Human leg neuromuscular diseases: P-31 MR spectroscopy.
Phosphorus-31 magnetic resonance (MR) spectra of leg muscles in patients with the neuromuscular diseases Duchenne dystrophy, myotonic dystrophy, postpoliomyelitis, Werdnig-Hoffmann disease, and pedal dystonia were recorded. Ratios of beta-adenosine triphosphate (ATP), inorganic phosphate (Pi), alpha-glycerophosphorylcholine (GPC), and phosphomonoesters to phosphocreatine (PCr) were calculated from peak integrals and compared with normal muscle ratios. In all diseases studied, beta-ATP/PCr and Pi/PCr values showed an increase from normal values. The extent of increase in beta-ATP/PCr was related to the clinical severity of the disease, suggesting that this could be a useful noninvasive means of monitoring effectiveness of therapy for neuromuscular disorders. In myotonic dystrophy and Werdnig-Hoffmann disease, GPC/PCr values increased greatly. The intracellular pH in Duchenne and postpoliomyelitis muscles was slightly elevated compared with that in normal muscles. Hydrogen-1 MR images of muscles showed fat infiltration in all patients, more in weaker muscles and less in stronger muscles.
Bárány
M
M
Department of Biological Chemistry, College of Medicine, University of Illinois, Chicago 60612.
Siegel
I M
IM
Venkatasubramanian
P N
PN
Mok
E
E
Wilbur
A C
AC
D000255:Adenosine Triphosphate / Q000378:metabolism; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004421:Dystonia / Q000378:metabolism; D005997:Glycerylphosphorylcholine / Q000378:metabolism; D006801:Humans; D007866:Leg; D009682:Magnetic Resonance Spectroscopy; D008875:Middle Aged; D009132:Muscles / Q000378:metabolism; D009136:Muscular Dystrophies / Q000378:metabolism; D009468:Neuromuscular Diseases / Q000378:metabolism*; D010710:Phosphates / Q000378:metabolism; D010725:Phosphocreatine / Q000378:metabolism; D011051:Poliomyelitis / Q000150:complications / Q000378:metabolism; D014897:Spinal Muscular Atrophies of Childhood / Q000378:metabolism
D010710:Phosphates; D010725:Phosphocreatine; D005997:Glycerylphosphorylcholine; D000255:Adenosine Triphosphate