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. 2021 Aug 26;3(3):fcab185. doi: 10.1093/braincomms/fcab185

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© The Author(s) (2021). Published by Oxford University Press on behalf of the Guarantors of Brain.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

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Neuroimaging findings in patients with KLHL11-Abs. Axial brain MRI (A), showing hypersignal asymmetrically involving both cerebellar hemispheres, colocalizing with an area of cerebellar hypometabolism on brain PET (B). Sagittal spine MRI showing spinal cord hypersignal in a patient with myelitis (C). Marked cerebellar atrophy is shown in both sagittal (D) and axial (E) brain MRI. The atrophy is more pronounced in the vermis and associates with an area of hypersignal involving the temporal lobe (E). Axial brain MRIs showing hypersignal involving the left mesial temporal lobe (F), the right thalamus (G) and coexistence of left mesial temporal lobe hypersignal and right hippocampal atrophy.