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The past decade has witnessed an explosion of evidence suggesting that many neurodegenerative diseases can be detected years, if not decades, earlier than previously thought. To date, these scientific advances have not provoked any parallel translational or clinical improvements. There is an urgency to capitalize on this momentum so earlier detection of(More)
The Unified Huntington's Disease Rating Scale is used to characterize motor impairments and establish motor diagnosis. Little is known about the timing of diagnostic confidence level categories and the trajectory of motor impairments during the prodromal phase. Goals of this study were to estimate the timing of categories, model the prodromal trajectory of(More)
Clinical trials to improve day-to-day function in Huntington disease (HD) require accurate outcome measures. The DSM-5 recommends the World Health Organization Disability Assessment Schedule (WHODAS) 2.0 for use in neuropsychiatric disorders. The DSM-5 also states proxy measures may be useful when cognitive function may be impaired. We tested WHODAS(More)
OBJECTIVE Psychiatric symptoms are a significant aspect of Huntington's disease, an inherited neurodegenerative illness. The presentation of these symptoms is highly variable, and their course does not fully correlate with motor or cognitive disease progression. The authors sought to better understand the development and longitudinal course of psychiatric(More)
The Diagnostic and Statistical Manual for Mental Disorders, Fifth Edition (DSM-5), recommends the World Health Organization Disability Assessment Schedule (WHODAS) 2.0 for routine clinical use. We tested the utility of the 12-item WHODAS 2.0 in prodromal Huntington disease. Using data from 726 participants and 630 companions over a 3-year follow-up, linear(More)
OBJECTIVE Assessment of daily functions affected by cognitive loss in prodromal Huntington's disease (HD) is necessary in practice and clinical trials. We evaluated baseline and longitudinal sensitivity of the Everyday Cognition (ECog) scales in prodromal HD and compared self- and companion-ratings. METHOD Everyday cognition was self-assessed by 850(More)
An intracranial tumor is a rare cause of secondary parkinsonism. Our patient presented to our clinic for recently-developed asymmetric parkinsonism without pyramidal signs. However, a meningioma located in the sphenoidal ridge was identified upon imaging studies. This case suggests that additional causes should be considered when approaching patients with(More)
A health outcome can be observed at a spatial location and we wish to relate this to a set of environmental measurements made on a sampling grid. The environmental measurements are covariates in the model but due to the interpolation associated with the grid there is an error inherent in the covariate value used at the outcome location. Since there may be(More)
Huntington disease (HD) is a neurodegenerative condition with prominent motor (including oculomotor), cognitive, and psychiatric effects. While neuropsychological deficits are present in HD, motor impairments may impact performance on neuropsychological measures, especially those requiring a speeded response, as has been demonstrated in multiple sclerosis(More)
The relationship between exposure to environmental chemicals during pregnancy and early childhood development is an important issue that has a spatial risk component. In this context, we have examined mental retardation and developmental delay (MRDD) outcome measures for children in a Medicaid population in South Carolina and sampled measures of soil(More)