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

Published online by Cambridge University Press:  18 September 2015

Douglas W. Zochodne ,
Wilma J. Koopman ,
Norbert J. Witt ,
Terry Thompson ,
Albert A. Driedger ,
Dennis Gravelle  and
Charles F. Bolton
Douglas W. Zochodne*
Affiliation:
Lasalle Building, Departments of Medicine and Physiology, Queen’s University, Kingston
Wilma J. Koopman
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, London
Norbert J. Witt
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, London
Terry Thompson
Affiliation:
Department of Radiology and Nuclear Medicine, Victoria Hospital, London
Albert A. Driedger
Affiliation:
Department of Radiology and Nuclear Medicine, Victoria Hospital, London
Dennis Gravelle
Affiliation:
Department of Radiology and Nuclear Medicine, Victoria Hospital, London
Charles F. Bolton
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, London
*
Lasalle Bldg., Room #206, Queen’s University, Kingston, Ontario, Canada K7L 3N6
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Abstract:

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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 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.

Type
Articles
Information
Canadian Journal of Neurological Sciences , Volume 19 , Issue 2 , May 1992 , pp. 174 - 179
Copyright
Copyright © Canadian Neurological Sciences Federation 1992

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