|
1
|
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
|
|
2
|
Faster perceptual learning through excitotoxic neurodegeneration.
|
Curr Biol
|
2012
|
1.46
|
|
3
|
NR2A and NR2B receptor gene variations modify age at onset in Huntington disease.
|
Neurogenetics
|
2004
|
1.31
|
|
4
|
PGC-1alpha as modifier of onset age in Huntington disease.
|
Mol Neurodegener
|
2009
|
1.22
|
|
5
|
Mitochondrial haplogroup H correlates with ATP levels and age at onset in Huntington disease.
|
J Mol Med (Berl)
|
2010
|
1.13
|
|
6
|
NR2A and NR2B receptor gene variations modify age at onset in Huntington disease in a sex-specific manner.
|
Hum Genet
|
2007
|
1.13
|
|
7
|
Efficacy of fumaric acid esters in the R6/2 and YAC128 models of Huntington's disease.
|
PLoS One
|
2011
|
1.10
|
|
8
|
The role of the immune system in Huntington's disease.
|
Clin Dev Immunol
|
2013
|
1.03
|
|
9
|
What is influencing the phenotype of the common homozygous polymerase-γ mutation p.Ala467Thr?
|
Brain
|
2012
|
1.01
|
|
10
|
Response inhibition subprocesses and dopaminergic pathways: basal ganglia disease effects.
|
Neuropsychologia
|
2009
|
1.01
|
|
11
|
ASK1 and MAP2K6 as modifiers of age at onset in Huntington's disease.
|
J Mol Med (Berl)
|
2008
|
1.01
|
|
12
|
Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation.
|
Mech Ageing Dev
|
2005
|
1.00
|
|
13
|
Huntington's disease as caused by 34 CAG repeats.
|
Mov Disord
|
2008
|
0.99
|
|
14
|
Mechanisms mediating parallel action monitoring in fronto-striatal circuits.
|
Neuroimage
|
2012
|
0.95
|
|
15
|
Chorein-sensitive polymerization of cortical actin and suicidal cell death in chorea-acanthocytosis.
|
FASEB J
|
2012
|
0.93
|
|
16
|
Levels of error processing in Huntington's disease: a combined study using event-related potentials and voxel-based morphometry.
|
Hum Brain Mapp
|
2008
|
0.93
|
|
17
|
Time processing in Huntington's disease: a group-control study.
|
PLoS One
|
2007
|
0.93
|
|
18
|
Error processing in Huntington's disease.
|
PLoS One
|
2006
|
0.92
|
|
19
|
Magnetic resonance perfusion imaging of resting-state cerebral blood flow in preclinical Huntington's disease.
|
J Cereb Blood Flow Metab
|
2011
|
0.92
|
|
20
|
Functional compensation or pathology in cortico-subcortical interactions in preclinical Huntington's disease?
|
Neuropsychologia
|
2007
|
0.91
|
|
21
|
Assessment of simple movements and progression of Huntington's disease.
|
J Neurol Neurosurg Psychiatry
|
2007
|
0.91
|
|
22
|
Age at onset in Huntington's disease: replication study on the associations of ADORA2A, HAP1 and OGG1.
|
Neurogenetics
|
2010
|
0.90
|
|
23
|
On the role of fronto-striatal neural synchronization processes for response inhibition--evidence from ERP phase-synchronization analyses in pre-manifest Huntington's disease gene mutation carriers.
|
Neuropsychologia
|
2011
|
0.90
|
|
24
|
Response inhibition in Huntington's disease-a study using ERPs and sLORETA.
|
Neuropsychologia
|
2007
|
0.90
|
|
25
|
Default-mode network changes in preclinical Huntington's disease.
|
Exp Neurol
|
2012
|
0.88
|
|
26
|
Action selection in a possible model of striatal medium spiny neuron dysfunction: behavioral and EEG data in a patient with benign hereditary chorea.
|
Brain Struct Funct
|
2013
|
0.88
|
|
27
|
Brain activation and functional connectivity in premanifest Huntington's disease during states of intrinsic and phasic alertness.
|
Hum Brain Mapp
|
2011
|
0.88
|
|
28
|
NMDA receptor gene variations as modifiers in Huntington disease: a replication study.
|
PLoS Curr
|
2011
|
0.87
|
|
29
|
Mutations in TITF1 are not relevant to sporadic and familial chorea of unknown cause.
|
Mov Disord
|
2006
|
0.87
|
|
30
|
Altered ventral striatal activation during reward and punishment processing in premanifest Huntington's disease: a functional magnetic resonance study.
|
Exp Neurol
|
2012
|
0.87
|
|
31
|
Impaired motor speech performance in Huntington's disease.
|
J Neural Transm (Vienna)
|
2013
|
0.87
|
|
32
|
Upper gastrointestinal findings in Huntington's disease: patients suffer but do not complain.
