Forearm P-31 nuclear magnetic resonance spectroscopy studies in oculopharyngeal muscular dystrophy.

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 less than 0.0001) despite submaximal exercise output and developed exercise-induced acidosis 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.

• Authors: D W Zochodne, W J Koopman, N J Witt, T Thompson, A A Driedger, D Gravelle, C F Bolton
• Journal: The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques (Can J Neurol Sci) Vol. 19 Issue 2 Pg. 174-9 (May 1992) ISSN: 0317-1671 [Print] England
• PMID: 1623442 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Phosphocreatine

Topics

• Acidosis, Lactic (metabolism)
• Aged
• Exercise Test
• Female
• Forearm (physiology)
• Humans
• Magnetic Resonance Spectroscopy
• Male
• Middle Aged
• Muscular Dystrophies (metabolism)
• Oculomotor Muscles (metabolism)
• Pharyngeal Muscles (metabolism)
• Phosphocreatine (metabolism)