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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 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.
D W Zochodne, and
W J Koopman, and
N J Witt, and
T Thompson, and
A A Driedger, and
D Gravelle, and
C F Bolton
December 2022,
Medecine sciences : M/S,
D W Zochodne, and
W J Koopman, and
N J Witt, and
T Thompson, and
A A Driedger, and
D Gravelle, and
C F Bolton
April 1982,
British medical journal (Clinical research ed.),
D W Zochodne, and
W J Koopman, and
N J Witt, and
T Thompson, and
A A Driedger, and
D Gravelle, and
C F Bolton
May 1981,
Archives of neurology,
D W Zochodne, and
W J Koopman, and
N J Witt, and
T Thompson, and
A A Driedger, and
D Gravelle, and
C F Bolton
May 1995,
Lancet (London, England),
D W Zochodne, and
W J Koopman, and
N J Witt, and
T Thompson, and
A A Driedger, and
D Gravelle, and
C F Bolton
November 1989,
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques,
D W Zochodne, and
W J Koopman, and
N J Witt, and
T Thompson, and
A A Driedger, and
D Gravelle, and
C F Bolton
July 2004,
Analytical biochemistry,
D W Zochodne, and
W J Koopman, and
N J Witt, and
T Thompson, and
A A Driedger, and
D Gravelle, and
C F Bolton
November 2011,
Muscle & nerve,
D W Zochodne, and
W J Koopman, and
N J Witt, and
T Thompson, and
A A Driedger, and
D Gravelle, and
C F Bolton
October 2000,
Arthritis and rheumatism,
D W Zochodne, and
W J Koopman, and
N J Witt, and
T Thompson, and
A A Driedger, and
D Gravelle, and
C F Bolton
June 1974,
American journal of ophthalmology,
D W Zochodne, and
W J Koopman, and
N J Witt, and
T Thompson, and
A A Driedger, and
D Gravelle, and
C F Bolton
January 1963,
The New England journal of medicine,
