Published in Lancet Neurol on May 09, 2013
Prediction of manifest Huntington's disease with clinical and imaging measures: a prospective observational study. Lancet Neurol (2014) 1.80
Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. J Clin Invest (2015) 1.74
Clinical and Biomarker Changes in Premanifest Huntington Disease Show Trial Feasibility: A Decade of the PREDICT-HD Study. Front Aging Neurosci (2014) 1.53
Reinstating aberrant mTORC1 activity in Huntington's disease mice improves disease phenotypes. Neuron (2014) 1.51
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Compensation in Preclinical Huntington's Disease: Evidence From the Track-On HD Study. EBioMedicine (2015) 1.08
Reassessing models of basal ganglia function and dysfunction. Annu Rev Neurosci (2014) 1.04
Huntington's disease accelerates epigenetic aging of human brain and disrupts DNA methylation levels. Aging (Albany NY) (2016) 1.00
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Manifestation of Huntington's disease pathology in human induced pluripotent stem cell-derived neurons. Mol Neurodegener (2016) 0.94
Allosteric activation of M4 muscarinic receptors improve behavioral and physiological alterations in early symptomatic YAC128 mice. Proc Natl Acad Sci U S A (2015) 0.93
Neurotrophin receptor p75(NTR) mediates Huntington's disease-associated synaptic and memory dysfunction. J Clin Invest (2014) 0.92
Attempted and successful compensation in preclinical and early manifest neurodegeneration - a review of task FMRI studies. Front Psychiatry (2014) 0.92
A two years longitudinal study of a transgenic Huntington disease monkey. BMC Neurosci (2014) 0.89
Triheptanoin improves brain energy metabolism in patients with Huntington disease. Neurology (2015) 0.89
Huntington's disease biomarker progression profile identified by transcriptome sequencing in peripheral blood. Eur J Hum Genet (2015) 0.88
Amyotrophic lateral sclerosis: cell vulnerability or system vulnerability? J Anat (2013) 0.87
Unmet needs for healthcare and social support services in patients with Huntington's disease: a cross-sectional population-based study. Orphanet J Rare Dis (2015) 0.87
Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease. Proc Natl Acad Sci U S A (2016) 0.85
Digitomotography in Parkinson's disease: a cross-sectional and longitudinal study. PLoS One (2015) 0.85
Progressive cognitive deficit, motor impairment and striatal pathology in a transgenic Huntington disease monkey model from infancy to adulthood. PLoS One (2015) 0.85
DNA repair pathways underlie a common genetic mechanism modulating onset in polyglutamine diseases. Ann Neurol (2016) 0.84
A clinical classification acknowledging neuropsychiatric and cognitive impairment in Huntington's disease. Orphanet J Rare Dis (2014) 0.83
An exploratory double-blind, randomized clinical trial with selisistat, a SirT1 inhibitor, in patients with Huntington's disease. Br J Clin Pharmacol (2015) 0.83
Clinical and genetic study of hereditary spastic paraplegia in Canada. Neurol Genet (2016) 0.83
Possible involvement of self-defense mechanisms in the preferential vulnerability of the striatum in Huntington's disease. Front Cell Neurosci (2014) 0.81
Recent imaging advances in neurology. J Neurol (2015) 0.81
Everyday cognition in prodromal Huntington disease. Neuropsychology (2014) 0.81
The effect of multidisciplinary rehabilitation on brain structure and cognition in Huntington's disease: an exploratory study. Brain Behav (2015) 0.80
Selective vulnerability of Rich Club brain regions is an organizational principle of structural connectivity loss in Huntington's disease. Brain (2015) 0.80
Preliminary analysis using multi-atlas labeling algorithms for tracing longitudinal change. Front Neurosci (2015) 0.80
Prospects for neuroprotective therapies in prodromal Huntington's disease. Mov Disord (2014) 0.80
Decade in review-movement disorders: Tracking the pathogenesis of movement disorders. Nat Rev Neurol (2015) 0.80
Medication Use in Early-HD Participants in Track-HD: an Investigation of its Effects on Clinical Performance. PLoS Curr (2016) 0.79
Systemic delivery of P42 peptide: a new weapon to fight Huntington's disease. Acta Neuropathol Commun (2014) 0.79
A retrospective analysis of hand tapping as a longitudinal marker of disease progression in Huntington's disease. BMC Neurol (2014) 0.79
The role of the amygdala during emotional processing in Huntington's disease: from pre-manifest to late stage disease. Neuropsychologia (2015) 0.79
Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/1 mouse model of Huntington's disease. PLoS One (2013) 0.79
A Computational Cognitive Biomarker for Early-Stage Huntington's Disease. PLoS One (2016) 0.78
Striatal metabolism and psychomotor speed as predictors of motor onset in Huntington's disease. J Neurol (2014) 0.78
