title: 7-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.
abstract: Purpose 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.
authors.affiliation: From the Clinic of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany (M.A.W., H.U.K.); Department of Radiology (M.A.W., P.G., M.B.W., H.P.S.) and Division of Medical Physics in Radiology (A.M.N., A.M.M., M.E.L.), German Cancer Research Center (DKFZ), Heidelberg, Germany; and Division of Neurophysiology, Ulm University, Ulm, Germany (K.J.R., F.L.H.).
authors.forename: Marc-André
authors.initials: MA
authors.lastname: Weber
authors.affiliation: From the Clinic of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany (M.A.W., H.U.K.); Department of Radiology (M.A.W., P.G., M.B.W., H.P.S.) and Division of Medical Physics in Radiology (A.M.N., A.M.M., M.E.L.), German Cancer Research Center (DKFZ), Heidelberg, Germany; and Division of Neurophysiology, Ulm University, Ulm, Germany (K.J.R., F.L.H.).
authors.forename: Armin M
authors.initials: AM
authors.lastname: Nagel
authors.affiliation: From the Clinic of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany (M.A.W., H.U.K.); Department of Radiology (M.A.W., P.G., M.B.W., H.P.S.) and Division of Medical Physics in Radiology (A.M.N., A.M.M., M.E.L.), German Cancer Research Center (DKFZ), Heidelberg, Germany; and Division of Neurophysiology, Ulm University, Ulm, Germany (K.J.R., F.L.H.).
authors.forename: Anja M
authors.initials: AM
authors.lastname: Marschar
authors.affiliation: From the Clinic of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany (M.A.W., H.U.K.); Department of Radiology (M.A.W., P.G., M.B.W., H.P.S.) and Division of Medical Physics in Radiology (A.M.N., A.M.M., M.E.L.), German Cancer Research Center (DKFZ), Heidelberg, Germany; and Division of Neurophysiology, Ulm University, Ulm, Germany (K.J.R., F.L.H.).
authors.forename: Philip
authors.initials: P
authors.lastname: Glemser
authors.affiliation: From the Clinic of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany (M.A.W., H.U.K.); Department of Radiology (M.A.W., P.G., M.B.W., H.P.S.) and Division of Medical Physics in Radiology (A.M.N., A.M.M., M.E.L.), German Cancer Research Center (DKFZ), Heidelberg, Germany; and Division of Neurophysiology, Ulm University, Ulm, Germany (K.J.R., F.L.H.).
authors.forename: Karin
authors.initials: K
authors.lastname: Jurkat-Rott
authors.affiliation: From the Clinic of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany (M.A.W., H.U.K.); Department of Radiology (M.A.W., P.G., M.B.W., H.P.S.) and Division of Medical Physics in Radiology (A.M.N., A.M.M., M.E.L.), German Cancer Research Center (DKFZ), Heidelberg, Germany; and Division of Neurophysiology, Ulm University, Ulm, Germany (K.J.R., F.L.H.).
authors.forename: Maya B
authors.initials: MB
authors.lastname: Wolf
authors.affiliation: From the Clinic of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany (M.A.W., H.U.K.); Department of Radiology (M.A.W., P.G., M.B.W., H.P.S.) and Division of Medical Physics in Radiology (A.M.N., A.M.M., M.E.L.), German Cancer Research Center (DKFZ), Heidelberg, Germany; and Division of Neurophysiology, Ulm University, Ulm, Germany (K.J.R., F.L.H.).
authors.forename: Mark E
authors.initials: ME
authors.lastname: Ladd
authors.affiliation: From the Clinic of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany (M.A.W., H.U.K.); Department of Radiology (M.A.W., P.G., M.B.W., H.P.S.) and Division of Medical Physics in Radiology (A.M.N., A.M.M., M.E.L.), German Cancer Research Center (DKFZ), Heidelberg, Germany; and Division of Neurophysiology, Ulm University, Ulm, Germany (K.J.R., F.L.H.).
authors.forename: Heinz-Peter
authors.initials: HP
authors.lastname: Schlemmer
authors.affiliation: From the Clinic of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany (M.A.W., H.U.K.); Department of Radiology (M.A.W., P.G., M.B.W., H.P.S.) and Division of Medical Physics in Radiology (A.M.N., A.M.M., M.E.L.), German Cancer Research Center (DKFZ), Heidelberg, Germany; and Division of Neurophysiology, Ulm University, Ulm, Germany (K.J.R., F.L.H.).
authors.forename: Hans-Ulrich
authors.initials: HU
authors.lastname: Kauczor
authors.affiliation: From the Clinic of Diagnostic and Interventional Radiology, University Hospital Heidelberg, Im Neuenheimer Feld 110, D-69120 Heidelberg, Germany (M.A.W., H.U.K.); Department of Radiology (M.A.W., P.G., M.B.W., H.P.S.) and Division of Medical Physics in Radiology (A.M.N., A.M.M., M.E.L.), German Cancer Research Center (DKFZ), Heidelberg, Germany; and Division of Neurophysiology, Ulm University, Ulm, Germany (K.J.R., F.L.H.).
authors.forename: Frank
authors.initials: F
authors.lastname: Lehmann-Horn
mesh_terms: D000273:Adipose Tissue / Q000000981:diagnostic imaging; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002713:Chlorine; D004487:Edema / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D020514:Hypokalemic Periodic Paralysis / Q000000981:diagnostic imaging* / Q000235:genetics; D007866:Leg / Q000000981:diagnostic imaging*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging; D009154:Mutation; D019047:Phantoms, Imaging; D012975:Sodium Isotopes
chemical_list: D012975:Sodium Isotopes; D002713:Chlorine