Published in J Neuropsychiatry Clin Neurosci on January 01, 2011
A Tale of Two Maladies? Pathogenesis of Depression with and without the Huntington's Disease Gene Mutation. Front Neurol (2013) 0.83
Understanding the Outcomes Measures used in Huntington Disease Pharmacological Trials: A Systematic Review. J Huntingtons Dis (2014) 0.77
Pattern of mother-child feeding interactions in preterm and term dyads at 18 and 24 months. Front Psychol (2015) 0.75
Assessing depression in obese women: an examination of two commonly-used measures. J Psychosom Res (2013) 0.75
Mutations in the DJ-1 gene associated with autosomal recessive early-onset parkinsonism. Science (2002) 9.90
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
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
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
Mutations in a gene encoding a novel protein containing a phosphotyrosine-binding domain cause type 2 cerebral cavernous malformations. Am J Hum Genet (2003) 2.58
Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients. Curr Biol (2009) 2.19
Mutations in SLC20A2 are a major cause of familial idiopathic basal ganglia calcification. Neurogenetics (2013) 2.11
Brain white-matter volume loss and glucose hypometabolism precede the clinical symptoms of Huntington's disease. J Nucl Med (2006) 1.83
Family communication about genetic risk: the little that is known. Community Genet (2004) 1.79
DJ-1 transcriptionally up-regulates the human tyrosine hydroxylase by inhibiting the sumoylation of pyrimidine tract-binding protein-associated splicing factor. J Biol Chem (2006) 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
The DJ-1L166P mutant protein associated with early onset Parkinson's disease is unstable and forms higher-order protein complexes. Hum Mol Genet (2003) 1.49
Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease. Mov Disord (2005) 1.33
Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study. BMC Med Genet (2006) 1.30
Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course. Brain (2003) 1.26
Low frequency of PDCD10 mutations in a panel of CCM3 probands: potential for a fourth CCM locus. Hum Mutat (2006) 1.24
Deletions in CCM2 are a common cause of cerebral cavernous malformations. Am J Hum Genet (2006) 1.19
Genetic professionals' reports of nondisclosure of genetic risk information within families. Eur J Hum Genet (2005) 1.18
Aberrant connectivity of lateral prefrontal networks in presymptomatic Huntington's disease. Exp Neurol (2008) 1.17
Common SNP-based haplotype analysis of the 4p16.3 Huntington disease gene region. Am J Hum Genet (2012) 1.05
Behavioural problems in Huntington's disease using the Problem Behaviours Assessment. Gen Hosp Psychiatry (2008) 1.05
Immunohistochemical analysis of KCNQ3 potassium channels in mouse brain. Neurosci Lett (2006) 1.03
18F-FDG PET uptake in the pre-Huntington disease caudate affects the time-to-onset independently of CAG expansion size. Eur J Nucl Med Mol Imaging (2012) 1.03
The gender effect in juvenile Huntington disease patients of Italian origin. Am J Med Genet B Neuropsychiatr Genet (2004) 0.99
Immunohistochemical analysis of KCNQ2 potassium channels in adult and developing mouse brain. Brain Res (2006) 0.98
Genotype-dependent priming to self- and xeno-cannibalism in heterozygous and homozygous lymphoblasts from patients with Huntington's disease. J Neurochem (2006) 0.97
FTY720 (fingolimod) is a neuroprotective and disease-modifying agent in cellular and mouse models of Huntington disease. Hum Mol Genet (2013) 0.95
Association between caffeine intake and age at onset in Huntington's disease. Neurobiol Dis (2013) 0.94
Different spectra of genomic deletions within the CCM genes between Italian and American CCM patient cohorts. Neurogenetics (2007) 0.94
How young people find out about their family history of Huntington's disease. Soc Sci Med (2009) 0.94
The influence of gender on phenotype and disease progression in patients with Huntington's disease. Parkinsonism Relat Disord (2012) 0.94
Novel parkin mutations detected in patients with early-onset Parkinson's disease. Mov Disord (2005) 0.94
Impaired regulation of brain mitochondria by extramitochondrial Ca2+ in transgenic Huntington disease rats. J Biol Chem (2008) 0.93
Early defect of transforming growth factor β1 formation in Huntington's disease. J Cell Mol Med (2011) 0.93
Seeking Huntington disease biomarkers by multimodal, cross-sectional basal ganglia imaging. Hum Brain Mapp (2012) 0.93
Localization of autosomal recessive early-onset parkinsonism to chromosome 1p36 (PARK7) in an independent dataset. Ann Neurol (2002) 0.90
One-year safety and tolerability profile of pridopidine in patients with Huntington disease. Neurology (2013) 0.89
Aberrant A2A receptor function in peripheral blood cells in Huntington's disease. FASEB J (2003) 0.89
Validation of the first quality-of-life measurement for patients with Huntington's disease: the Huntington Quality of Life Instrument. Int Clin Psychopharmacol (2012) 0.89
Truncated peroxisome proliferator-activated receptor-γ coactivator 1α splice variant is severely altered in Huntington's disease. Neurodegener Dis (2011) 0.88
Seeking brain biomarkers for preventive therapy in Huntington disease. CNS Neurosci Ther (2010) 0.88
A Europe-wide assessment of current medication choices in Huntington's disease. Mov Disord (2008) 0.88
Structural MRI in Huntington's disease and recommendations for its potential use in clinical trials. Neurosci Biobehav Rev (2013) 0.88
