Published in Lancet Neurol on December 02, 2010
Cognitive impairment in Huntington disease: diagnosis and treatment. Curr Neurol Neurosci Rep (2011) 1.55
Energy deficit in Huntington disease: why it matters. J Clin Invest (2011) 1.51
Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease. J Neurol Neurosurg Psychiatry (2012) 1.11
Compensation in Preclinical Huntington's Disease: Evidence From the Track-On HD Study. EBioMedicine (2015) 1.08
Cognitive decline in prodromal Huntington Disease: implications for clinical trials. J Neurol Neurosurg Psychiatry (2013) 1.07
Hdac6 knock-out increases tubulin acetylation but does not modify disease progression in the R6/2 mouse model of Huntington's disease. PLoS One (2011) 1.07
Regionally selective atrophy of subcortical structures in prodromal HD as revealed by statistical shape analysis. Hum Brain Mapp (2012) 1.07
Bone marrow transplantation confers modest benefits in mouse models of Huntington's disease. J Neurosci (2012) 1.03
A review of quality of life after predictive testing for and earlier identification of neurodegenerative diseases. Prog Neurobiol (2013) 1.03
Identification of health-related quality of life (HRQOL) issues relevant to individuals with Huntington disease. J Health Psychol (2012) 1.02
Stability of resting fMRI interregional correlations analyzed in subject-native space: a one-year longitudinal study in healthy adults and premanifest Huntington's disease. Neuroimage (2011) 0.98
Brain metabolite alterations and cognitive dysfunction in early Huntington's disease. Mov Disord (2012) 0.97
The long-term safety and efficacy of bilateral transplantation of human fetal striatal tissue in patients with mild to moderate Huntington's disease. J Neurol Neurosurg Psychiatry (2013) 0.96
Self Reports of Day-to-Day Function in a Small Cohort of People with Prodromal and Early HD. PLoS Curr (2011) 0.96
Loss of corticostriatal and thalamostriatal synaptic terminals precedes striatal projection neuron pathology in heterozygous Q140 Huntington's disease mice. Neurobiol Dis (2013) 0.95
HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response. Proc Natl Acad Sci U S A (2014) 0.94
Pathogenic cellular phenotypes are germline transmissible in a transgenic primate model of Huntington's disease. Stem Cells Dev (2013) 0.94
Progress and prospects for genetic modification of nonhuman primate models in biomedical research. ILAR J (2013) 0.93
Iron accumulates in Huntington's disease neurons: protection by deferoxamine. PLoS One (2013) 0.93
Ophthalmic manifestations of inherited neurodegenerative disorders. Nat Rev Neurol (2014) 0.92
Transgenic mouse model expressing the caspase 6 fragment of mutant huntingtin. J Neurosci (2012) 0.92
Neuroimaging biomarkers of neurodegenerative diseases and dementia. Semin Neurol (2013) 0.92
Abnormal synchrony of resting state networks in premanifest and symptomatic Huntington disease: the IMAGE-HD study. J Psychiatry Neurosci (2014) 0.92
Substantial and reversible brain gray matter reduction but no acute brain lesions in ultramarathon runners: experience from the TransEurope-FootRace Project. BMC Med (2012) 0.92
Attempted and successful compensation in preclinical and early manifest neurodegeneration - a review of task FMRI studies. Front Psychiatry (2014) 0.92
Eye movements in patients with neurodegenerative disorders. Nat Rev Neurol (2013) 0.91
Preserving cortico-striatal function: deep brain stimulation in Huntington's disease. Front Syst Neurosci (2015) 0.91
Frontolimbic neural circuitry at 6 months predicts individual differences in joint attention at 9 months. Dev Sci (2012) 0.90
Multi-modal neuroimaging in premanifest and early Huntington's disease: 18 month longitudinal data from the IMAGE-HD study. PLoS One (2013) 0.89
Basal ganglia atrophy in prodromal Huntington's disease is detectable over one year using automated segmentation. Mov Disord (2011) 0.89
Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/2 mouse model of HD. PLoS One (2013) 0.89
Striatal neuronal loss correlates with clinical motor impairment in Huntington's disease. Mov Disord (2012) 0.89
A two years longitudinal study of a transgenic Huntington disease monkey. BMC Neurosci (2014) 0.89
Voxel-based morphometry with templates and validation in a mouse model of Huntington's disease. Magn Reson Imaging (2013) 0.89
