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Huntington Disease l: Introduction

Huntington Disease l: Introduction

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 reduced penetrance,...

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Symmetric Bihemispheric Postmortem Brain Cutting to Study Healthy and Pathological Brain Conditions in Humans

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Published on: December 18, 2016

Asymmetric brain atrophy in Huntington's disease: A postmortem MRI study.

Eardi Lila¹, David Hunt², Daniel D Child³

¹Department of Biostatistics, University of Washington, Seattle, WA, USA.

Journal of Huntington'S Disease

|April 13, 2025

Summary

Huntington's disease causes progressive brain atrophy, particularly in the striatum. This study found significant gray and white matter volume loss, predominantly in the left hemisphere of patients.

Keywords:

Huntington's disease MRI asymmetry atrophy

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Area of Science:

Neuroscience
Neuroimaging
Genetics

Background:

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder.
It results from a CAG repeat expansion in the HTT gene.
Medium spiny neurons in the striatum are particularly vulnerable, leading to caudate and putamen atrophy.

Purpose of the Study:

To investigate region-specific gray and white matter volume differences in Huntington's disease patients compared to controls.
To characterize the pattern and extent of brain atrophy in symptomatic HD.

Main Methods:

Postmortem MRI analysis of brains from 15 symptomatic Huntington's disease patients and 26 control subjects.
Comparative assessment of regional gray and white matter volumes between the two groups.

Main Results:

Significant gray and white matter volume reductions were observed in Huntington's disease patients.
The largest volume decreases were noted in the caudate and putamen.
Atrophy was predominantly left-hemisphere biased, affecting regions near the pars opercularis, precentral, supramarginal, pars orbitalis gyri, and lateral orbitofrontal cortex.

Conclusions:

Huntington's disease is characterized by progressive, diffuse, and asymmetric gray and white matter atrophy.
The observed hemispheric asymmetry in atrophy patterns differs from controls, though its cause remains unclear.
This study enhances the detailed characterization of brain changes in Huntington's disease using postmortem MRI.