Motor network structure and function are associated with motor performance in Huntington's disease. J Neurol (2016) 0.78
Abnormal cerebellar volume and corticocerebellar dysfunction in early manifest Huntington's disease. J Neurol (2015) 0.78
Functional connectivity modeling of consistent cortico-striatal degeneration in Huntington's disease. Neuroimage Clin (2015) 0.78
Neuropsychiatry and White Matter Microstructure in Huntington's Disease. J Huntingtons Dis (2015) 0.78
Neuroimaging in Huntington's disease. World J Radiol (2014) 0.78
Cerebrospinal Fluid Inflammatory Biomarkers Reflect Clinical Severity in Huntington's Disease. PLoS One (2016) 0.78
Phonatory dysfunction as a preclinical symptom of Huntington disease. PLoS One (2014) 0.78
Neurofilament light chain: a biomarker for genetic frontotemporal dementia. Ann Clin Transl Neurol (2016) 0.77
Cholinergic interneurons in the Q140 knockin mouse model of Huntington's disease: Reductions in dendritic branching and thalamostriatal input. J Comp Neurol (2016) 0.77
Huntington's disease: the road to progress. Lancet Neurol (2013) 0.77
Cross-sectional and longitudinal multimodal structural imaging in prodromal Huntington's disease. Mov Disord (2016) 0.77
Multivariate clustering of progression profiles reveals different depression patterns in prodromal Huntington disease. Neuropsychology (2015) 0.77
Linking white matter and deep gray matter alterations in premanifest Huntington disease. Neuroimage Clin (2016) 0.77
Early grey matter changes in structural covariance networks in Huntington's disease. Neuroimage Clin (2016) 0.76
Abnormal Electrophysiological Motor Responses in Huntington's Disease: Evidence of Premanifest Compensation. PLoS One (2015) 0.76
Test-Retest Reliability of Diffusion Tensor Imaging in Huntington's Disease. PLoS Curr (2014) 0.76
Common disease signatures from gene expression analysis in Huntington's disease human blood and brain. Orphanet J Rare Dis (2016) 0.76
The many facets of unawareness in huntington disease. Tremor Other Hyperkinet Mov (N Y) (2014) 0.76
Structural and molecular myelination deficits occur prior to neuronal loss in the YAC128 and BACHD models of Huntington disease. Hum Mol Genet (2016) 0.76
Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntington's disease: a retrospective cohort analysis. Lancet Neurol (2017) 0.76
Quantitative motor assessment of muscular weakness in myasthenia gravis: a pilot study. BMC Neurol (2015) 0.75
Characterising Upper Limb Movements in Huntington's Disease and the Impact of Restricted Visual Cues. PLoS One (2015) 0.75
Huntington disease reduced penetrance alleles occur at high frequency in the general population. Neurology (2016) 0.75
Novel Nut and Bolt Task Quantifies Motor Deficits in Premanifest and Manifest Huntington's Disease. PLoS Curr (2015) 0.75
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Laquinimod rescues striatal, cortical and white matter pathology and results in modest behavioural improvements in the YAC128 model of Huntington disease. Sci Rep (2016) 0.75
Assistive Technology for Cognition and Health-related Quality of Life in Huntington's Disease. J Huntingtons Dis (2016) 0.75
Increased irritability, anxiety, and immune reactivity in transgenic Huntington's disease monkeys. Brain Behav Immun (2016) 0.75
Engaging cognitive circuits to promote motor recovery in degenerative disorders. exercise as a learning modality. J Hum Kinet (2016) 0.75
Health-related quality of life and unmet healthcare needs in Huntington's disease. Health Qual Life Outcomes (2017) 0.75
Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.Htt(Q111/+) model of Huntington's disease. Sci Rep (2017) 0.75
Social Cognition, Executive Functions and Self-Report of Psychological Distress in Huntington's Disease. PLoS Curr (2016) 0.75
Depressive symptoms can amplify embarrassment in essential tremor. J Clin Mov Disord (2016) 0.75
Longitudinal Changes in the Motor Learning-Related Brain Activation Response in Presymptomatic Huntington's Disease. PLoS One (2016) 0.75
Validation of a prognostic index for Huntington's disease. Mov Disord (2016) 0.75
An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes. Hum Mol Genet (2016) 0.75
An automated and quantitative method to evaluate progression of striatal pathology in Huntington's disease transgenic mice. J Huntingtons Dis (2014) 0.75
Dysregulation of Corticostriatal Connectivity in Huntington's Disease: A Role for Dopamine Modulation. J Huntingtons Dis (2016) 0.75
The BACHD Rat Model of Huntington Disease Shows Signs of Fronto-Striatal Dysfunction in Two Operant Conditioning Tests of Short-Term Memory. PLoS One (2017) 0.75
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Reverse Translation in Parkinson Disease. J Nucl Med (2016) 0.75
