Differences in duration of Huntington’s disease based on age at onset

No abstract is available for this paper.

• Publication year

  1999

• Venue

  Journal of Neurology Neurosurgery & Psychiatry

• Publication date

  1999-01-01

• Fields of study

  Medicine

• Identifiers
  DOI 10.1136/jnnp.66.1.52PMID 9886451PMCID PMC1736160
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.

  1997cited by this paper
• Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo.

  1997cited by this paper
• Longitudinal change in basal ganglia volume in patients with Huntington's disease

  1997cited by this paper
• HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain

  1997cited by this paper
• Trinucleotide repeat length and clinical progression in Huntington's disease

  1996cited by this paper
• Relationship between trinucliotide repeats and neuropathological changes in Huntington's diease

  1996cited by this paper
• A huntingtin-associated protein enriched in brain with implications for pathology

  1995cited by this paper
• Functional decline in Huntington's disease

  1995cited by this paper
• Expression of the Huntington's disease (IT15) protein product in HD patients.

  1995cited by this paper
• Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

  1995cited by this paper
• Trinucleotide repeat length and progression of illness in Huntington's disease.

  1994cited by this paper
• Trinucleotide repeat length and rate of progression of Huntington's disease

  1994cited by this paper
• A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group.

  1993cited by this paper
• Duration of illness in Huntington's disease is not related to age at onset.

  1993cited by this paper
• Trinucleotide repeat length instability and age of onset in Huntington's disease

  1993cited by this paper
• Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease

  1993cited by this paper
• Factors associated with slow progression in Huntington's disease.

  1991cited by this paper
• Huntington's disease : a disorder of families

  1989cited by this paper
• Clinical and neuropathologic assessment of severity in Huntington's disease

  1988cited by this paper
• Factors related to onset age of Huntington disease.

  1982cited by this paper
• Progression rate and age at onset are related in autosomal dominant neurologic diseases

  1982cited by this paper
• Factors influencing age at onset and duration of survival in Huntington's chorea

  1981cited by this paper
• International Classification of Diseases--9th revision.

  1978cited by this paper
• Juvenile Huntington's chorea

  1968cited by this paper

• Efficacy of aripiprazole in pediatric-onset Huntington’s disease: a case report and literature review

  2025cites this paper
• Safety and Efficacy of Deutetrabenazine at High versus Lower Daily Dosages in the ARC-HD Study to Treat Chorea in Huntington Disease

  2025cites this paper
• Large animal models for Huntington’s disease research

  2024cites this paper
• Performance Comparison Between Deep Neural Network and Machine Learning Based Classifiers for Huntington Disease Prediction From Human DNA Sequence

  2024cites this paper
• Sex-Specific Differences in the Progression of Huntington’s Disease Symptoms – A National Study in China

  2024cites this paper
• A Practical Guide for Clinical Approach to Patients With Huntington’s Disease in Korea

  2024cites this paper
• Modifiable factors associated with Huntington's disease progression in presymptomatic participants

  2024cites this paper
• Targeting N-Methyl-d-Aspartate Receptors in Neurodegenerative Diseases

  2024cites this paper
• Novel drug delivery to the brain for neurodegenerative disorder treatment using carbon nanotubes

  2024cites this paper
• Gene-dosage- and sex-dependent differences in the prodromal-Like phase of the F344tgHD rat model for Huntington disease

  2024cites this paper
• Sex contribution to average age at onset of Huntington’s disease depends on the number of (CAG)n repeats

  2024cites this paper
• Huntington’s Disease-Related Mortality Patterns: A Two-Decade Analysis of Mortality Trends in the United States, from 1999–2019

  2024cites this paper
• Sex differences in sleep architecture in a mouse model of Huntington’s disease

  2024cites this paper
• Racial Differences in the Presentation and Progression of Huntington's Disease

  2023cites this paper
• Sex Differences in Sleep Phenotypes in the BACHD Mouse Model of Huntington’s Disease

  2023cites this paper
• Clinical Characterisation of Dystonia in Adult Patients with Huntington’s Disease

  2023cites this paper
• Healthcare resource utilization and cost among individuals with late-onset versus adult-onset Huntington’s disease: a claims‑based retrospective cohort study

  2023cites this paper
• Comparison of Huntington’s disease phenotype progression in male and female heterozygous FDNQ175 mice

  2023cites this paper
• Exploring Huntington’s Disease Diagnosis via Artificial Intelligence Models: A Comprehensive Review

  2023cites this paper
• From Pathogenesis to Therapeutics: A Review of 150 Years of Huntington’s Disease Research

  2023cites this paper
• Graphomotor Dysfluency as a Predictor of Disease Progression in Premanifest Huntington’s Disease

  2023cites this paper
• 22 years of Predictive Testing: The Experience of the UK Huntington’s Prediction Consortium.

