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From the Service Hospitalier Frédéric Joliot, CEA (Drs. Fiorelli, Mazoyer, Blin, and Samson), Orsay; Cardiologic Division, Hôpital Cochin (Dr. Duboc), Paris; and Clinique des Maladies du Système Nerveux, Hôpital de la Salpêtrière and INSERM U 153 (Drs. Eymard and Fardeau), Paris, France.
To test the hypothesis that cerebral metabolism is altered in myotonic dystrophy (MyD), we investigated cerebral glucose kinetics and utilization in 11 adult patients with MyD and 14 healthy controls, using 18F-labeled 2-fluoro-2-deoxy-D-glucose (FDG) and dynamic positron emission tomography. Estimation of rate constants in MyD revealed a reduction of FDG delivery to the brain. Cortical glucose utilization rate was reduced by about 20% in MyD. These findings may be related to the presence of neurologic impairment in MyD and prompt further investigations on the metabolic and clinical features of brain dysfunction in this disease.
Address correspondence and reprint requests to Dr. Y. Samson, Service Hospitalier Frédéric Joliot, CEA, 4, Place du Général Leclerc, 91406 Orsay, France.
M. Fiorelli was on leave from the Department of Neurological Sciences, University of Rome "La Sapienza," Rome, Italy.
Funded in part by the Association Française contre les Myopathies (AFM).
Presented in part at the 42nd annual meeting of the American Academy of Neurology, Miami Beach, FL, April 1990.
Received October 22, 1990. Accepted for publication in final form June 26, 1991.
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