Fractional anisotropy for assessment of white matter tracts injury in methylmalonic acidemia.
BACKGROUND
Methylmalonic acidemia (MMA) is a multifactorial autosomal recessive inborn error of organic acid metabolism, often presenting with neurological symptoms. As neurological disorders are often related to white matter injury, diffusion tensor imaging (DTI) is an excellent tool for assessment of white matter injury and possibly for diagnosing this disorder.


METHODS
We retrospectively analyzed DTI images of 12 patients with MMA (7 males, 5 females, age range: 7 - 12 months, mean age: 9.25 +/- 1.70 months) with negative MRI findings. And another 12 age-matched and gender-matched infants were enrolled as control subjects. Fractional anisotropy (FA) of different white matter tracts of the brain was measured in both groups.


RESULTS
For patients with negative MRI findings, compared with healthy infants, a statistically significant reduction in DTI FA value of the frontal white matter, temporal white matter, and occipital white matter was observed (P < 0.01).


CONCLUSIONS
In addition to conventional T1W and T2W MR Image, Brain DTI presents a useful, sensitive and complementary tool for the assessment of brain damage in patients with MMA.
Gao
Yu
Y
Department of Radiology, Xinhua Hospital, Medical School of Shanghai Jiao Tong University, Shanghai 200092, China. gaoandyu@hotmail.com
Guan
Wen-ye
WY
Wang
Jiang
J
Zhang
Yu-zhen
YZ
Li
Yu-hua
YH
Han
Lian-shu
LS
D000592:Amino Acid Metabolism, Inborn Errors / Q000097:blood / Q000473:pathology*; D016880:Anisotropy; D001921:Brain / Q000473:pathology*; D038524:Diffusion Magnetic Resonance Imaging / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008764:Methylmalonic Acid / Q000097:blood*; D009413:Nerve Fibers, Myelinated / Q000473:pathology*
D008764:Methylmalonic Acid