Forearm P-31 Nuclear Magnetic Resonance Spectroscopy Studies in Oculopharyngeal Muscular Dystrophy

Douglas W. Zochodne, Wilma J. Koopman, Norbert J. Witt, Terry Thompson, Albert A. Driedger, Dennis Gravelle, Charles F. Bolton
1992 Canadian Journal of Neurological Sciences  
ABSTRACT:Five siblings with autosomal dominant oculopharyngeal muscular dystrophy (OPMD) underwent P-31 Nuclear Magnetic Resonance Spectroscopy studies of forearm flexor muscles. Mean values of PCr/(PCr + Pi) in the patients were reduced (p = 0.01) and pH elevated (p = 0.02) in resting muscle when compared to controls. During exercise PCr/(PCr + Pi) fell quickly to values less than controls (p < 0.0001) despite submaximal exercise output and developed exercise-induced acidosis which exceeded
more » ... s which exceeded that of controls (p = 0.05). Acidosis recovered slowly despite relatively normal recovery of PCr/(PCr + Pi) following exercise. Within the patient group, however, one member had normal resting, exercise and recovery values. The studies suggest that OPMD is a more widespread disorder of striated muscle than clinically appreciated. The pattern of findings observed in OPMD differs from those identified in denervation, disuse and mitochondrial myopathy.
doi:10.1017/s0317167100042220 fatcat:37w4s4anuje27lgetqipokwymi