Positron emission tomography in myotonic dystrophy.
Regional cerebral glucose metabolism (rCMRG1) was studied in three patients with myotonic dystrophy by positron emission tomography with 18F-2-fluoro-2-deoxy-D-glucose as the radiotracer. Clinically, all patients presented an organic personality syndrome. Impairment of rCMRG1 was found in all cortical and subcortical regions, particularly in the frontal cortex and the lentiform nucleus. The results suggest that the organic personality changes that occur in myotonic dystrophy reflect widespread cerebral pathology. The metabolic pattern was similar to that seen in normal aging but was qualitatively more severe and is therefore consistent with the classification of myotonic dystrophy as a progeric disease.
Max-Planck-Institut für Neurologische Forschung, Köln, Germany.
R
R
Mielke
K
K
Herholz
G
G
Fink
D
D
Ritter
W D
WD
Heiss
D000328:Adult; D001479:Basal Ganglia / Q000000981:diagnostic imaging / Q000378:metabolism; D001921:Brain / Q000000981:diagnostic imaging / Q000378:metabolism*; D002560:Cerebrovascular Circulation; D003847:Deoxyglucose / Q000031:analogs & derivatives; D005260:Female; D019788:Fluorodeoxyglucose F18; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000000981:diagnostic imaging* / Q000235:genetics / Q000378:metabolism; D014055:Tomography, Emission-Computed
D019788:Fluorodeoxyglucose F18; D003847:Deoxyglucose; D005947:Glucose