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