RT Journal Article
SR Electronic
T1 Tract-Based Spatial Statistical Analysis of Diffusion Tensor Imaging in Pediatric Patients with Mitochondrial Disease: Widespread Reduction in Fractional Anisotropy of White Matter Tracts
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1726
OP 1730
DO 10.3174/ajnr.A3045
VO 33
IS 9
A1 G.E. Ishak
A1 A.V. Poliakov
A1 S.L. Poliachik
A1 R.P. Saneto
A1 E.J. Novotny, Jr
A1 S. McDaniel
A1 J.G. Ojemann
A1 D.W.W. Shaw
A1 S.D. Friedman
YR 2012
UL http://www.ajnr.org/content/33/9/1726.abstract
AB BACKGROUND AND PURPOSE: Often diagnosed at birth or in early childhood, mitochondrial disease presents with a variety of clinical symptoms, particularly in organs and tissues that require high energetic demand such as brain, heart, liver, and skeletal muscles. In a group of pediatric patients identified as having complex I or I/III deficits on muscle biopsy but with white matter tissue appearing qualitatively normal for age, we hypothesized that quantitative DTI analyses might unmask disturbance in microstructural integrity. MATERIALS AND METHODS: In a retrospective study, DTI and structural MR brain imaging data from 10 pediatric patients with confirmed mitochondrial disease and 10 clinical control subjects were matched for age, sex, scanning parameters, and date of examination. Paired TBSS was performed to evaluate differences in FA, MD, and the separate diffusion direction terms (λr and λa). RESULTS: In patients with mitochondrial disease, significant widespread reductions in FA values were shown in white matter tracts. Mean diffusivity values were significantly increased in patients, having a sparser distribution of affected regions compared with FA. Separate diffusion maps showed significant increase in λr and no significant changes in λa. CONCLUSIONS: Despite qualitatively normal-appearing white matter tissues, patients with complex I or I/III deficiency have widespread microstructural changes measurable with quantitative DTI. FAfractional anisotropyFSLFMRIB software libraryλaaxial diffusivityλrradial diffusivityMDmean diffusivityMELASmitochondrial encephalopathy, lactic acidosis, and strokelike episodesMNIMontreal Neurological InstituteTBSStract-based spatial statistics