparing of memory.