Aromatic L-Amino Acid Decarboxylase deficiency studied with [18F]fluorodopa PET

• Main Authors: Tung-Ming Chang, 張通銘
• Other Authors: 鄭瑞棠
• Format: Others
• Language: zh-TW
• Published: 2009
• Online Access: http://ndltd.ncl.edu.tw/handle/74269426133424710311

碩士 === 長榮大學 === 醫學研究所 === 97 === Objective: Although the symptomatology of aromatic L-Amino acid decarboxylase (AADC) deficiency has been well documented, the cerebral pathogenesis of this disorder is still unknown. Methods: We used [18F]fluorodopa (FDOPA) PET to compare the presynaptic dopamine synthesis capacity in the striatum of six AADC deficient children and five non-AADC deficient children. Caudate and putamen FDOPA PET uptake values were measured and expressed as striatal-to-occipital ratio (SOR) and ratio R defined as SOR-1. Results: A conventional region of interest (ROI) analysis revealed that FDOPA uptake (SOR) in the caudate and putamen was significantly lower in the AADC deficient group than in the control group. The mean SOR of FDOPA uptake value in the caudate nucleus was 51% (p<0.005) and in the putamen 54% (p<0.005) of the control mean values.( 20% and 19% for ratio R, respectively ) Conclusions: We confirm the presynaptic nigrostriatal dopaminergic hypofunction in AADC deficient children which results in lower levels of biogenic amine neurotransmitters and associated neurological symptoms. This work further has demonstrated that FDOPA PET may serve as a method to investigate the cerebral dopaminergic abnormality in AADC deficient children.