  2022cites this paper
• Risk of mortality from suicide in patients with Huntington’s disease is increased compared to the general population in England

  2022cites this paper
• Comorbidities and clinical outcomes in adult- and juvenile-onset Huntington’s disease: a study of linked Swedish National Registries (2002–2019)

  2022cites this paper
• Functional Intercellular Transmission of miHTT via Extracellular Vesicles: An In Vitro Proof-of-Mechanism Study

  2022cites this paper
• Clinical and genetic characteristics of late‐onset Huntington's disease in a large European cohort

  2022cites this paper
• Second Sphere Interactions in Amyloidogenic Diseases.

  2022cites this paper
• Natural history and burden of Huntington's disease in the UK: A population‐based cohort study

  2022cites this paper
• Uncovering the universality of self-replication in protein aggregation and its link to disease

  2022cites this paper
• Association between sex and Huntington’s disease: an updated review on symptomatology and prognosis of neurodegenerative disorders

  2022cites this paper
• Natural history of Huntington’s disease: evolution of modeling onset

  2022cites this paper
• The association between educational attainment and SCA 3 age of onset and disease course.

  2022cites this paper
• Searching for Answers: Information-Seeking by Young People At-Risk for Huntington’s Disease

  2022cites this paper
• The Safety of Deutetrabenazine for Chorea in Huntington Disease: An Open-Label Extension Study

  2022cites this paper
• Krankheitsmodifizierende Therapieansätze bei der Huntington-Krankheit

  2021cites this paper
• Sex Differences in Huntington's Disease: Evaluating the Enroll‐HD Database

  2021cites this paper
• Genetic epidemiological characteristics of a Hungarian subpopulation of patients with Huntington’s disease

  2021cites this paper
• High Frequency of Concomitant Bladder, Bowel, and Sexual Symptoms in Huntington’s Disease: A Self-Reported Questionnaire Study

  2021cites this paper
• Huntington’s Disease in Israel: A Population-Based Study Using 20 Years of Routinely-Collected Healthcare Data

  2021influential citation
• Sex differences in movement disorders

  2020cites this paper
• Huntington’s Disease

  2020cites this paper
• Index

  2020cites this paper
• VMAT2 inhibitors for the treatment of hyperkinetic movement disorders.

  2020cites this paper
• Preface

  2020cites this paper
• Answers to Exercises

  2020cites this paper
• Glossary

  2020cites this paper
• Bibliography

  2020cites this paper
• Test Protocol

  2020cites this paper
• Alzheimer’s Dementia

  2020cites this paper
• Vascular Dementia

  2020cites this paper
• Parkinson’s Disease Dementia

  2020cites this paper
• Nanoengineering of stem cells for neural regenerative medicine

  2020cites this paper
• Age of onset and behavioral manifestations in Huntington's disease: An Enroll‐HD cohort analysis

  2020cites this paper
• Test Material

  2020cites this paper
• Language in Dementia

  2020cites this paper
• Neuropsychology of Movement Disorders and Motor Neuron Disease: Parkinson’s Disease, Progressive Supranuclear Palsy, Essential Tremor, Huntington’s Disease, and Amyotrophic Lateral Sclerosis

  2019cites this paper
• Stress and Glucocorticoids as Experience-Dependent Modulators of Huntington's Disease

  2019cites this paper
• Chapter 2 Huntington ’ s Disease and Cell Therapies : Past , Present , and Future

  2019cites this paper
• Sleep in Huntington's disease: A systematic review and meta-analysis of polysomongraphic findings.

  2019cites this paper
• Deterioration of neuroregenerative plasticity in association with testicular atrophy and dysregulation of the hypothalamic-pituitary-gonadal (HPG) axis in Huntington's disease: A putative role of the Huntingtin gene in steroidogenesis.

  2019cites this paper
• Depression in neurodegenerative diseases: Common mechanisms and current treatment options.

  2019cites this paper
• Sex-Specific Differences in Redox Homeostasis in Brain Norm and Disease

  2019cites this paper
• The psychopharmacology of Huntington disease.