8OHdG is not a biomarker for Huntington disease state or progression. Neurology (2013) 0.88
Neuroprotective effects of psychotropic drugs in Huntington's disease. Int J Mol Sci (2013) 0.88
Convergent pathogenic pathways in Alzheimer's and Huntington's diseases: shared targets for drug development. Nat Rev Drug Discov (2011) 0.87
Reduced functional brain connectivity prior to and after disease onset in Huntington's disease. Neuroimage Clin (2013) 0.87
The Current Status of Neural Grafting in the Treatment of Huntington's Disease. A Review. Front Integr Neurosci (2011) 0.87
Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease. Hum Mol Genet (2015) 0.87
Current Status of Huntington's Disease in Korea: A Nationwide Survey and National Registry Analysis. J Mov Disord (2015) 0.87
An International Survey-based Algorithm for the Pharmacologic Treatment of Chorea in Huntington's Disease. PLoS Curr (2011) 0.86
Striatal atrophy and dendritic alterations in a knock-in mouse model of Huntington's disease. Brain Res Bull (2012) 0.86
Neuroanatomical correlates of cognitive functioning in prodromal Huntington disease. Brain Behav (2013) 0.85
Interregional compensatory mechanisms of motor functioning in progressing preclinical neurodegeneration. Neuroimage (2013) 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
Deficient sustained attention to response task and P300 characteristics in early Huntington's disease. J Neurol (2011) 0.84
Reflexive and volitional saccades: biomarkers of Huntington disease severity and progression. J Neurol Sci (2011) 0.84
Huntington's disease mouse models online: high-resolution MRI images with stereotaxic templates for computational neuroanatomy. PLoS One (2012) 0.83
Cognitive reserve and brain reserve in prodromal Huntington's disease. J Int Neuropsychol Soc (2013) 0.83
Prefrontal cortex white matter tracts in prodromal Huntington disease. Hum Brain Mapp (2015) 0.82
Evaluation of multi-modal, multi-site neuroimaging measures in Huntington's disease: Baseline results from the PADDINGTON study. Neuroimage Clin (2012) 0.82
The Addenbrooke's Cognitive Examination-Revised accurately detects cognitive decline in Huntington's disease. J Neurol (2013) 0.81
Longitudinal Change in Gait and Motor Function in Pre-manifest Huntington's Disease. PLoS Curr (2011) 0.81
Depressive symptoms in prodromal Huntington's Disease correlate with Stroop-interference related functional connectivity in the ventromedial prefrontal cortex. Psychiatry Res (2012) 0.81
Differential loss of thalamostriatal and corticostriatal input to striatal projection neuron types prior to overt motor symptoms in the Q140 knock-in mouse model of Huntington's disease. Front Syst Neurosci (2014) 0.81
Couples' coping in prodromal Huntington disease: a mixed methods study. J Genet Couns (2012) 0.81
HD-PRO-TRIAD™ Validation: A Patient-reported Instrument for the Symptom Triad of Huntington's Disease. Tremor Other Hyperkinet Mov (N Y) (2014) 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
Translational research in Huntington's disease: opening up for disease modifying treatment. Transl Neurodegener (2013) 0.80
Prospects for neuroprotective therapies in prodromal Huntington's disease. Mov Disord (2014) 0.80
Movement sequencing in Huntington disease. World J Biol Psychiatry (2014) 0.80
Characterization of Striatal Neuronal Loss and Atrophy in the R6/2 Mouse Model of Huntington's Disease. PLoS Curr (2014) 0.80
Huntingtin protein is essential for mitochondrial metabolism, bioenergetics and structure in murine embryonic stem cells. Dev Biol (2014) 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
Volumetric analysis of the hypothalamus in Huntington Disease using 3T MRI: the IMAGE-HD Study. PLoS One (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
Differential putaminal morphology in Huntington's disease, frontotemporal dementia and Alzheimer's disease. Aust N Z J Psychiatry (2012) 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
AMPK activation protects from neuronal dysfunction and vulnerability across nematode, cellular and mouse models of Huntington's disease. Hum Mol Genet (2015) 0.79
Elevated arteriolar cerebral blood volume in prodromal Huntington's disease. Mov Disord (2013) 0.79
Spatiotemporal mapping of brain atrophy in mouse models of Huntington's disease using longitudinal in vivo magnetic resonance imaging. Neuroimage (2012) 0.79
