Hyperkalemic periodic paralysis and permanent weakness: 3-T MR imaging depicts intracellular 23Na overload--initial results.
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TL;DR: 23Na MR imaging may have potential to aid monitoring of medical treatment that reduces this overload and depicts increased myoplasmic Na+ in HyperPP with permanent weakness, which may cause muscle degeneration developing with age.
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Abstract: 23Na MR imaging showed that muscle tissue of patients with hyperkalemic periodic paralysis (HyperPP) with permanent muscle weakness features a higher average Na+ concentration, as well as a higher 23Na inversion-recovery signal intensity during rest, compared with patients with HyperPP with transient episodes of weakness.
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References
Hyperkalemic periodic paralysis and the adult muscle sodium channel alpha-subunit gene.
Bertrand Fontaine,Tejvir S. Khurana,Eric P. Hoffman,Gail A. P. Bruns,Jonathan L. Haines,James A. Trofatter,Mark P. Hanson,Jaimie Rich,Heather McFarlane,Diane McKenna Yasek,Donna M. Romano,James F. Gusella,Robert H. Brown +12 more
TL;DR: Parts of the adult muscle sodium channel alpha-subunit gene were cloned and mapped near the human growth hormone locus (GH1) on chromosome 17 and showed tight linkage to the genetic defect with no recombinants detected.
Non-dystrophic myotonias and periodic paralyses. A European Neuromuscular Center Workshop held 4-6 October 1992, Ulm, Germany.
TL;DR: The participants of the workshop approved of this classification of skeletal muscle CIchannel diseases and suggested that the Editor of Neuromuscular Disorders be asked to adopt it for his Table of Gene Locations.
3 Tesla sodium inversion recovery magnetic resonance imaging allows for improved visualization of intracellular sodium content changes in muscular channelopathies
Armin M. Nagel,Erick Amarteifio,Erick Amarteifio,Frank Lehmann-Horn,Karin Jurkat-Rott,Wolfhard Semmler,Lothar R. Schad,Marc-André Weber,Marc-André Weber +8 more
TL;DR: The results indicate that the 23Na-IR sequence allows for a weighting toward intracellular sodium and enables an improved analysis of pathophysiological changes that occur in muscles of patients with muscular channelopathies.
Evaluation of patients with paramyotonia at 23Na MR imaging during cold-induced weakness.
Marc-André Weber,Sonia Nielles-Vallespin,Hagen B. Huttner,Johannes C. Wöhrle,Karin Jurkat-Rott,Frank Lehmann-Horn,Lothar R. Schad,Hans-Ulrich Kauczor,Marco Essig,Hans-Michael Meinck +9 more
TL;DR: 23Na MR imaging enables visualization of muscular Na+ accumulation associated with muscle weakness in patients with PC, and effects of specific therapy can be detected.