Examining Huntington's disease patient and informant concordance on frontally mediated behaviors.
INTRODUCTION
Huntington's disease (HD) is a genetic, neurodegenerative disease that affects cognitive, psychiatric and motor functioning. Frontal subcortical circuits are impacted by disease pathology, resulting in frontally mediated behavioral dysfunction. The purpose of this study was (a) to examine the relationship between both patient and informant reports of frontally mediated behaviors and disease progression and (b) to determine rate of agreement between patient and informant reports of these behaviors in relation to disease progression.


METHOD
Twenty-six HD patients and their informants participated in the study at the University of South Florida. Patient-informant pairs completed the Frontal Systems Behavior Scale (FrSBe) Self and Family ratings forms, respectively. UHDRS (Unified Huntington's Disease Rating Scale) motor scores were obtained from medical records as an index of disease progression.


RESULTS
Only informant report of frontally mediated behaviors of apathy, disinhibition, and executive dysfunction was related to neurological examination results. On average, ratings by patients with less severe motor symptoms were comparable to informant ratings, suggesting intact awareness of deficits. In contrast, ratings of patients with more severe motor symptoms were discrepant from informant data, with informants providing more severe ratings than patients.


CONCLUSIONS
HD patients may show intact awareness of frontally mediated behaviors in less severe stages but become increasingly unaware in more severe stages of the disease. This underscores the importance of clinical decisions regarding patient versus informant report at various stages of the disease.
['Hergert|Danielle C Blinkoff|DC|', 'Sanchez-Ramos|Juan|J|', 'Cimino|Cynthia R|CR|']
D000328:Adult D000368:Aged D014150:Antipsychotic Agents D001364:Awareness D003072:Cognition Disorders D018450:Disease Progression D005260:Female D005625:Frontal Lobe D006220:Haloperidol D006801:Humans D006816:Huntington Disease D008297:Male D001523:Mental Disorders D008875:Middle Aged D012189:Retrospective Studies D018967:Risperidone D012720:Severity of Illness Index
Apathy Disinhibition Executive dysfunction Awareness Huntington’s disease