@article {Melhem839, author = {Elias R. Melhem and Daniel J. Loes and Christos S. Georgiades and Gerald V. Raymond and Hugo W. Moser}, title = {X-linked Adrenoleukodystrophy: The Role of Contrast-enhanced MR Imaging in Predicting Disease Progression}, volume = {21}, number = {5}, pages = {839--844}, year = {2000}, publisher = {American Journal of Neuroradiology}, abstract = {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.}, issn = {0195-6108}, URL = {https://www.ajnr.org/content/21/5/839}, eprint = {https://www.ajnr.org/content/21/5/839.full.pdf}, journal = {American Journal of Neuroradiology} }