ReviewJournal of Huntington's disease2019
The Role of Hypothalamic Pathology for Non-Motor Features of Huntington's Disease.
Review in Journal of Huntington's disease, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 37 citations in OpenAlex.
- The Evolving Landscape of Stem Cell Therapies for Huntington's Disease.Molecular diagnosis & therapy · 2026Review
- TDP-43 overexpression in the hypothalamus drives neuropathology, dysregulates metabolism and impairs behavior in mice.Acta neuropathologica communications · 2025Article
- Early Diagnosis of Huntington Disease: Insights from Magnetic Resonance Spectroscopy-A Systematic Review.Journal of clinical medicine · 2024Review
- Structural Plasticity of the Hippocampus in Neurodegenerative Diseases.International journal of molecular sciences · 2022Review
- IKKβ signaling mediates metabolic changes in the hypothalamus of a Huntington disease mouse model.iScience · 2022Article
- Microarray profiling of hypothalamic gene expression changes in Huntington's disease mouse models.Frontiers in neuroscience · 2022Article
- Oxytocin in Huntington's disease and the spectrum of amyotrophic lateral sclerosis-frontotemporal dementia.Frontiers in molecular neuroscience · 2022Review
- Morphological alterations of the hypothalamus in idiopathic intracranial hypertension.Therapeutic advances in chronic disease · 2022Article
- Clinical, pathological and genetic characteristics of Perry disease-new cases and literature review.European journal of neurology · 2021Review
- Early white matter pathology in the fornix of the limbic system in Huntington disease.Acta neuropathologica · 2021Article
- Effects of excitotoxicity in the hypothalamus in transgenic mouse models of Huntington disease.Heliyon · 2021Article
- Effect of Body Weight on Age at Onset in Huntington Disease: A Mendelian Randomization Study.Neurology. Genetics · 2021Article
- Effects of mutant huntingtin inactivation on Huntington disease-related behaviours in the BACHD mouse model.Neuropathology and applied neurobiology · 2021Article
- The relationship between non-motor features and weight-loss in the premanifest stage of Huntington's disease.PloS one · 2021Article
- Effects of exercise on sleep in neurodegenerative disease.Neurobiology of disease · 2020Review
- Environment and Gene Association With Obesity and Their Impact on Neurodegenerative and Neurodevelopmental Diseases.Frontiers in neuroscience · 2020Review
- Article
- Involvement of orexinergic system in psychiatric and neurodegenerative disorders: A scoping review.Brain circulationArticle
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Huntington's disease (HD) is a fatal genetic neurodegenerative disorder. It has mainly been considered a movement disorder with cognitive symptoms and these features have been associated with pathology of the striatum and cerebral cortex. Importantly, individuals with the mutant huntingtin gene suffer from a spectrum of non-motor features often decades before the motor disorder manifests. These symptoms and signs include a range of psychiatric symptoms, sleep problems and metabolic changes with weight loss particularly in later stages. A higher body mass index at diagnosis is associated with slower disease progression. The common psychiatric symptom of apathy progresses with the disease. The fact that non-motor features are present early in the disease and that they show an association to disease progression suggest that unravelling the underlying neurobiological mechanisms may uncover novel targets for early disease intervention and better symptomatic treatment. The hypothalamus and the limbic system are important brain regions that regulate emotion, social cognition, sleep and metabolism. A number of studies using neuroimaging, postmortem human tissue and genetic manipulation in animal models of the disease has collectively shown that the hypothalamus and the limbic system are affected in HD. These findings include the loss of neuropeptide-expressing neurons such as orexin (hypocretin), oxytocin, vasopressin, somatostatin and VIP, and increased levels of SIRT1 in distinct nuclei of the hypothalamus. This review provides a summary of the results obtained so far and highlights the potential importance of these changes for the understanding of non-motor features in HD.
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