Table 1

 Specific striatal 123I-FP-CIT binding: clinical data and biochemical data in skeletal muscle

PatientAge (years)Clinical diagnosisHeteroplasmy of mtDNA mutationCI/CSCOX/CSNPDSpecific striatal 123I-FP-CIT binding
Control values (n = 21; mean (SD)): CI/CS = 0.11 (0.03); COX/CS = 0.64 (0.18).
CI, complex I; COX, cytochrome c oxidase; CPEO, chronic progressive external ophthalmoplegia; CS, citrate synthase; KSS, Kearns-Sayre syndrome; MELAS, mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes; mtDNA, mitochondrial DNA; NPD, neuropsychological deficit.
135CPEO plus16% deletion0.0880.26No3.59
240KSS78% deletion0.0040.05Yes3.57
340KSS62% deletion0.0170.08Yes2.87
443CPEO plus26% deletion0.0240.12Yes3.22
546MELAS75% A3243G point mutation0.0680.43Yes3.52
651CPEO plus53% deletion0.0050.10Yes3.09
753CPEO plus58% deletion0.0210.10No2.90
861CPEO plus23% deletion0.0710.33Yes3.09
962CPEO plus52% A3243G point mutation0.0420.17Yes2.83
1069CPEOplus<20%, multiple deletions0.0440.22Yes2.50