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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 21:839-844 (5 2000)
© 2000 American Society of Neuroradiology

ARTICLE

X-linked Adrenoleukodystrophy: The Role of Contrast-enhanced MR Imaging in Predicting Disease Progression

Elias R. Melhem^,a, Daniel J. Loes^a, Christos S. Georgiades^a, Gerald V. Raymond^a and Hugo W. Moser^a

^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.

BACKGROUND AND PURPOSE: Early assignment of disease progression among patients with X-linked adrenoleukodystrophy (ALD) is critical for the appropriate selection of effective therapy. We evaluated the association between contrast enhancement on T1-weighted spin-echo MR images and disease progression.

METHODS: Clinical charts of patients with X-linked ALD were reviewed for age, availability of MR images of the brain, severity of neurologic impairment, and duration and number of follow-up evaluations. Forty-three male patients with X-linked ALD had undergone multiple MR imaging examinations of the brain that consisted of at least sagittal and axial T1-weighted spin-echo, axial double-echo spin-echo, and contrast-enhanced axial T1-weighted spin-echo imaging. The MR images were reviewed for the presence of contrast enhancement. In addition, global disease burden, as shown by the double-echo spin-echo images, was assessed using a visual scoring method (Loes score).

RESULTS: Enhancement was seen on the initial T1-weighted spin-echo MR images of 21 (49%) patients; 18 (86%) of the 21 patients had disease progression revealed by the follow-up evaluations based on MR imaging (Loes) and neurologic scores. No enhancement was seen on the initial T1-weighted spin-echo MR images of 22 (51%) patients; for 18 (82%) of the 22 patients, no evidence of disease progression was revealed by the follow-up evaluations.

CONCLUSION: There is a very strong association between the presence of contrast enhancement on T1-weighted MR images and X-linked ALD progression based on clinical evaluation and MR imaging.

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