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OBJECTIVE The objective of the Predict-HD study is to use genetic, neurobiological and refined clinical markers to understand the early progression of Huntington's disease (HD), prior to the point of traditional diagnosis, in persons with a known gene mutation. Here we estimate the approximate onset and initial course of various measurable aspects of HD(More)
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)
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)
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)
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)
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)
BACKGROUND Cognitive decline has been reported in Huntington disease (HD), as well as in the period before diagnosis of motor symptoms (i.e., pre-HD). However, the severity, frequency, and characterization of cognitive difficulties have not been well-described. Applying similar cutoffs to those used in mild cognitive impairment (MCI) research, the current(More)
BACKGROUND Although functional task-specific training is a viable approach for upper extremity neurorehabilitation, its appropriateness for older populations is unclear. If task-specific training is to be prescribed to older adults, it must be efficacious and feasible, even in patients with cognitive decline due to advancing age. OBJECTIVE This(More)