Published online before print August 30, 2002, 10.1148/radiol.2251011040
Proton MR Spectroscopic and Diffusion Tensor Brain MR Imaging in X-linked Adrenoleukodystrophy: Initial Experience1
Florian S. Eichler, MD,
Ryuta Itoh, MD,
Peter B. Barker, PhD,
Susumu Mori, PhD,
Elizabeth S. Garrett, PhD,
Peter C. M. van Zijl, PhD,
Hugo W. Moser, MD,
Gerald V. Raymond, MD and
Elias R. Melhem, MD
1 From the F. M. Kirby Research Ctr for Functional Brain Imaging, Johns Hopkins University, Baltimore, Md (F.S.E., R.I., P.B.B., E.R.M., S.M., P.C.M.V.Z.); the Dept of Neurogenetics, Kennedy Krieger Institute, Baltimore, Md (F.S.E., H.W.M., G.V.R.); and the Dept of Radiology (R.I., P.B.B., E.R.M., S.M., P.C.M.V.Z.) and Oncology Center, Division of Biostatistics (E.S.G.), Johns Hopkins Univ School of Medicine, Baltimore, Md. Received Jun 13, 2001; revision requested Aug 6; final revision received March 12, 2002; accepted Apr 15. Supported in part by grants RR 00052 and HD 10981 from the U.S. Public Health Service. Address correspondence to E.R.M., Dept of Radiology, Hospital of the Univ of Pennsylvania, 3400 Spruce St, Ground Floor Founders Building, Philadelphia, PA 19104 (e-mail: emelhem@rad.upenn.edu).

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Figure 1. MR spectroscopic images show metabolite concentrations (NAA, creatine [Cr], choline [Cho]) and IADC (ADCi) and FA values in a column of white matter voxels in the centrum semiovale of the 11 healthy control subjects (left) and the six asymptomatic patients with Addison-only X-ALD (right) as percentages of mean concentration in the healthy subjects. No consistent regional variation is apparent.
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Figure 2. Patient 7. MR spectroscopic images show metabolite concentrations (NAA, creatine [Cr], choline [Cho]) and FA and IADC (ADCi) values (as percentages of mean concentration in healthy control subjects) in a 7-year-old boy with CCALD who had gait abnormalities and right-sided hemiparesis. Transverse T2-weighted conventional MR images (3,000/30-100) revealed an extensive white matter lesion in the parieto-occipital region that was predominantly right sided. Changes in metabolite (NAA, creatine, choline) concentrations occur in NAWM distant to the lesion and are more drastic than changes in quantitative measures of diffusion and anisotropy (IADC, FA).
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Figure 3. Graph describes the white matter of patients with X-ALD: correlation of NAA concentration (mmol/L) with IADC (mm2/sec), r = -0.69 (P < .001).
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Figure 4. Graph describes the white matter of patients with X-ALD: correlation of NAA concentration (mmol/L) with FA, r = 0.64 (P < .001).
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Figure 5. Graph describes the white matter of patients with X-ALD: correlation of choline concentration (mmol/L) with IADC (mm2/sec). In AWM, r = -0.36 (P < .007). In NAWM, r = 0.27 (P = .14).
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Figure 6. Graph describes the white matter of patients with X-ALD: correlation of choline concentration (mmol/L) with FA. In AWM, r = 0.20 (P = .006). In NAWM, r = -0.28 (P = .28).
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Figure 7. Graph describes the white matter of patients with X-ALD: correlation of creatine concentration (mmol/L) with IADC (mm2/sec). In AWM, r = -0.55 (P < .001). In NAWM, r = -0.20 (P = .57).
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Figure 8. Graph describes the white matter of patients with X-ALD: correlation of creatine concentration (mmol/L) with FA. In AWM, r = 0.56 (P = .06). In NAWM, r = 0.08 (P = .66).
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Copyright © 2002 by the Radiological Society of North America.