A new noninvasive test to detect mitochondrial dysfunction of skeletal muscles in progressive supranuclear palsy.

We present usage of technetium-99m methoxyisobutyl isonitrile (99mTc-sestamibi) single photon emission computed tomography (SPECT) as a novel noninvasive method to evaluate muscular mitochondrial function in patients with progressive supranuclear palsy (PSP). 99mTc-sestamibi SPECT revealed a statistically significant decrease in radionucleotide uptake in the quadriceps in PSP patients as compared with other neurodegenerative parkinsonism (P < 0.05) or control group (P < 0.05). This study demonstrates a remarkable deficit of skeletal muscle bioenergetics in patients with PSP. Our findings suggest a distinctive role of mitochondrial dysfunction in the pathogenesis of PSP. Furthermore, 99mTc-sestamibi SPECT provides a relatively simple, inexpensive, and noninvasive modality in further assessment of mitochondrial function and bioenergetic features in various muscular disorders.