Tractography of the corpus callosum in Huntington's disease. PLoS One (2013) 0.86
Assessment of depression, anxiety and apathy in prodromal and early huntington disease. PLoS Curr (2011) 0.86
Mycophenolate mofetil embryopathy may be dose and timing dependent. Am J Med Genet A (2008) 0.85
The platelet maximum number of A2A-receptor binding sites (Bmax) linearly correlates with age at onset and CAG repeat expansion in Huntington's disease patients with predominant chorea. Neurosci Lett (2005) 0.85
Highly variable penetrance in subjects affected with cavernous cerebral angiomas (CCM) carrying novel CCM1 and CCM2 mutations. Am J Med Genet B Neuropsychiatr Genet (2007) 0.85
How do partners find out about the risk of Huntington's disease in couple relationships? J Genet Couns (2013) 0.84
Caudate nucleus and insular activation during a pain suppression paradigm comparing thermal and electrical stimulation. Open Neuroimag J (2011) 0.84
Caudate glucose hypometabolism in a subject carrying an unstable allele of intermediate CAG(33) repeat length in the Huntington's disease gene. Mov Disord (2011) 0.84
Factor analysis of behavioural symptoms in Huntington's disease. J Neurol Neurosurg Psychiatry (2010) 0.84
Aripiprazole in the treatment of Huntington's disease: a case series. Neuropsychiatr Dis Treat (2009) 0.84
Reduced activity of cortico-striatal fibres in the R6/2 mouse model of Huntington's disease. Neuroreport (2007) 0.83
Population stratification may bias analysis of PGC-1α as a modifier of age at Huntington disease motor onset. Hum Genet (2012) 0.83
Current Pharmacological Management in Juvenile Huntington's Disease. PLoS Curr (2012) 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
An International Survey-based Algorithm for the Pharmacologic Treatment of Irritability in Huntington's Disease. PLoS Curr (2011) 0.81
The role of iron in gray matter degeneration in Huntington's disease: a magnetic resonance imaging study. Hum Brain Mapp (2014) 0.81
Reliability and factor structure of the Short Problem Behaviors Assessment for Huntington's disease (PBA-s) in the TRACK-HD and REGISTRY studies. J Neuropsychiatry Clin Neurosci (2015) 0.81
TAA repeat variation in the GRIK2 gene does not influence age at onset in Huntington's disease. Biochem Biophys Res Commun (2012) 0.81
An International Survey-based Algorithm for the Pharmacologic Treatment of Obsessive-Compulsive Behaviors in Huntington's Disease. PLoS Curr (2011) 0.81
Huntington disease in subjects from an Israeli Karaite community carrying alleles of intermediate and expanded CAG repeats in the HTT gene: Huntington disease or phenocopy? J Neurol Sci (2008) 0.80
DNA instability in replicating Huntington's disease lymphoblasts. BMC Med Genet (2009) 0.80
New Huntington disease mutation arising from a paternal CAG34 allele showing somatic length variation in serially passaged lymphoblasts. Am J Med Genet B Neuropsychiatr Genet (2005) 0.80
Huntingtin fragmentation and increased caspase 3, 8 and 9 activities in lymphoblasts with heterozygous and homozygous Huntington's disease mutation. Mech Ageing Dev (2005) 0.80
Novel T719P AbetaPP mutation unbalances the relative proportion of amyloid-beta peptides. J Alzheimers Dis (2009) 0.80
What is the impact of education on Huntington's disease? Mov Disord (2011) 0.79
Rate of change in early Huntington's disease: a clinicometric analysis. Mov Disord (2011) 0.79
Cocaine- and amphetamine-regulated transcript is increased in Huntington disease. Mov Disord (2007) 0.79
Further evidence of reliability and validity of the Huntington's disease quality of life battery for carers: Italian and French translations. Qual Life Res (2012) 0.79
PET translates neurophysiology into images: A review to stimulate a network between neuroimaging and basic research. J Cell Physiol (2011) 0.78
The personal experience of parenting a child with juvenile Huntington's disease: perceptions across Europe. Eur J Hum Genet (2013) 0.77
Graded cutaneous electrical vs thermal stimulation in humans shows different insular and cingulate cortex activation. Somatosens Mot Res (2010) 0.77
Discrepancies in reporting the CAG repeat lengths for Huntington's disease. Eur J Hum Genet (2011) 0.77
Neuroprotective effects of riluzole in Huntington's disease. Eur J Nucl Med Mol Imaging (2007) 0.77
Candidate glutamatergic and dopaminergic pathway gene variants do not influence Huntington's disease motor onset. Neurogenetics (2013) 0.77
Incidence, course, and predictors of apathy in Huntington's disease: a two-year prospective study. J Neuropsychiatry Clin Neurosci (2011) 0.77
Huntington's disease: new aspects on phenotype and genotype. Parkinsonism Relat Disord (2012) 0.77
Cerebellar abnormalities in Huntington's disease: a role in motor and psychiatric impairment? Mov Disord (2014) 0.76
Neurodegenerative disease: 'fifty shades of grey' in the Huntington disease gene. Nat Rev Neurol (2013) 0.76
Differential pattern of brain-specific CSF proteins tau and amyloid-β in Parkinsonian syndromes. Mov Disord (2010) 0.76
Suicidal ideation in a European Huntington's disease population. J Affect Disord (2013) 0.75
De novo seven extra repeat expanded mutation in the PRNP gene in an Italian patient with early onset dementia. BMJ Case Rep (2009) 0.75
Irritability in Huntington's disease. Psychiatry Res (2012) 0.75
Key role of nuclear medicine in seeking biomarkers of Huntington's disease. Eur J Nucl Med Mol Imaging (2010) 0.75
New challenges for gamete donation programmes: changes in guidelines are needed. Hum Fertil (Camb) (2002) 0.75