Behavioral changes in Huntington disease

Objectives: This study aimed to gain a better understanding of behavioral a bnormalities in Huntington disease (HD) and to develop a method for reliabl y assessing these changes. Background: Behavioral changes are a central fea ture of HD and often cause considerable distress and difficulty to patients and their relatives. However, they have received little attention from res earch despite their prevalence and clinical significance. Methods: One hund red thirty-four patients with HD were assessed using the Problem Behaviors Assessment for Huntington Disease (PBA-HD), an instrument for rating the pr esence, severity and frequency of behavioral abnormalities in HD. Results: The findings confirm that behavioral problems are common among patients wit h HD. The most corm-non symptoms were loss of energy and initiative, poor p erseverance and quality of work, impaired judgment, poor self-care and emot ional blunting. Affective symptoms such as depression. anxiety and irritabi lity occurred in around half the patients studied. Psychotic symptoms (hall ucinations and delusions) were rarely reported. Factor analysis distinguish ed three clusters of behavioral symptoms, which were interpreted respective ly as reflecting Apathy, Depression and Irritability. The 'Apathy' factor w as highly correlated with duration of illness. whereas no such relationship was observed for the 'Depression' and 'Irritability' factors. Conclusions: The results suggest that certain behavioral changes are fundamental to the progression of HD, whereas others have a more complex relationship to the disease process, The findings have implications for the choice of behaviora l measures used to evaluate efficacy of therapeutic interventions.