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  Pattern of Cerebellar Atrophy in Friedreich’s Ataxia: Using the SUIT Template

Lindig, T., Bender, B., Kumar, V., Hauser, T.-K., Grodd, W., Brendel, B., et al. (2019). Pattern of Cerebellar Atrophy in Friedreich’s Ataxia: Using the SUIT Template. The Cerebellum, 18(3), 435-447. doi:10.1007/s12311-019-1008-z.

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Item Permalink: http://hdl.handle.net/21.11116/0000-0003-034A-7 Version Permalink: http://hdl.handle.net/21.11116/0000-0003-9A35-4
Genre: Journal Article

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Lindig, T1, 2, Author              
Bender, B1, 2, Author              
Kumar, V1, 2, Author              
Hauser, T-K, Author
Grodd, W1, 2, Author              
Brendel, B, Author
Just, J, Author
Synofzik, M, Author
Klose, U, Author
Scheffler, K1, 2, Author              
Ernemann, U, Author
Schöls, L, Author
Affiliations:
1Max Planck Institute for Biological Cybernetics, Max Planck Society, ou_1497794              
2Department High-Field Magnetic Resonance, Max Planck Institute for Biological Cybernetics, Max Planck Society, ou_1497796              

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 Abstract: Whole-brain voxel-based morphometry (VBM) studies revealed patterns of patchy atrophy within the cerebellum of Friedreich’s ataxia patients, missing clear clinico-anatomic correlations. Studies so far are lacking an appropriate registration to the infratentorial space. To circumvent these limitations, we applied a high-resolution atlas template of the human cerebellum and brainstem (SUIT template) to characterize regional cerebellar atrophy in Friedreich’s ataxia (FRDA) on 3-T MRI data. We used a spatially unbiased voxel-based morphometry approach together with T2-based manual segmentation, T2 histogram analysis, and atlas generation of the dentate nuclei in a representative cohort of 18 FRDA patients and matched healthy controls. We demonstrate that the cerebellar volume in FRDA is generally not significantly different from healthy controls but mild lobular atrophy develops beyond normal aging. The medial parts of lobule VI, housing the somatotopic representation of tongue and lips, are the major site of this lobular atrophy, which possibly reflects speech impairment. Extended white matter affection correlates with disease severity across and beyond the cerebellar inflow and outflow tracts. The dentate nucleus, as a major site of cerebellar degeneration, shows a mean volume loss of about 30%. Remarkably, not the atrophy but the T2 signal decrease of the dentate nuclei highly correlates with disease duration and severity.

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 Dates: 2019-022019-06
 Publication Status: Published in print
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 Table of Contents: -
 Rev. Method: -
 Identifiers: DOI: 10.1007/s12311-019-1008-z
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Title: The Cerebellum
Source Genre: Journal
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Publ. Info: New York, NY : Springer
Pages: - Volume / Issue: 18 (3) Sequence Number: - Start / End Page: 435 - 447 Identifier: ISSN: 1473-4222
CoNE: https://pure.mpg.de/cone/journals/resource/1473-4222