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Case Reports
. 2023 Feb;49(1):e12858.
doi: 10.1111/nan.12858. Epub 2022 Nov 13.

Post-mortem 7T MR imaging and neuropathology in middle stage juvenile-onset Huntington disease: A case report

Hannah S Bakels  1 , Sjoerd G van Duinen  2 , Jeroen de Bresser  3 , Willeke M C van Roon-Mom  4 , Louise van der Weerd  3   4 , Susanne T de Bot  1
Affiliations
Case Reports

Post-mortem 7T MR imaging and neuropathology in middle stage juvenile-onset Huntington disease: A case report

Hannah S Bakels et al. Neuropathol Appl Neurobiol. 2023 Feb.
No abstract available

Keywords: Huntington disease; Vonsattel grading system; juvenile-onset Huntington disease; magnetic resonance imaging; neuropathologic staging; neuropathology; post-mortem.

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Conflict of interest statement

The authors declare to have no conflict of interest with the presented findings.

Figures

FIGURE 1
FIGURE 1
Post‐mortem 7T MRI imaging and neuropathology of a juvenile‐onset Huntington disease (HD) brain donor. T1‐weighted image in a transverse plane of the HD brain donor (A) demonstrates severe bilateral atrophy of the putamen (dashed green line) and slight to moderate atrophy of the HCN (dashed blue line) as compared to an age‐ and sex‐matched control (B). T1‐weighted image in the coronal plane of the HD brain donor (C) parallels neuropathology macroscopy findings of the right hemisphere of the same HD donor (D), demonstrating a normal concave contour of the HCN into the lateral ventricle (right side of the dashed blue line) and moderate to severe atrophy of the putamen (dashed green line). H&E staining of the HCN (E) and rostral putamen (not shown) reveals normal neuronal cellularity (circles) and no signs of gliosis. Microscopy of the BCN (F) and caudal putamen (G) reveals mild neuronal loss (fewer circles) and gliosis. Mutant–Huntingtin staining (H) reveals scattered nuclear immunoreactivity (circles) of neurons in the BCN, similar to neurons in the frontal lobe and putamen (not shown). Legend: BCN, body of caudate nucleus; HCN, head of caudate nucleus; H&E, haematoxylin and eosin
See this image and copyright information in PMC

References

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