Gastric hypomotility in Duchenne's muscular dystrophy.
In Duchenne's muscular dystrophy, functional impairment of smooth muscle in the gastrointestinal tract can cause acute gastric dilatation and intestinal pseudo-obstruction that may be fatal. We describe a patient with this syndrome who at autopsy had smooth-muscle degeneration of the stomach. To provide objective evidence of functional smooth-muscle impairment in Duchenne's dystrophy, we performed gastric-emptying studies in 11 patients and 11 normal controls, using technetium-99m radionuclide scintigraphy in a test meal of oatmeal. The patients with Duchenne's dystrophy had delayed gastric-emptying times (118.18 +/- 32.21 minutes [mean +/- SEM]) as compared with controls (42.5 +/- 3.4 minutes, P less than 0.01). The cause of the pathological and functional abnormalities we describe in smooth muscle is unknown but may be a deficiency of dystrophin, the recently identified gene product of the Duchenne's muscular dystrophy locus.
Barohn
R J
RJ
Department of Neurology, Ohio State University College of Medicine, Columbus 43210.
Levine
E J
EJ
Olson
J O
JO
Mendell
J R
JR
D000293:Adolescent; D000328:Adult; D005260:Female; D013271:Gastric Dilatation / Q000209:etiology; D005746:Gastric Emptying; D006801:Humans; D007415:Intestinal Obstruction / Q000209:etiology; D008297:Male; D009130:Muscle, Smooth / Q000473:pathology; D009136:Muscular Dystrophies / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D013270:Stomach / Q000473:pathology