phosphocreatine and Spinal-Muscular-Atrophies-of-Childhood

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.

Topics: Adenosine Triphosphate; Adolescent; Adult; Child; Child, Preschool; Dystonia; Glycerylphosphorylcholine; Humans; Leg; Magnetic Resonance Spectroscopy; Middle Aged; Muscles; Muscular Dystrophies; Neuromuscular Diseases; Phosphates; Phosphocreatine; Poliomyelitis; Spinal Muscular Atrophies of Childhood