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Previous studies of emotion recognition suggest that detection of disgust relies on processing within the basal ganglia and insula. Research involving individuals with symptomatic and pre-diagnostic Huntington's disease (HD), a disease with known basal ganglia atrophy, has generally indicated a relative impairment in recognizing disgust. However, some data(More)
Although initially developed as a brief dementia battery, the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) has not yet demonstrated its sensitivity, specificity, and positive and negative predictive powers in detecting cognitive impairment in patients with Alzheimer's disease (AD). Therefore, the current study examined the(More)
We examined the gold standard for Huntington disease (HD) functional assessment, the Unified Huntington's Disease Rating Scale (UHDRS), in a group of at-risk participants not yet diagnosed but who later phenoconverted to manifest HD. We also sought to determine which skill domains first weaken and the clinical correlates of declines. Using the UHDRS Total(More)
OBJECTIVE This study compares self-paced timing performance (cross-sectionally and longitudinally) between participants with prodromal Huntington's disease (pr-HD) and a comparison group of gene non-expanded participants from affected families (NC). METHOD Participants (747 pr-HD: 188 NC) listened to tones presented at 550-ms intervals, matched that pace(More)
Parkinson's disease (PD) is progressive neurological disease characterized by resting tremor, rigidity, akinesia, postural instability and cognitive changes. The symptoms of PD are debilitating and often become unsatisfactorily treated by medication. Deep brain stimulation (DBS) is an effective treatment to significantly reduce the cardinal motor symptoms(More)
OBJECTIVE PREDICT-HD is a large-scale international study of people with the Huntington disease (HD) CAG-repeat expansion who are not yet diagnosed with HD. The objective of this study was to determine the stage in the HD prodrome at which cognitive differences from CAG-normal controls can be reliably detected. METHOD For each of 738 HD CAG-expanded(More)
BACKGROUND Psychiatric disturbances are relatively common in manifest Huntington's disease (HD), but less is known about these symptoms in the earliest phase of the illness. METHODS This study examined self-reported psychiatric symptoms in a large sample (N = 681) of prediagnosed individuals who show the gene expansion for HD ("expansion-positive")(More)
Accurate understanding of practice characteristics, performance stability, and error on neuropsychological tests is essential to both valid clinical assessment and maximization of signal detection for clinical trials of cognitive enhancing drugs. We examined practice effects in 28 healthy adults. As part of a larger study using donepezil and simulating a(More)
  • Kevin Duff
  • 2012
Repeated assessments are a relatively common occurrence in clinical neuropsychology. The current paper will review some of the relevant concepts (e.g., reliability, practice effects, alternate forms) and methods (e.g., reliable change index, standardized based regression) that are used in repeated neuropsychological evaluations. The focus will be on the(More)
Huntington's disease has been linked with fronto-subcortical neuropathology and behaviors consistent with this dysfunction. Little is known about these "frontal" behaviors in the earliest phase of the illness. Comparisons between participants in the Predict-HD study (745 "expansion-positive" and 163 "expansion-negative" control subjects) on the Frontal(More)