  2019cites this paper
• Late-onset Huntington’s disease with 40–42 CAG expansion

  2019cites this paper
• Life expectancy and mortality in chorea-acanthocytosis and McLeod syndrome.

  2019cites this paper
• Frecuencia De Casos Juveniles Con Enfermedad De Huntington En Población Mexicana

  2019cites this paper
• Pathology, Prevention and Therapeutics of Neurodegenerative Disease

  2019cites this paper
• A probabilistic disease progression modeling approach and its application to integrated Huntington’s disease observational data

  2019cites this paper
• Robust Preimplantation Genetic Testing of Huntington Disease by Combined Triplet-Primed PCR Analysis of the HTT CAG Repeat and Multi-Microsatellite Haplotyping

  2019cites this paper
• Identification of common coexpression modules based on quantitative network comparison

  2018cites this paper
• Epidemiology of Huntington disease in Cyprus: A 20‐year retrospective study

  2018cites this paper
• Altered energy metabolism and nucleating aggregates found in normal cells as a consequence of the cell-to-cell transfer of the pathogenic polyglutamine aggregate from HD diseased cells

  2018cites this paper
• Tetrabenazine Treatment Patterns and Outcomes for Chorea Associated with Huntington Disease: A Retrospective Chart Review

  2018cites this paper
• The development, validation and demonstration of an automated rodent tracker and whisker detector

  2018cites this paper
• Clinical Symptomatology of Huntington’s Disease

  2018cites this paper
• Large-scale transcriptomic analysis reveals that pridopidine reverses aberrant gene expression and activates neuroprotective pathways in the YAC128 HD mouse

  2018cites this paper
• hPSC-Derived Striatal Cells Generated Using a Scalable 3D Hydrogel Promote Recovery in a Huntington Disease Mouse Model

  2018influential citation
• Retrospective Analysis of Healthcare Resource Use, Treatment Patterns, and Treatment-related Events in Patients with Huntington’s Disease–associated Chorea Initiated on Tetrabenazine

  2018cites this paper
• Therapeutic effects of stem cells in rodent models of Huntington's disease: Review and electrophysiological findings

  2018cites this paper
• Understanding How Chorea Affects Health-Related Quality of Life in Huntington Disease: An Online Survey of Patients and Caregivers in the United States

  2018cites this paper
• Pharmacological disruption of the MID 1 / 4 interaction reduces mutant Huntingtin levels in primary neuronal cultures

  2018cites this paper
• The Lifetime Economic Burden of Monogenic Diseases And The Social Motivations For Their Treatment With Genetic Therapy

  2018cites this paper
• Semiparametric Statistical Estimation and Inference with Latent Information

  2018influential citation
• Pharmacological disruption of the MID1/α4 interaction reduces mutant Huntingtin levels in primary neuronal cultures.

  2018cites this paper
• Stem cell transplantation for Huntington's diseases.

  2017cites this paper
• Clinical characterization of dystonia in adult patients with Huntington's disease

  2017cites this paper
• Enhanced retinal responses in Huntington’s disease patients

  2017cites this paper
• The role of dopamine in positive and negative prediction error utilization during incidental learning - Insights from Positron Emission Tomography, Parkinson's disease and Huntington's disease.

  2017cites this paper
• The sigma-1 receptor mediates the beneficial effects of pridopidine in a mouse model of Huntington disease.

  2017cites this paper
• Wake and Sleep EEG in Patients With Huntington Disease

  2017cites this paper
• Juvenile Huntington’s Disease: Diagnostic and Treatment Considerations for the Psychiatrist

  2017cites this paper
• Total Word count : 3470 Running Title : Characterisation of Dystonia in Huntington ’ s Disease

  2017cites this paper
• Nonparametric distribution estimation in the presence of familial correlation and censoring

  2017cites this paper
• Survival, Mortality, Causes and Places of Death in a European Huntington's Disease Prospective Cohort

  2017cites this paper
• What do we know about Late Onset Huntington’s Disease?

  2017cites this paper
• Risk factors for the onset and progression of Huntington disease

  2017influential citation
• Predicting disease Risk by Transformation Models in the Presence of Unspecified Subgroup Membership.

  2017influential citation
• Comparison of mid-age-onset and late-onset Huntington’s disease in Finnish patients

  2017cites this paper
• Diagnostic Evaluation of Huntington's Disease Within the Frame of Bioinformatics.

  2017cites this paper
• The Relationships Among Emotion, Cognitive Dysfunction and Anosognosia in Huntington’s Disease

  2017cites this paper