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Published in: Neuroradiology 9/2019

01-09-2019 | Magnetic Resonance Imaging | Paediatric Neuroradiology

Aberrant myelination in patients with Sturge-Weber syndrome analyzed using synthetic quantitative magnetic resonance imaging

Authors: Christina Andica, Akifumi Hagiwara, Masaaki Hori, Takuya Haruyama, Shohei Fujita, Tomoko Maekawa, Koji Kamagata, Mariko Takemura Yoshida, Michimasa Suzuki, Hidenori Sugano, Hajime Arai, Shigeki Aoki

Published in: Neuroradiology | Issue 9/2019

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Abstract

Purpose

Accelerated myelination in the affected hemisphere has been demonstrated previously in patients with Sturge-Weber syndrome (SWS). This prospective study investigated myelin-related changes in patients with unilateral SWS using synthetic quantitative magnetic resonance imaging (qMRI).

Methods

Fourteen children with unilateral SWS were categorized according to age, i.e., ≤ 2 years (group A, n = 5, mean age 1.1 years, 3 males) and > 2 years (group B, n = 9, mean age 3.9 years, 4 males). All children underwent two-dimensional synthetic qMRI. The myelin volume in the cerebral hemisphere and white matter (WM) myelin volume fraction (MVF), proton density (PD), R1 and R2 relaxation rates ipsilateral to the leptomeningeal enhancement, and/or a port-wine birthmark were compared with the corresponding values in the contralateral hemisphere.

Results

In group A, 3 patients had a higher myelin volume in the ipsilateral hemisphere and a higher MVF, R1, and R2 and lower PD in the ipsilateral WM than on the contralateral side; the findings were the opposite in the remaining two patients. All patients in group B had a significantly lower myelin volume in the ipsilateral hemisphere (P < 0.05) and a lower MVF and R1 and higher PD in the ipsilateral WM than on the contralateral side (P < 0.0125).

Conclusion

Higher estimated myelin was observed on the ipsilateral side in some patients aged ≤ 2 years and lower myelin on the ipsilateral side in all older patients. Synthetic qMRI might be useful for showing myelin-related abnormalities in SWS.
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Metadata
Title
Aberrant myelination in patients with Sturge-Weber syndrome analyzed using synthetic quantitative magnetic resonance imaging
Authors
Christina Andica
Akifumi Hagiwara
Masaaki Hori
Takuya Haruyama
Shohei Fujita
Tomoko Maekawa
Koji Kamagata
Mariko Takemura Yoshida
Michimasa Suzuki
Hidenori Sugano
Hajime Arai
Shigeki Aoki
Publication date
01-09-2019
Publisher
Springer Berlin Heidelberg
Published in
Neuroradiology / Issue 9/2019
Print ISSN: 0028-3940
Electronic ISSN: 1432-1920
DOI
https://doi.org/10.1007/s00234-019-02250-9

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