Longitudinal diffusion changes in prodromal and early HD: Evidence of white-matter tract deterioration. Hum Brain Mapp (2017) 0.75
Intact sensory-motor network structure and function in far from onset premanifest Huntington's disease. Sci Rep (2017) 0.75
Bupropion for the treatment of apathy in Huntington's disease: A multicenter, randomised, double-blind, placebo-controlled, prospective crossover trial. PLoS One (2017) 0.75
Molecular Imaging Markers to Track Huntington's Disease Pathology. Front Neurol (2017) 0.75
Modeling Variability in the Progression of Huntington's Disease A Novel Modeling Approach Applied to Structural Imaging Markers from TRACK-HD. CPT Pharmacometrics Syst Pharmacol (2016) 0.75
Integration of targeted metabolomics and transcriptomics identifies deregulation of phosphatidylcholine metabolism in Huntington's disease peripheral blood samples. Metabolomics (2016) 0.75
Huntington's disease: of mice and men. Oncotarget (2017) 0.75
COMT Val158Met Polymorphism Modulates Huntington's Disease Progression. PLoS One (2016) 0.75
Cystathionine Levels in Patients With Huntington Disease. PLoS Curr (2015) 0.75
Longitudinal in vivo MRI in a Huntington's disease mouse model: Global atrophy in the absence of white matter microstructural damage. Sci Rep (2016) 0.75
Striatal morphology correlates with frontostriatal electrophysiological motor processing in Huntington's disease: an IMAGE-HD study. Brain Behav (2016) 0.75
Problem solving, impulse control and planning in patients with early- and late-stage Huntington's disease. Eur Arch Psychiatry Clin Neurosci (2016) 0.75
The Generation of Mouse and Human Huntington Disease iPS Cells Suitable for In vitro Studies on Huntingtin Function. Front Mol Neurosci (2017) 0.75
Therapies targeting DNA and RNA in Huntington's disease. Lancet Neurol (2017) 0.75
Progress in Huntington's disease: the search for markers of disease onset and progression. J Neurol (2015) 0.75
Statistical Approaches to Longitudinal Data Analysis in Neurodegenerative Diseases: Huntington's Disease as a Model. Curr Neurol Neurosci Rep (2017) 0.75
Quantitative motor assessment of dyskinesias in Parkinson's disease. J Neural Transm (Vienna) (2015) 0.75
Phenotype, genotype, and worldwide genetic penetrance of LRRK2-associated Parkinson's disease: a case-control study. Lancet Neurol (2008) 6.76
Automatic classification of MR scans in Alzheimer's disease. Brain (2008) 6.71
Infant feeding patterns and risks of death and hospitalization in the first half of infancy: multicentre cohort study. Bull World Health Organ (2005) 5.07
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat Genet (2003) 4.76
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell (2006) 4.53
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat Neurosci (2002) 4.15
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet (2003) 4.07
Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data. Lancet Neurol (2009) 3.82
Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. Proc Natl Acad Sci U S A (2004) 3.73
Preparing for preventive clinical trials: the Predict-HD study. Arch Neurol (2006) 3.57
Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis. Lancet Neurol (2010) 3.47
A longitudinal study of brain volume changes in normal aging using serial registered magnetic resonance imaging. Arch Neurol (2003) 3.44
Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron (2002) 3.15
Automatic differentiation of anatomical patterns in the human brain: validation with studies of degenerative dementias. Neuroimage (2002) 3.06
Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data. Lancet Neurol (2011) 2.99
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Psychiatric symptoms in Huntington's disease before diagnosis: the predict-HD study. Biol Psychiatry (2007) 2.71
Prevalence of angina in women versus men: a systematic review and meta-analysis of international variations across 31 countries. Circulation (2008) 2.56
Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Hum Mol Genet (2007) 2.54
Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nat Cell Biol (2002) 2.50
Adult polyglucosan body disease: Natural History and Key Magnetic Resonance Imaging Findings. Ann Neurol (2012) 2.33
A multicenter study of glucocerebrosidase mutations in dementia with Lewy bodies. JAMA Neurol (2013) 2.32
A contribution of cognitive decision models to clinical assessment: decomposing performance on the Bechara gambling task. Psychol Assess (2002) 2.32
Registration and machine learning-based automated segmentation of subcortical and cerebellar brain structures. Neuroimage (2007) 2.31
Abnormal brain structure in children with isolated clefts of the lip or palate. Arch Pediatr Adolesc Med (2007) 2.29
