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7T 39K/23Na磁共振成像评估低钾性周期性麻痹肌肉离子平衡的可行性

Feasibility of 7 T 39 K/ 23 Na Magnetic Resonance Imaging for assessing muscular ion balance in hypokalemic periodic paralysis.

作者信息

Mathy Claudius S, Nagel Armin M, Türk Matthias, Stuprich Christoph M, Gerhalter Teresa, Marty Benjamin, Bickelhaupt Sebastian, Laun Frederik B, Dörfler Arnd, Uder Michael, Bäuerle Tobias, Heiss Rafael, Weber Marc-André, Gast Lena V

机构信息

From the Institute of Radiology, University Hospital Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Germany (C.S.M., A.M.N., C.M.S., T.G., S.B., F.B.L., M.U., T.B., R.H., L.V.G.); Division of Medical Physics in Radiology, German Cancer Research Center (DKFZ), Heidelberg, Germany (A.M.N.); Department of Neurology, University Hospital Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Germany (M.T.); Centre for Rare Diseases Erlangen (ZSEER), University Hospital Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Germany (M.T.); Department of Neurology, Medical University of Graz, Graz, Austria (T.G.); Institute of Myology, Neuromuscular Investigation Center, NMR Laboratory, Paris, France (B.M.); Department of Neuroradiology, University Hospital Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Germany (A.D.); Institute of Diagnostic and Interventional Radiology, University Medical Center of Johannes Gutenberg-University, Mainz, Germany (T.B.); and Institute of Diagnostic and Interventional Radiology, Pediatric Radiology and Neuroradiology, University Medical Center Rostock, Rostock, Germany (M.-A.W.).

出版信息

Invest Radiol. 2025 Apr 25. doi: 10.1097/RLI.0000000000001188.

Abstract

OBJECTIVES

Recently introduced potassium-39-( 39 K)-MRI provides a noninvasive approach to assess typically high intracellular K + -levels and can be combined with sodium-23-( 23 Na)-MRI. The aim of this study was to evaluate 39 K/ 23 Na muscle ion homeostasis in hypokalemic periodic paralysis (HypoPP), a rare muscular ion channelopathy, using 7 T MRI.

MATERIALS AND METHODS

Lower legs of patients with HypoPP and healthy controls were prospectively examined between August 2022 and July 2023 (case-control study). Scanning protocol at 3 T included T 1 -weighted, T 2 -weighted STIR sequences, a 6-point-Dixon-type gradient echo and a T2-mapping sequence. 39 K/ 23 Na data were acquired at 7 T using acquisition-weighted Stack-of-Stars sequences. Apparent tissue 39 K/ 23 Na concentrations (aTPC/aTSC) were calculated after correcting for partial-volume and relaxation effects and corrected for proton-density fat-fractions to account for fatty replacement. A 23 Na-inversion-recovery ( 23 Na-IR) sequence served to introduce a stronger intracellular weighting. Differences in central tendency between the HypoPP and control groups and correlations were analyzed.

RESULTS

Thirteen HypoPP-participants and 13 controls were included. Extent of fatty replacement/edema-like changes varied highly with the gastrocnemius medialis muscle most affected. The HypoPP group showed significantly increased aTSC in all 7 analyzed muscles and decreased aTPC in 3 specific muscles. Across all muscles, the mean aTSC was higher in the HypoPP group (median: 33.4 vs 22.5 mM, mean ± SD: 34.3 ± 6.8 vs 21.0 ± 4.8 mM, P < 0.001), whereas the mean aTPC was lower (98.7 vs 109.0 mM, 97.9 ± 12.0 vs 108.7 ± 10.4 mM, P = 0.02). The 23 Na-IR signal was strongly correlated with aTSC (r = 0.77, P < 0.001).

CONCLUSIONS

Combined 39 K/ 23 Na MRI at 7 T demonstrated alterations of sodium and potassium ion homeostasis in HypoPP. These findings could be helpful for a better pathophysiological understanding of HypoPP and may aid in future studies to assess disease extent or monitor treatment efficacy.

摘要

目的

最近推出的钾-39(³⁹K)-MRI提供了一种非侵入性方法来评估通常较高的细胞内钾离子水平,并且可以与钠-23(²³Na)-MRI相结合。本研究的目的是使用7T MRI评估低钾性周期性麻痹(HypoPP,一种罕见的肌肉离子通道病)中的³⁹K/²³Na肌肉离子稳态。

材料与方法

在2022年8月至2023年7月期间对HypoPP患者和健康对照者的小腿进行前瞻性检查(病例对照研究)。3T扫描方案包括T1加权、T2加权短tau反转恢复(STIR)序列、六点狄克逊型梯度回波和T2映射序列。³⁹K/²³Na数据在7T下使用采集加权星状堆叠序列获取。在校正部分容积和弛豫效应后,并校正质子密度脂肪分数以考虑脂肪替代后,计算表观组织³⁹K/²³Na浓度(aTPC/aTSC)。一个²³Na反转恢复(²³Na-IR)序列用于引入更强的细胞内加权。分析HypoPP组和对照组之间的集中趋势差异及相关性。

结果

纳入了13名HypoPP参与者和13名对照者。脂肪替代/水肿样改变的程度差异很大,其中内侧腓肠肌受影响最大。HypoPP组在所有7块分析的肌肉中aTSC显著增加,在3块特定肌肉中aTPC降低。在所有肌肉中,HypoPP组的平均aTSC更高(中位数:33.4对22.5 mM,均值±标准差:34.3±6.8对21.0±4.8 mM,P<0.001),而平均aTPC更低(98.7对109.0 mM,97.9±12.0对108.7±10.4 mM,P = 0.02)。²³Na-IR信号与aTSC密切相关(r = 0.77,P<0.001)。

结论

7T下的³⁹K/²³Na联合MRI显示了HypoPP中钠和钾离子稳态的改变。这些发现可能有助于更好地从病理生理学角度理解HypoPP,并可能有助于未来评估疾病程度或监测治疗效果的研究。

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