ArticleBrain pathology (Zurich, Switzerland)2014
Huntington's disease (HD): degeneration of select nuclei, widespread occurrence of neuronal nuclear and axonal inclusions in the brainstem.
Article in Brain pathology (Zurich, Switzerland), 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.
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Who cites it
29 citing papers in PubMed, 1 synthesis or guideline pooled it, 62 citations in OpenAlex.
- Systemic Symptoms in Huntington's Disease: A Comprehensive Review.Movement disorders clinical practice · 2024Pooled it
- The Hsp40 cochaperone DNAJC7 regulates polyglutamine aggregation and exhibits context-dependent effects on polyglycine aggregation.The Journal of biological chemistry · 2026Article
- Associations Between Vestibular Function and White Matter Microstructure in Healthy Older Adults.medRxiv : the preprint server for health sciences · 2025Article
- Reduced Vestibular Function is Associated With Cortical Surface Shape Changes in the Frontal Cortex.Human brain mapping · 2025Article
- Reduced Vestibular Function is Associated with Cortical Surface Shape Changes in the Frontal Cortex.medRxiv : the preprint server for health sciences · 2024Article
- Circadian Interventions in Preclinical Models of Huntington's Disease: A Narrative Review.Biomedicines · 2024Review
- Longitudinal imaging highlights preferential basal ganglia circuit atrophy in Huntington's disease.Brain communications · 2023Article
- Disordered Decision Making: A Cognitive Framework for Apathy and Impulsivity in Huntington's Disease.Movement disorders : official journal of the Movement Disorder Society · 2022Review
- The distribution and density of Huntingtin inclusions across the Huntington disease neocortex: regional correlations with Huntingtin repeat expansion independent of pathologic grade.Acta neuropathologica communications · 2022Article
- Juvenile-Onset Huntington Disease Pathophysiology and Neurodevelopment: A Review.Movement disorders : official journal of the Movement Disorder Society · 2022Review
- Recent developments in the understanding of the interactions between the vestibular system, memory, the hippocampus, and the striatum.Frontiers in neurology · 2022Review
- Label-free photothermal disruption of cytotoxic aggregates rescues pathology in a C. elegans model of Huntington's disease.Scientific reports · 2021Article
- New Avenues for the Treatment of Huntington's Disease.International journal of molecular sciences · 2021Review
- Autonomic Changes in Juvenile-Onset Huntington's Disease.Brain sciences · 2020Article
- Disruption of zinc transporter ZnT3 transcriptional activity and synaptic vesicular zinc in the brain of Huntington's disease transgenic mouse.Cell & bioscience · 2020Article
- Neuropathology and pathogenesis of extrapyramidal movement disorders: a critical update. II. Hyperkinetic disorders.Journal of neural transmission (Vienna, Austria : 1996) · 2019Review
- Do Disruptions in the Circadian Timing System Contribute to Autonomic Dysfunction in Huntington's Disease?The Yale journal of biology and medicine · 2019Review
- Endogenous mouse huntingtin is highly abundant in cranial nerve nuclei, co-aggregates to Abeta plaques and is induced in reactive astrocytes in a transgenic mouse model of Alzheimer's disease.Acta neuropathologica communications · 2019Article
- Multiple clinical features of Huntington's disease correlate with mutant HTT gene CAG repeat lengths and neurodegeneration.Journal of neurology · 2019Review
- Computerised Dynamic Posturography in Premanifest and Manifest individuals with Huntington's Disease.Scientific reports · 2018Article
Corrections and comments
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Authors and funding
13 authors at 5 institutions in 3 countries.
Funding
Abstract
Huntington's disease (HD) is a progressive polyglutamine disease that leads to a severe striatal and layer-specific neuronal loss in the cerebral neo-and allocortex. As some of the clinical symptoms (eg, oculomotor dysfunctions) suggested a degeneration of select brainstem nuclei, we performed a systematic investigation of the brainstem of eight clinically diagnosed and genetically confirmed HD patients. This post-mortem investigation revealed a consistent neuronal loss in the substantia nigra, pontine nuclei, reticulotegmental nucleus of the pons, superior and inferior olives, in the area of the excitatory burst neurons for horizontal saccades, raphe interpositus nucleus and vestibular nuclei. Immunoreactive intranuclear neuronal inclusions were present in all degenerated and apparently spared brainstem nuclei and immunoreactive axonal inclusions were observed in all brainstem fiber tracts of the HD patients. Degeneration of brainstem nuclei can account for a number of less well-understood clinical HD symptoms (ie, cerebellar, oculomotor and vestibular symptoms), while the formation of axonal aggregates may represent a crucial event in the cascades of pathological events leading to neurodegeneration in HD.
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