Using cognitive models to map relations between neuropsychological disorders and human decision-making deficits. Psychol Sci (2005) 2.25
Biological and clinical characteristics of individuals at risk for spinocerebellar ataxia types 1, 2, 3, and 6 in the longitudinal RISCA study: analysis of baseline data. Lancet Neurol (2013) 2.24
Genome-wide association study confirms BST1 and suggests a locus on 12q24 as the risk loci for Parkinson's disease in the European population. Hum Mol Genet (2010) 2.21
Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients. Curr Biol (2009) 2.19
Mutations in SPG11, encoding spatacsin, are a major cause of spastic paraplegia with thin corpus callosum. Nat Genet (2007) 2.17
Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington's disease. Brain (2007) 2.17
Neurocognitive signs in prodromal Huntington disease. Neuropsychology (2011) 2.14
fMRI biomarker of early neuronal dysfunction in presymptomatic Huntington's Disease. AJNR Am J Neuroradiol (2004) 2.10
Assessing the onset of structural change in familial Alzheimer's disease. Ann Neurol (2003) 2.07
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease. J Neurosci (2002) 2.05
Change in rates of cerebral atrophy over time in early-onset Alzheimer's disease: longitudinal MRI study. Lancet (2003) 2.02
Oxytocin attenuates amygdala reactivity to fear in generalized social anxiety disorder. Neuropsychopharmacology (2010) 1.98
Benign hereditary chorea: phenotype, prognosis, therapeutic outcome and long term follow-up in a large series with new mutations in the TITF1/NKX2-1 gene. J Neurol Neurosurg Psychiatry (2012) 1.94
Intensity non-uniformity correction using N3 on 3-T scanners with multichannel phased array coils. Neuroimage (2007) 1.93
Follow-up by mail in clinical trials: does questionnaire length matter? Control Clin Trials (2004) 1.88
Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proc Natl Acad Sci U S A (2005) 1.87
Clinical relevance of low serum vitamin B12 concentrations in older people: the Banbury B12 study. Age Ageing (2006) 1.86
A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study. Am J Hum Genet (2003) 1.85
Hereditary spastic paraplegias: an update. Curr Opin Neurol (2007) 1.83
Neurocognitive insights into substance abuse. Trends Cogn Sci (2005) 1.82
Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease. J Neurosci (2005) 1.82
Amyloid load and cerebral atrophy in Alzheimer's disease: an 11C-PIB positron emission tomography study. Ann Neurol (2006) 1.81
Identification of the SPG15 gene, encoding spastizin, as a frequent cause of complicated autosomal-recessive spastic paraplegia, including Kjellin syndrome. Am J Hum Genet (2008) 1.79
Striatal and white matter predictors of estimated diagnosis for Huntington disease. Brain Res Bull (2010) 1.79
HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum Mol Genet (2002) 1.79
A genome-scale DNA repair RNAi screen identifies SPG48 as a novel gene associated with hereditary spastic paraplegia. PLoS Biol (2010) 1.77
Practice effects in the prediction of long-term cognitive outcome in three patient samples: a novel prognostic index. Arch Clin Neuropsychol (2006) 1.77
Mutations in KCND3 cause spinocerebellar ataxia type 22. Ann Neurol (2012) 1.76
"Frontal" behaviors before the diagnosis of Huntington's disease and their relationship to markers of disease progression: evidence of early lack of awareness. J Neuropsychiatry Clin Neurosci (2010) 1.76
Mutations in SPG11 are frequent in autosomal recessive spastic paraplegia with thin corpus callosum, cognitive decline and lower motor neuron degeneration. Brain (2007) 1.76
Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington's disease patients. J Clin Invest (2015) 1.74
Motor abnormalities in premanifest persons with Huntington's disease: the PREDICT-HD study. Mov Disord (2009) 1.73
Alteration of fatty-acid-metabolizing enzymes affects mitochondrial form and function in hereditary spastic paraplegia. Am J Hum Genet (2012) 1.71
Using genome-wide complex trait analysis to quantify 'missing heritability' in Parkinson's disease. Hum Mol Genet (2012) 1.68
An item response analysis of the motor and behavioral subscales of the unified Huntington's disease rating scale in huntington disease gene expansion carriers. Mov Disord (2011) 1.68
Transgenic rat model of Huntington's disease. Hum Mol Genet (2003) 1.66
Pridopidine for the treatment of motor function in patients with Huntington's disease (MermaiHD): a phase 3, randomised, double-blind, placebo-controlled trial. Lancet Neurol (2011) 1.62
A meta-analysis of hippocampal atrophy rates in Alzheimer's disease. Neurobiol Aging (2008) 1.61
Meta-analysis of randomised trials of monetary incentives and response to mailed questionnaires. J Epidemiol Community Health (2005) 1.60