X-linked adrenoleukodystrophy: The role of contrast-enhanced MR imaging in predicting disease progression

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

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
Pages (from-to)839-844
Number of pages6
JournalAmerican Journal of Neuroradiology
Volume21
Issue number5
StatePublished - May 25 2000

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Adrenoleukodystrophy
Disease Progression
Nervous System
Neuroimaging
Research Design
Magnetic Resonance Imaging
Brain

All Science Journal Classification (ASJC) codes

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology

Cite this

Melhem, E. R., Loes, D. J., Georgiades, C. S., Raymond, G. V., & Moser, H. W. (2000). X-linked adrenoleukodystrophy: The role of contrast-enhanced MR imaging in predicting disease progression. American Journal of Neuroradiology, 21(5), 839-844.
Melhem, Elias R. ; Loes, Daniel J. ; Georgiades, Christos S. ; Raymond, Gerald V. ; Moser, Hugo W. / X-linked adrenoleukodystrophy : The role of contrast-enhanced MR imaging in predicting disease progression. In: American Journal of Neuroradiology. 2000 ; Vol. 21, No. 5. pp. 839-844.
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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.",
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Melhem, ER, Loes, DJ, Georgiades, CS, Raymond, GV & Moser, HW 2000, 'X-linked adrenoleukodystrophy: The role of contrast-enhanced MR imaging in predicting disease progression', American Journal of Neuroradiology, vol. 21, no. 5, pp. 839-844.

X-linked adrenoleukodystrophy : The role of contrast-enhanced MR imaging in predicting disease progression. / Melhem, Elias R.; Loes, Daniel J.; Georgiades, Christos S.; Raymond, Gerald V.; Moser, Hugo W.

In: American Journal of Neuroradiology, Vol. 21, No. 5, 25.05.2000, p. 839-844.

Research output: Contribution to journalArticle

TY - JOUR

T1 - X-linked adrenoleukodystrophy

T2 - The role of contrast-enhanced MR imaging in predicting disease progression

AU - Melhem, Elias R.

AU - Loes, Daniel J.

AU - Georgiades, Christos S.

AU - Raymond, Gerald V.

AU - Moser, Hugo W.

PY - 2000/5/25

Y1 - 2000/5/25

N2 - 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.

AB - 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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