Objective acoustic quantification of phonatory dysfunction in Huntington's disease. PLoS One (2013) 0.79
A retrospective analysis of hand tapping as a longitudinal marker of disease progression in Huntington's disease. BMC Neurol (2014) 0.79
Magnetic resonance imaging and spectroscopy: how useful is it for prediction and prognosis? EPMA J (2011) 0.79
Dynamics of the connectome in Huntington's disease: A longitudinal diffusion MRI study. Neuroimage Clin (2015) 0.78
Functional connectivity modeling of consistent cortico-striatal degeneration in Huntington's disease. Neuroimage Clin (2015) 0.78
Assessment of cognitive symptoms in prodromal and early huntington disease. PLoS Curr (2011) 0.78
Is a motor criterion essential for the diagnosis of clinical huntington disease? PLoS Curr (2013) 0.78
Quantitative Susceptibility Mapping Suggests Altered Brain Iron in Premanifest Huntington Disease. AJNR Am J Neuroradiol (2015) 0.78
Depression and clinical progression in spinocerebellar ataxias. Parkinsonism Relat Disord (2015) 0.78
Abnormal cerebellar volume and corticocerebellar dysfunction in early manifest Huntington's disease. J Neurol (2015) 0.78
Neuroimaging in Huntington's disease. World J Radiol (2014) 0.78
Neuropsychiatry and White Matter Microstructure in Huntington's Disease. J Huntingtons Dis (2015) 0.78
Neuroimaging of motor neuron diseases. Ther Adv Neurol Disord (2012) 0.78
Phonatory dysfunction as a preclinical symptom of Huntington disease. PLoS One (2014) 0.78
Music perception and movement deterioration in Huntington's disease. PLoS Curr (2011) 0.77
Understanding the Outcomes Measures used in Huntington Disease Pharmacological Trials: A Systematic Review. J Huntingtons Dis (2014) 0.77
Cross-sectional and longitudinal multimodal structural imaging in prodromal Huntington's disease. Mov Disord (2016) 0.77
Basal ganglia and kinematics modulation: insights from Parkinson's and Huntington's diseases. Parkinsonism Relat Disord (2011) 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
Recent advances in the management of choreas. Ther Adv Neurol Disord (2013) 0.77
Robust MR-based approaches to quantifying white matter structure and structure/function alterations in Huntington's disease. J Neurosci Methods (2015) 0.77
Comparison of cognitive and UHDRS measures in monitoring disease progression in Huntington's disease: a 12-month longitudinal study. Transl Neurodegener (2014) 0.77
Abnormal Electrophysiological Motor Responses in Huntington's Disease: Evidence of Premanifest Compensation. PLoS One (2015) 0.76
Magnetic resonance imaging striatal volumes: a biomarker for clinical trials in Huntington's disease. Mov Disord (2014) 0.76
Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study. PLoS One (2014) 0.76
Unified Huntington's disease rating scale for advanced patients: validation and follow-up study. Mov Disord (2013) 0.76
Progression of premanifest and early Huntington's disease detectable after 1 year—does TRACK-HD open the door to disease-modifying trials in HD and beyond? Mov Disord (2011) 0.75
The diagnosis of mild cognitive impairment due to Alzheimer's disease: recommendations from the National Institute on Aging-Alzheimer's Association workgroups on diagnostic guidelines for Alzheimer's disease. Alzheimers Dement (2011) 21.80
The Alzheimer's Disease Neuroimaging Initiative (ADNI): MRI methods. J Magn Reson Imaging (2008) 14.68
Clinical and biomarker changes in dominantly inherited Alzheimer's disease. N Engl J Med (2012) 14.14
Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain (2011) 9.90
Common variants at ABCA7, MS4A6A/MS4A4E, EPHA1, CD33 and CD2AP are associated with Alzheimer's disease. Nat Genet (2011) 9.23
Revising the definition of Alzheimer's disease: a new lexicon. Lancet Neurol (2010) 8.74
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
Two phase 3 trials of bapineuzumab in mild-to-moderate Alzheimer's disease. N Engl J Med (2014) 6.50
Neuroimaging standards for research into small vessel disease and its contribution to ageing and neurodegeneration. Lancet Neurol (2013) 6.18
Functional role of the supplementary and pre-supplementary motor areas. Nat Rev Neurosci (2008) 5.20
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
