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Multimodal Imaging and Visual Evoked Potentials Reveal Key Structural and Functional Features That Distinguish Symptomatic from Presymptomatic Huntington's Disease Brain

Thota, SM and Chan, KL and Pradhan, SS and Nagabushana, B and Priyanka, GB and Sunil, HV and Kanneganti, V and Vasoya, P and Vinnakote, KM and Viswamitra, S and Thambisetty, M and Kumar, D and Tiwari, V and Joshy, EV and Sivaramakrishnan, V (2021) Multimodal Imaging and Visual Evoked Potentials Reveal Key Structural and Functional Features That Distinguish Symptomatic from Presymptomatic Huntington's Disease Brain. In: Neurology India, 69 (5). pp. 1247-1258.

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Official URL: https://doi.org/10.4103/0028-3886.329528

Abstract

Background: Huntington's disease (HD) is a progressive neurodegenerative disorder characterized by motor, cognitive, and psychiatric abnormalities. Currently, matched analyses of structural and functional differences in the brain from the same study cohort and, specifically, in HD patients from an ethnically diverse Indian population are lacking. Such findings aid in identifying noninvasive and sensitive imaging biomarkers. Objective: The aim of the study was to understand the structural and functional differences between HD and control brain, and presymptomatic and symptomatic HD brain in the Indian population. Materials and Methods: Seventeen HD (11 symptomatic HD S-HD and six presymptomatic HD P-HD, with comparable CAG repeats), and 12 healthy controls were examined. Macrostructural (volume), microstructural (diffusivity), and functional (neurochemical levels and glucose metabolism) imaging of the brain was done along with the determination of visual latencies. Results: HD brain showed increased intercaudate distance; significant subcortical volumetric loss; reduced fractional anisotropy; increased mean, axial, and radial diffusivity; lower levels of total N-acetyl aspartate; elevated total choline levels; and reduced glucose metabolism compared with control brain. Interestingly, compared with P-HD, S-HD patients demonstrated a strong inverse correlation between age at onset and CAG repeat length, and prolonged P100 latency. In addition, caudate and putamen in S-HD brain showed significant volumetric loss and increased diffusivity compared with P-HD brain. Conclusions: HD brain showed distinct macrostructural, microstructural, and functional differences compared with control brain in the Indian population. Interestingly, patients with S-HD had a significant volumetric loss, increased diffusivity, altered neurochemical profile, and delayed P100 latency compared with P-HD patients. Examining these alterations clinically could aid in monitoring the progression of HD. © 2021 Wolters Kluwer Medknow Publications. All rights reserved.

Item Type: Journal Article
Publication: Neurology India
Publisher: Wolters Kluwer Medknow Publications
Additional Information: The copyright for this article belongs to Wolters Kluwer Medknow Publications
Department/Centre: Autonomous Societies / Centres > Centre for Brain Research
Date Deposited: 03 Dec 2021 08:49
Last Modified: 03 Dec 2021 08:49
URI: http://eprints.iisc.ac.in/id/eprint/70629

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