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Abstract #2783

Detection of accumulated iron and microglia in the striatum of Huntington’s Disease patients: evidence from post-mortem MRI and histology

Marjolein Bulk1, Ernst Suidgeest1, Ingrid Hegeman-Kleinn2, Sjoerd van Duinen2, Jan Lewerenz3, Bernhard Landwehrmeyer3, Itamar Ronen1, and Louise van der Weerd1,4

1Radiology, LUMC, Leiden, Netherlands, 2Pathology, LUMC, Leiden, Netherlands, 3Neurology, Ulm University Hospital, Ulm, Germany, 4Human Genetics, LUMC, Leiden, Netherlands

We detected increased iron deposits in the striatum of post-mortem brain tissue of Huntington’s Disease (HD) patients. High-field T2*-weighted MRI of the striatum showed a different imaging phenotype in HD patients compared to controls, and spatially correlated with the iron distribution obtained from histology. Increased iron was observed in the matrix and in cells morphologically resembling glial cells. These findings bridge the gap between neuropathological and clinical imaging findings and point to iron accumulation as a potential imaging biomarker for disease progression in vivo, possibly reflecting neuroinflammation.

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