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ORIGINAL RESEARCH PEDIATRICS Accumulation of Brain Hypointense Foci on Susceptibility- Weighted Imaging in Childhood Ataxia Telangiectasia R.A. Dineen, C.V. Blanchard, S. Pszczolkowski, S. Paine, M. Prasad, G. Chow, W.P. Whitehouse, and D.P. Auer ABSTRACT BACKGROUND AND PURPOSE: SWI hypointense cerebral lesions have been reported in adults with the inherited cerebellar neuro- degenerative disorder ataxia telangiectasia. This study aims to establish the prevalence, age-dependency, and spatial distribution of these lesions in children and young people with ataxia telangiectasia. MATERIALS AND METHODS: Participants with classic ataxia telangiectasia and matched controls underwent SWI acquisition at 3T at 1 or 2 time points. SWI hypointense lesions were manually labeled according to the Microbleed Anatomical Rating Scale. Differences in preva- lence of lesion number between groups with ataxia telangiectasia and without ataxia telangiectasia were tested with the Fisher exact test, and differences in age between participants with ataxia telangiectasia with and without lesions were tested using independent sam- ples Mann-Whitney U test. The relationship between age and lesion number was modeled as an exponential function. RESULTS: Analyzable SWI datasets from 17 participants with ataxia telangiectasia (with median age at first scan of 12.4 years; range, 4.6– 20.2 years; 8 [47%] were female) and 22
American Journal of Neuroradiology – American Journal of Neuroradiology
Published: Jun 1, 2021
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