|
J Neural Transm (Vienna)
|
2009
|
0.86
|
|
33
|
Increased cognitive functioning in symptomatic Huntington's disease as revealed by behavioral and event-related potential indices of auditory sensory memory and attention.
|
J Neurosci
|
2008
|
0.86
|
|
34
|
Hepatic mitochondrial dysfunction in manifest and premanifest Huntington disease.
|
Neurology
|
2013
|
0.86
|
|
35
|
Behavioral and neurophysiological evidence for the enhancement of cognitive control under dorsal pallidal deep brain stimulation in Huntington's disease.
|
Brain Struct Funct
|
2014
|
0.85
|
|
36
|
Depression in patients with Huntington disease correlates with alterations of the brain stem raphe depicted by transcranial sonography.
|
J Psychiatry Neurosci
|
2011
|
0.85
|
|
37
|
PGC-1alpha downstream transcription factors NRF-1 and TFAM are genetic modifiers of Huntington disease.
|
Mol Neurodegener
|
2011
|
0.84
|
|
38
|
Complex movement behaviour and progression of Huntington's disease.
|
Neurosci Lett
|
2007
|
0.84
|
|
39
|
Teaching Video NeuroImages: spinal and bulbar muscular atrophy mimicking essential tremor.
|
Neurology
|
2012
|
0.84
|
|
40
|
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
|
|
41
|
Stimulus-response compatibility in Huntington's disease: a cognitive-neurophysiological analysis.
|
J Neurophysiol
|
2008
|
0.82
|
|
42
|
Better global and cognitive functioning in choreatic versus hypokinetic-rigid Huntington's disease.
|
Mov Disord
|
2013
|
0.82
|
|
43
|
"Theory of mind" is impaired in Huntington's disease.
|
Mov Disord
|
2011
|
0.82
|
|
44
|
No association between polymorphisms in the BDNF gene and age at onset in Huntington disease.
|
BMC Med Genet
|
2006
|
0.82
|
|
45
|
Progressive hepatic mitochondrial dysfunction in premanifest Huntington's disease.
|
Mov Disord
|
2014
|
0.81
|
|
46
|
Is the retina affected in Huntington disease?
|
Acta Neuropathol
|
2005
|
0.80
|
|
47
|
Alterations in voluntary movement execution in Huntington's disease are related to the dominant motor system: evidence from event-related potentials.
|
Exp Neurol
|
2008
|
0.80
|
|
48
|
A novel cognitive-neurophysiological state biomarker in premanifest Huntington's disease validated on longitudinal data.
|
Sci Rep
|
2013
|
0.80
|
|
49
|
A randomized, placebo-controlled trial of AFQ056 for the treatment of chorea in Huntington's disease.
|
Mov Disord
|
2015
|
0.80
|
|
50
|
Hepatic mitochondrial dysfunction in Friedreich ataxia.
|
BMC Neurol
|
2011
|
0.78
|
|
51
|
Cross sectional PET study of cerebral adenosine A₁ receptors in premanifest and manifest Huntington's disease.
|
Eur J Nucl Med Mol Imaging
|
2014
|
0.78
|
|
52
|
Failure to confirm influence of methyltetrahydrofolate reductase (MTHFR) polymorphisms on age at onset of Huntington disease.
|
J Negat Results Biomed
|
2005
|
0.77
|
|
53
|
Age at onset of Huntington disease is not modulated by the R72P variation in TP53 and the R196K variation in the gene coding for the human caspase activated DNase (hCAD).
|
BMC Med Genet
|
2005
|
0.77
|
|
54
|
Music perception and movement deterioration in Huntington's disease.
|
PLoS Curr
|
2011
|
0.77
|
|
55
|
Evidence for divergent effects of neurodegeneration in Huntington's disease on attentional selection and neural plasticity: implications for excitotoxicity.
|
Brain Struct Funct
|
2014
|
0.77
|
|
56
|
Association of age at onset in Huntington disease with functional promoter variations in NPY and NPY2R.
|
J Mol Med (Berl)
|
2014
|
0.76
|
|
57
|
CNR1 variation is associated with the age at onset in Huntington disease.
|
Eur J Med Genet
|
2013
|
0.76
|
|
58
|
Glutathione S-Transferase Omega 1 variation does not influence age at onset of Huntington's disease.
|
BMC Med Genet
|
2004
|
0.76
|
|
59
|
Functional and structural MRI biomarkers to detect pre-clinical neurodegeneration.
|
Curr Alzheimer Res
|
2013
|
0.76
|
|
60
|
Oral and dental health in Huntington's disease - an observational study.
|
BMC Neurol
|
2013
|
0.75
|
|
61
|
Hyperhomocysteinaemia in treated patients with Huntington's disease homocysteine in HD.
|
Mov Disord
|
2004
|
0.75
|