PMID: 9447608Feb 3, 1998Paper

Correlative MR imaging and 31P-MR spectroscopy study in sarcoglycan deficient limb girdle muscular dystrophy

Neuromuscular Disorders : NMD
R LodiD J Taylor

Abstract

We combined magnetic resonance (MR) imaging and phosphorus magnetic resonance spectroscopy (31P-MRS) to study skeletal muscle in seven patients with limb girdle muscular dystrophy (LGMD) with a variable deficiency of the alpha-, beta-, and gamma-sarcoglycan but normal dystrophin expression on muscle biopsy. T1- and T2-weighted spin-echo axial leg images showed the highest degree of fat replacement in soleus, tibialis anterior and peroneal muscles while gastrocnemius and tibialis posterior were less affected. In LGMD patients as a group, calf muscle phosphorylated compound content did not differ from controls, but the cytosolic pH was increased (P = 0.02). The degree of calf muscle fat replacement correlated inversely with cytosolic pH (r = 0.74) and directly with PCr/ATP (r = 0.74). Muscle oxidative metabolism was normal in LGMD patients. Our findings show that primary deficits of sarcoglycan complex lead to specific morphological and metabolic patterns of skeletal muscle involvement.

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Citations

May 8, 2010·Skeletal Radiology·Adrian DegardinTanya Stojkovic
Apr 28, 2010·European Radiology·Mike P WattjesDirk Fischer
Feb 24, 2005·Journal of Neurology·Dirk FischerRolf Schröder
Sep 26, 2012·Neuromuscular Disorders : NMD·Giorgio TascaEnzo Ricci
Jun 6, 2009·The Journal of Biological Chemistry·Séverine GrohKevin P Campbell
Jul 9, 2013·Radiologic Clinics of North America·Hee Kyung KimTal Laor
Feb 25, 2015·Current Reviews in Musculoskeletal Medicine·Michel D CremaAbdalla Y Skaf
Jan 24, 2017·Neuropathology and Applied Neurobiology·H Jungbluth
Sep 25, 2002·Journal of Child Neurology·Ulrike ScharaWilhelm Mortier
Sep 11, 2017·Journal of Neurology, Neurosurgery, and Psychiatry·Giorgio TascaClaudio Bruno
Jan 30, 2007·Journal of Magnetic Resonance Imaging : JMRI·Eugenio MercuriFrancesco Muntoni
Aug 5, 1999·Journal of Computer Assisted Tomography·J D McDanielL P Mark
Jul 9, 2020·Veterinary Pathology·Peter VogelDavid R Powell
Feb 26, 2019·Frontiers in Neurology·Matteo PaolettiMassimiliano Filosto
Dec 28, 2019·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Stephen M ChrzanowskiSeward B Rutkove
Jul 22, 2015·Journal of Neuromuscular Diseases·Alexander Peter Murphy, Volker Straub
May 9, 2000·Journal of Neuroimaging : Official Journal of the American Society of Neuroimaging·M B FranczakR W Prost

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