Lack of dystrophin, a protein localized to the inner surface of the sarcolemma of the muscle fiber, is the cause of Duchenne type muscular dystrophy. Plasma membrane damage of the muscular fiber occurs, followed by Ca++ influx into the fibers. There is severe mitochondrial damage in dystrophic but still viable fibers. Five children aged 5-7 years were studied with MRI, Tl-201, and Tc-99m sestamibi scintigraphy of the thighs. These three methods showed that the sartorius is the least damaged muscle in Duchenne type muscular dystrophy. MRI showed mild damage of adductors and quadriceps; Tl-201 scintigraphy showed a marked reduction of radioactivity in the same muscles; Tc-99m sestamibi uptake occurred only in the sartorius muscle; the quadriceps was not imaged and adductors showed a faint image. A decrease of water in muscular fibers as well as fatty fibrous substitution, occurs after death of the fibers, whereas plasma membrane and mitochondrial damage reduced the uptake of tracers when the fiber is still viable. The interesting mismatch between sestamibi and Tl-201 can be explained by considering that the cellular mechanism of uptake and retention of Tc-99m sestamibi involves both plasma membrane and mitochondria, whereas the uptake of Tl-201 is only affected by plasma membrane damage.
ASJC Scopus subject areas
- Radiology Nuclear Medicine and imaging
- Radiological and Ultrasound Technology