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Striato-cortical connectivity patterns predict clinical profiles in Huntington's disease.

Audrey E De Paepe¹, Vasiliki Bikou², Eylül Turan³

¹Cognition and Brain Plasticity Unit [Bellvitge Biomedical Research Institute - IDIBELL], 08097 L'Hospitalet de Llobregat, Barcelona, Spain; Department of Cognition, Development and Education Psychology, Universitat de Barcelona, Barcelona, Spain.

Neuroimage. Clinical

|April 25, 2025

Summary

Distinct clinical profiles in Huntington's disease, motor-cognitive and behavioral, are linked to unique brain connectivity patterns. This research reveals how striato-cortical circuits change in Huntington's disease.

Keywords:

Clinical profiles Huntington’s disease Individual differences Principal component analysis rs-fMRI

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Area of Science:

Neuroscience
Genetics
Neurology

Background:

Huntington's disease is an inherited neurodegenerative disorder impacting striato-cortical circuits.
It presents with significant heterogeneity in symptom severity, progression, and neurodegenerative patterns.

Purpose of the Study:

To identify distinct functional striato-cortical connectivity signatures.
To determine if these signatures can predict clinical profiles in Huntington's disease.

Main Methods:

38 Huntington's disease gene expansion carriers underwent clinical assessments and multimodal MRI.
Principal component analysis characterized clinical profiles (motor-cognitive, behavioral).
Seed-based functional connectivity maps (basal ganglia seeds) and regression analyses identified relationships between connectivity and clinical profiles.

Main Results:

Two primary clinical profiles were identified: motor-cognitive and behavioral.
Motor-cognitive symptoms correlated with altered connectivity in executive and premotor areas and the nucleus accumbens.
Behavioral symptoms were linked to decreased connectivity in limbic networks; basal ganglia atrophy correlated with connectivity and symptom severity.

Conclusions:

Distinct clinical profiles in Huntington's disease are associated with unique functional and structural brain signatures.
Striato-cortical circuits demonstrate functional interaction and potential reorganization in Huntington's disease.