Search Results - "Langbehn, Douglas R."
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Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data
Published in Lancet neurology (01-07-2013)“…Summary Background TRACK-HD is a multinational prospective observational study of Huntington's disease (HD) that examines clinical and biological findings of…”
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Huntington disease: natural history, biomarkers and prospects for therapeutics
Published in Nature reviews. Neurology (01-04-2014)“…Key Points No disease-modifying treatments are currently available for Huntington disease (HD), but clinical trials of potential compounds are imminent;…”
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Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis
Published in Lancet neurology (2011)“…Summary Background TRACK-HD is a prospective observational study of Huntington's disease (HD) that examines disease progression in premanifest individuals…”
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Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data
Published in Lancet neurology (2012)“…Summary Background TRACK-HD is a prospective observational biomarker study in premanifest and early Huntington's disease (HD). In this report we define a…”
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Psychiatric Symptoms in Huntington’s Disease before Diagnosis: The Predict-HD Study
Published in Biological psychiatry (1969) (15-12-2007)“…Background Psychiatric disturbances are relatively common in manifest Huntington’s disease (HD), but less is known about these symptoms in the earliest phase…”
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Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data
Published in Lancet neurology (01-09-2009)“…Summary Background Huntington's disease (HD) is an autosomal dominant, fully penetrant, neurodegenerative disease that most commonly affects adults in…”
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CAG-repeat length and the age of onset in Huntington disease (HD): A review and validation study of statistical approaches
Published in American journal of medical genetics. Part B, Neuropsychiatric genetics (01-03-2010)“…CAG‐repeat length in the gene for HD is inversely correlated with age of onset (AOO). A number of statistical models elucidating the relationship between CAG…”
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Operationalizing compensation over time in neurodegenerative disease
Published in Brain (London, England : 1878) (01-04-2017)“…In pre-clinical Huntington's disease, normal behaviour is maintained despite neurodegeneration, suggesting a mechanism of compensation. Gregory, Long et al …”
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A genetic association study of glutamine-encoding DNA sequence structures, somatic CAG expansion, and DNA repair gene variants, with Huntington disease clinical outcomes
Published in EBioMedicine (01-10-2019)“…Huntington disease (HD) is caused by an unstable CAG/CAA repeat expansion encoding a toxic polyglutamine tract. Here, we tested the hypotheses that HD outcomes…”
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Longer CAG repeat length is associated with shorter survival after disease onset in Huntington disease
Published in American journal of human genetics (06-01-2022)“…It is well known that the length of the CAG trinucleotide expansion of the huntingtin gene is associated with many aspects of Huntington disease progression…”
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The impact of occipital lobe cortical thickness on cognitive task performance: An investigation in Huntington's Disease
Published in Neuropsychologia (01-12-2015)“…The occipital lobe is an important visual processing region of the brain. Following consistent findings of early neural changes in the occipital lobe in…”
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Cerebral cortex structure in prodromal Huntington disease
Published in Neurobiology of disease (01-12-2010)“…Abstract Neuroimaging studies of subjects who are gene-expanded for Huntington Disease, but not yet diagnosed (termed prodromal HD), report that the cortex is…”
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Early atrophy of pallidum and accumbens nucleus in Huntington’s disease
Published in Journal of neurology (01-03-2011)“…In Huntington’s disease (HD) atrophy of the caudate nucleus and putamen has been described many years before clinical manifestation. Volume changes of the…”
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Response to Lee et al
Published in American journal of human genetics (07-07-2022)Get full text
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Progressive alterations in white matter microstructure across the timecourse of Huntington's disease
Published in Brain and behavior (01-04-2023)“…Background Whole‐brain longitudinal diffusion studies are crucial to examine changes in structural connectivity in neurodegeneration. Here, we investigated the…”
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Behavioral features in child and adolescent huntingtin gene‐mutation carriers
Published in Brain and behavior (01-07-2022)“…Introduction We compared neuropsychiatric symptoms between child and adolescent huntingtin gene‐mutation carriers and noncarriers. Given previous evidence of…”
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Self-Paced Timing Detects and Tracks Change in Prodromal Huntington Disease
Published in Neuropsychology (01-07-2010)“…Objective: This study compares self-paced timing performance (cross-sectionally and longitudinally) between participants with prodromal Huntington's disease (…”
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Clinical Outcomes and Selection Criteria for Prodromal Huntington's Disease Trials
Published in Movement disorders (01-12-2020)“…Background Huntington's disease (HD) develops in individuals with extended cytosine‐adenine‐guanine (CAG) repeats within the huntingtin (HTT) gene, causing…”
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Utility of the Huntington's Disease Prognostic Index Score for a Perimanifest Clinical Trial
Published in Movement disorders (01-05-2022)“…Background Subtle neurodegenerative motor and cognitive impairments accumulate over a prodromal period several years before clinical diagnosis of Huntington's…”
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Abnormal brain development in child and adolescent carriers of mutant huntingtin
Published in Neurology (03-09-2019)“…OBJECTIVEThe huntingtin gene is critical for the formation and differentiation of the CNS, which raises questions about the neurodevelopmental effect of CAG…”
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