title: A new noninvasive test to detect mitochondrial dysfunction of skeletal muscles in progressive supranuclear palsy.
abstract: 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.
authors.affiliation: Department of Neurology, Kaohsiung Chang-Gung Memorial Hospital, Ta-Pei Road, Niao-Sung, Kaohsiung 833, Taiwan.
authors.forename: Yung-Yee
authors.initials: YY
authors.lastname: Chang
authors.forename: Chiang-Hsuan
authors.initials: CH
authors.lastname: Lee
authors.forename: Min-Yu
authors.initials: MY
authors.lastname: Lan
authors.forename: Hsiu-Shan
authors.initials: HS
authors.lastname: Wu
authors.forename: Chiung-Chih
authors.initials: CC
authors.lastname: Chang
authors.forename: Jia-Shou
authors.initials: JS
authors.lastname: Liu
mesh_terms: D000328:Adult; D000368:Aged; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D028361:Mitochondrial Diseases / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging / Q000503:physiopathology*; D013494:Supranuclear Palsy, Progressive / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology*; D015899:Tomography, Emission-Computed, Single-Photon / Q000379:methods*