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000130829 0247_ $$2doi$$a10.1148/radiol.2016151617
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000130829 0247_ $$2ISSN$$a1527-1315
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000130829 1001_ $$0P:(DE-He78)5d10a98475a036b2a1ad3c2316d4b3ab$$aWeber, Marc-Andre$$b0$$eFirst author$$udkfz
000130829 245__ $$a7-T (35)Cl and (23)Na MR Imaging for Detection of Mutation-dependent Alterations in Muscular Edema and Fat Fraction with Sodium and Chloride Concentrations in Muscular Periodic Paralyses.
000130829 260__ $$aOak Brook, Ill.$$bSoc.$$c2016
000130829 3367_ $$2DRIVER$$aarticle
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000130829 520__ $$aPurpose To determine whether altered sodium (Na(+)) and chloride (Cl(-)) homeostasis can be visualized in periodic paralyses by using 7-T sodium 23 ((23)Na) and chlorine 35 ((35)Cl) magnetic resonance (MR) imaging. Materials and Methods Institutional review board approval and informed consent of all participants were obtained. (23)Na (repetition time msec/echo time msec, 160/0.35) and (35)Cl (40/0.6) MR imaging of both lower legs was performed with a 7-T whole-body system in patients with genetically confirmed hypokalemic periodic paralysis (Cav1.1-R1239H mutation, n = 5; Cav1.1-R528H mutation, n = 8) and Andersen-Tawil syndrome (n = 3) and in 16 healthy volunteers. Additionally, each participant underwent 3-T proton MR imaging on the same day by using T1-weighted, short-tau inversion-recovery, and Dixon-type sequences. Muscle edema was assessed on short-tau inversion-recovery images, fatty degeneration was assessed on T1-weighted images, and muscular fat fraction was quantified with Dixon-type imaging. Na(+) and Cl(-) were quantified in the soleus muscle by using three phantoms that contained 10-, 20-, and 30-mmol/L NaCl solution and 5% agarose gel as a reference. Parametric data for all subpopulations were tested by using one-way analysis of variance with the Dunnett test, and correlations were assessed with the Spearman rank correlation coefficient. Results Median muscular (23)Na concentration was higher in patients with Cav1.1-R1239H (34.7 mmol/L, P < .001), Cav1.1-R528H (32.0 mmol/L, P < .001), and Kir2.1 (24.3 mmol/L, P = .035) mutations than in healthy volunteers (19.9 mmol/L). Median muscular normalized (35)Cl signal intensity was higher in patients with Cav1.1-R1239H (27.6, P < .001) and Cav1.1-R528H (23.6, P < .001) than in healthy volunteers (12.6), but not in patients with the Kir2.1 mutation (14.3, P = .517). When compared with volunteers, patients with Cav1.1-R1239H and Cav1.1-R528H showed increased muscular edema (P < .001 and P = .003, respectively) and muscle fat fraction (P < .001 and P = .017, respectively). Conclusion With 7-T MR imaging, changes of Na(+) and Cl(-) homeostasis can be visualized in periodic paralyses and are most pronounced in the severe phenotype Cav1.1-R1239H, with up to daily paralytic episodes. (©) RSNA, 2016 An earlier incorrect version of this article appeared online. This article was corrected on April 18, 2016.
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000130829 650_7 $$2NLM Chemicals$$aSodium Isotopes
000130829 650_7 $$04R7X1O2820$$2NLM Chemicals$$aChlorine
000130829 7001_ $$0P:(DE-He78)054fd7a5195b75b11fbdc5c360276011$$aNagel, Armin$$b1$$udkfz
000130829 7001_ $$0P:(DE-He78)c6bc8de11c5017573e6c2dd76481a259$$aMarschar, Anja$$b2$$udkfz
000130829 7001_ $$0P:(DE-He78)ec15551e1bf57c41065d3502b66b1fe7$$aGlemser, Philip Alexander$$b3$$udkfz
000130829 7001_ $$aJurkat-Rott, Karin$$b4
000130829 7001_ $$0P:(DE-HGF)0$$aWolf, Maya B$$b5
000130829 7001_ $$0P:(DE-He78)022611a2317e4de40fd912e0a72293a8$$aLadd, Mark$$b6$$udkfz
000130829 7001_ $$0P:(DE-He78)3d04c8fee58c9ab71f62ff80d06b6fec$$aSchlemmer, Heinz-Peter$$b7$$udkfz
000130829 7001_ $$aKauczor, Hans-Ulrich$$b8
000130829 7001_ $$aLehmann-Horn, Frank$$b9
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