Comparison of the clinical state and its changes in patients with Duchenne and Becker muscular dystrophy with results of in vivo 31P magnetic resonance spectroscopy

M. Hájek, A. Grosmanová, A. Horská, P. Urban

Research output: Contribution to journalArticle

Abstract

A total of 14 boys with the Duchenne and Becker forms of muscular dystrophy (DMD, BMD) were examined using 31P magnetic resonance (MR) spectroscopy; 12 boys were examined repeatedly. The results were correlated with clinical findings (including those of genetic tests) and with data obtained from examinations of an age-matched control group. Evaluation of results using principal component analysis revealed maximum variability in the following ratios: phosphocreatine/inorganic phosphate (PCr/Pi), phosphocreatine/phosphodiesters (PCr/PDe) and phosphocreatine/phosphomonoesters (PCr/PMe). A decrease in PCr/Pi correlates with weakness of the hip girdle and of the lower part of the shoulder girdle in DMD/BMD patients. The values of all ratios in the group of patients with the DMD phenotype differ significantly from results obtained in the group with the BMD phenotype. Continoous follow-up of patients using 31P MR spectroscopy revealed a marked decrease in PCr/Pi in DMD/BMD patients at an age that could be expected in subjects with a typical clinical course of DMD/BMD. An attempt to manage a concomitant disease with prednisone and carnitene was followed by an increase in PCr/Pi in 3 cases. A rise in the PCr/Pi ratio signalled clinical improvement in the patients. A decrease in PCr/Pi was found after controlled physical training, a finding consistent with data obtained from clinical observations describing an adverse effect of physical stress on the dystrophic process.

Original languageEnglish (US)
Pages (from-to)499-506
Number of pages8
JournalEuropean radiology
Volume3
Issue number6
DOIs
StatePublished - Dec 1 1993

Keywords

  • Becker dystrophy
  • Duchenne dystrophy
  • High-energy phosphates
  • P MR spectroscopy
  • Principal component analysis

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

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