X-linked Adrenoleukodystrophy: The Role of Contrast-enhanced MR Imaging in Predicting Disease Progression
Elias R. Melhem
,a,
Daniel J. Loesa,
Christos S. Georgiadesa,
Gerald V. Raymonda and
Hugo W. Mosera
a From the Department of Radiology and Radiological Sciences (E.R.M., C.S.G.), The Johns Hopkins Medical Institutions, Baltimore, MD; Suburban Radiologic Consultants, Ltd. (D.J.L.), Minneapolis, MN; and the Department of Neurogenetics (G.V.R., H.W.M.), Kennedy Krieger Institute, The Johns Hopkins Medical Institutions, Baltimore, MD.
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FIG 1. Plots of the Loes scores, based on double-echo spin-echo MR images obtained during different follow-up examinations for 21 patients with ALD. The T1-weighted spin-echo images were contrast-enhanced. There is an increase in the MR score (increased disease burden) on the follow-up images of all except two patients
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FIG 2. X-linked ALD in 43-year-old man with rapidly progressive spasticity, deafness, visual deficits, and personality changes.
A, Initial contrast-enhanced axial T1-weighted MR image (550/24), obtained at the level of the lateral ventricles, shows curvilinear enhancement outlining the periphery of the zone of demyelination (arrowheads).
B, Contiguous axial proton density–weighted MR images (2500/30), obtained at the level of the centrum semiovale at the same time as the image shown in panel A, show very mild white matter abnormalities.
C, Six-month follow-up axial proton density–weighted MR images (3000/30), obtained at a level similar to that shown in panel B, show confluent and symmetrical white matter hyperintensity in both parietal lobes (arrows), confirming the progression of disease.
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FIG 3. Images of a 12-year-old male patient with X-linked ALD with stable neurologic function.
A, Initial contrast-enhanced axial T1-weighted MR image (500/20), obtained at the level of the splenium of the corpus callosum, shows minimal linear enhancement outlining the periphery of the zone of demyelination (arrowheads).
B, Axial T2-weighted MR image (3000/100), obtained at the level of the splenium of the corpus callosum at the same time as the image shown in panel A, shows confluent and symetrical white matter hyperintensity limited the splenium of the corpus callosum and both forceps major (arrows).
C, Thirty-month follow-up axial T2-weighted MR image (3000/100), obtained at a level similar to that shown in panel B, shows no interval change (arrows).
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FIG 4. Plots of the Loes scores, based on double-echo spin-echo MR images, obtained at different follow-up examinations for 22 patients with ALD. The T1-weighted spin-echo images were unenhanced. There is no change in the MR score (no change in disease burden) on the follow-up images of all three patients
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