11C-PiB PET assessment of change in fibrillar amyloid-beta load in patients with Alzheimer's disease treated with bapineuzumab: a phase 2, double-blind, placebo-controlled, ascending-dose study. Lancet Neurol (2010) 5.03
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat Genet (2003) 4.76
The clinical use of structural MRI in Alzheimer disease. Nat Rev Neurol (2010) 4.59
Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell (2006) 4.53
Tracking atrophy progression in familial Alzheimer's disease: a serial MRI study. Lancet Neurol (2006) 4.19
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat Neurosci (2002) 4.15
Mapping the evolution of regional atrophy in Alzheimer's disease: unbiased analysis of fluid-registered serial MRI. Proc Natl Acad Sci U S A (2002) 4.07
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum Mol Genet (2003) 4.07
Longitudinal stability of MRI for mapping brain change using tensor-based morphometry. Neuroimage (2006) 3.85
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
Preparing for preventive clinical trials: the Predict-HD study. Arch Neurol (2006) 3.57
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. Lancet Neurol (2013) 3.56
A longitudinal study of brain volume changes in normal aging using serial registered magnetic resonance imaging. Arch Neurol (2003) 3.44
Computer-assisted imaging to assess brain structure in healthy and diseased brains. Lancet Neurol (2003) 3.26
Effect of rivastigmine on delay to diagnosis of Alzheimer's disease from mild cognitive impairment: the InDDEx study. Lancet Neurol (2007) 3.22
Fast free-form deformation using graphics processing units. Comput Methods Programs Biomed (2009) 3.18
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
Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions. Mol Cell (2002) 2.98
Update on the magnetic resonance imaging core of the Alzheimer's disease neuroimaging initiative. Alzheimers Dement (2010) 2.88
A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease. J Exp Med (2008) 2.78
Human medial frontal cortex mediates unconscious inhibition of voluntary action. Neuron (2007) 2.75
Amyloid-related imaging abnormalities in patients with Alzheimer's disease treated with bapineuzumab: a retrospective analysis. Lancet Neurol (2012) 2.75
Psychiatric symptoms in Huntington's disease before diagnosis: the predict-HD study. Biol Psychiatry (2007) 2.71
Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features. Brain (2012) 2.65
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
Autosomal-dominant Alzheimer's disease: a review and proposal for the prevention of Alzheimer's disease. Alzheimers Res Ther (2011) 2.51
Recruitment and activation of caspase-8 by the Huntingtin-interacting protein Hip-1 and a novel partner Hippi. Nat Cell Biol (2002) 2.50
Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review. J Neurol Neurosurg Psychiatry (2010) 2.44
Posterior cortical atrophy. Lancet Neurol (2012) 2.41
3D characterization of brain atrophy in Alzheimer's disease and mild cognitive impairment using tensor-based morphometry. Neuroimage (2008) 2.40
Volition and conflict in human medial frontal cortex. Curr Biol (2005) 2.38
Adult polyglucosan body disease: Natural History and Key Magnetic Resonance Imaging Findings. Ann Neurol (2012) 2.33
Automated cross-sectional and longitudinal hippocampal volume measurement in mild cognitive impairment and Alzheimer's disease. Neuroimage (2010) 2.32
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
Early-onset versus late-onset Alzheimer's disease: the case of the missing APOE ɛ4 allele. Lancet Neurol (2010) 2.18
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
Increased brain atrophy rates in cognitively normal older adults with low cerebrospinal fluid Aβ1-42. Ann Neurol (2010) 2.15
Neurocognitive signs in prodromal Huntington disease. Neuropsychology (2011) 2.14
A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series. Brain (2008) 2.13
Clinical and neuroanatomical signatures of tissue pathology in frontotemporal lobar degeneration. Brain (2011) 2.13
fMRI biomarker of early neuronal dysfunction in presymptomatic Huntington's Disease. AJNR Am J Neuroradiol (2004) 2.10
Large C9orf72 hexanucleotide repeat expansions are seen in multiple neurodegenerative syndromes and are more frequent than expected in the UK population. Am J Hum Genet (2013) 2.09