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Related Concept Videos

Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

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Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show...
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Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and...
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Functional Brain Systems: Limbic System01:15

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The limbic system, often called the "emotional brain," is a complex set of structures located deep within the brain. The intricate network of the limbic system supports a wide range of psychological functions, from emotional regulation to memory formation and sensory processing. This functional brain region encompasses specific parts of the diencephalon and the cerebrum, integrating the higher mental functions of the cerebral cortex with the primitive emotional responses of the deep brain...
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Diencephalon: Anatomical Regions01:30

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The diencephalon, etymologically translated as 'through brain,' plays an integral role as the conduit between the cerebrum and the vast extent of the nervous system. However, the olfactory system is an exception, as it interfaces directly with the cerebrum. The diencephalon, deeply ensconced beneath the cerebrum, primarily consists of three paired structures — the thalamus, hypothalamus, and epithelamus. It also includes accessory structures such as the subthalamus, which houses the...
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Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
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Diencephalon: Hypothalamus and Coordination01:23

Diencephalon: Hypothalamus and Coordination

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The hypothalamus is a small yet highly complex and essential brain region that plays a crucial role in regulating various bodily functions. Anatomically, it is located at the base of the brain, just above the brainstem and below the thalamus, forming part of the limbic system.
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Related Experiment Video

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Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
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Hypothalamic and Limbic System Changes in Huntington's Disease.

Asa Petersén1, Sanaz Gabery2

  • 1Translational Neuroendocrine Research Unit, Department of Experimental Medical Sciences, Lund University, Lund, Sweden. Asa.Petersen@med.lu.se

Journal of Huntington'S Disease
|July 27, 2014
PubMed
Summary

Huntington's disease (HD) involves early changes in the hypothalamus and limbic system, not just motor symptoms. These brain region alterations contribute to non-motor and motor aspects of this neurodegenerative disorder.

Keywords:
Huntingtinamygdalahuntingtonhypothalamusorexinoxytocin

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Area of Science:

  • Neuroscience
  • Genetics
  • Neurology

Background:

  • Huntington's disease (HD) is a neurodegenerative disorder caused by an expanded CAG repeat in the huntingtin gene.
  • Clinical diagnosis traditionally relies on motor disturbances linked to striatal pathology.
  • Emerging evidence highlights early and common non-motor symptoms in HD patients.

Purpose of the Study:

  • To review the current understanding of early pathological changes in the hypothalamus and limbic system in Huntington's disease.
  • To establish the role of these brain regions in both non-motor and motor aspects of HD.
  • To synthesize findings from clinical studies and animal models.

Main Methods:

  • Review of clinical studies on Huntington's disease.
  • Analysis of findings from animal models of Huntington's disease.
  • Examination of data from postmortem tissue, MRI, and PET imaging.

Main Results:

  • Non-motor symptoms such as psychiatric, cognitive, and sleep disturbances are prevalent early in HD.
  • Hypothalamic and limbic system dysfunction is implicated in these non-motor features.
  • Evidence from multiple study types confirms early changes in these interconnected brain regions in HD.

Conclusions:

  • Hypothalamic and limbic system alterations are integral to the pathology of Huntington's disease.
  • These changes contribute significantly to the early clinical presentation of HD, including non-motor symptoms.
  • Future research should continue to explore the role of these regions in HD progression and treatment strategies.