Lack of dystrophin, a protein localized to the inner surface of the sa
rcolemma of the muscle fiber, is the cause of Duchenne type muscular d
ystrophy, Plasma membrane damage of the muscular fiber occurs, followe
d by Ca++ influx into the fibers, There is severe mitochondrial damage
in dystrophic but still viable fibers, Five children aged 5-7 years w
ere studied with MRI, TI-201, and Tc-99m sestamibi scintigraphy of the
thighs, These three methods showed that the sartorius is the least da
maged muscle in Duchenne type muscular dystrophy, MRI showed mild dama
ge of adductors and quadriceps; TI-201 scintigraphy showed a marked re
duction of radioactivity in the same muscles; Tc-99m sestamibi uptake
occurred only in the sartorius muscle; the quadriceps was not imaged a
nd adductors showed a faint image, A decrease of water in muscular fib
ers as well as fatty fibrous substitution, occurs after death of the f
ibers, whereas plasma membrane and mitochondria[ damage reduced the up
take of tracers when the fiber is still viable. The interesting mismat
ch between sestamibi and TI-201 can be explained by considering that t
he cellular mechanism of uptake and retention of Tc-99m sestamibi invo
lves both plasma membrane and mitochondria, whereas the uptake of TI-2
01 is only affected by plasma membrane damage.