Magnetization transfer MRI demonstrates spinal cord abnormalities in adrenomyeloneuropathy
Abstract
BACKGROUND: In adrenomyeloneuropathy (AMN) conventional MRI detects only spinal cord atrophy in the late stages. OBJECTIVE: To apply a magnetization transfer-weighted (MTw) imaging to patients with AMN and AMN-like syndrome in order to visualize and quantitatively assess the pathology of white matter tracts in the cervical spinal cord. METHODS: MTw studies were conducted in nine men with AMN, eight symptomatic heterozygous women, and 10 age- and sex-matched controls and compared to the Expanded Disability Status Scale (EDSS) and quantitative tests of vibratory sense and postural sway. MTw data sets were obtained at the level of C1 to C3 using a three-dimensional gradient echo acquisition technique, these images were then standardized between subjects by using the in-slice CSF signal as a normalization reference, allowing a quantitative assessment of the MTw signal. RESULTS: In contrast to conventional MRI, MTw images showed signal hyperintensities in the lateral and dorsal columns of all patients. The MT signal quantified in the dorsal column showed significant differences between patients with AMN, X-linked adrenoleukodystrophy heterozygotes, and controls. MT hyperintensity in the dorsal column correlated with EDSS, vibratory sense, and postural sway. CONCLUSION: Magnetization transfer-weighted imaging is a sensitive modality for the visual and quantitative assessment of spinal cord pathology in adrenomyeloneuropathy, and is a potential tool for evaluation of new therapies.
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Magnetization transfer or diffusion tensor-based imaging sequences can detect abnormalities in spinal cord tracts in X-ALD.
“Magnetization transfer or diffusion tensor-based imaging sequences can detect abnormalities in spinal cord tracts [65,97,98].”
From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [98]
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Moser, H. W., Mahmood, A., & Raymond, G. V. (2007) · Nature Clinical Practice Neurology · doi:10.1038/ncpneuro0421 · Cited by 663 · see citing works
Self-citationKemp, S., Pujol, A., Waterham, H. R., van Geel, B. M., Boehm, C. D., Raymond, G. V., Cutting, G. R., Wanders, R. J. A., & Moser, H. W. (2001) · Human Mutation · doi:10.1002/humu.1227 · Cited by 332 · see citing works
Self-citationAdrenoleukodystrophy: Incidence, new mutation rate, and results of extended family screening
Bezman, L., Moser, A. B., Raymond, G. V., Rinaldo, P., Watkins, P. A., Smith, K. D., Kass, N. E., & Moser, H. W. (2001) · Annals of Neurology · doi:10.1002/ana.101 · Cited by 324 · see citing works
Self-citationAnalysis of MRI patterns aids prediction of progression in X-linked adrenoleukodystrophy
Loes, D. J., Fatemi, A., Melhem, E. R., Gupte, N., Bezman, L., Moser, H. W., & Raymond, G. V. (2003) · Neurology · doi:10.1212/01.wnl.0000079050.91337.83 · Cited by 268 · see citing works
Self-citationMoser, H. W., Loes, D. J., Melhem, E. R., Raymond, G. V., Bezman, L., Cox, C. S., & Lu, S.-E. (2000) · Neuropediatrics · doi:10.1055/s-2000-9236 · Cited by 210 · see citing works
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Moser, H. W., Mahmood, A., & Raymond, G. V. (2007) · Nature Clinical Practice Neurology · doi:10.1038/ncpneuro0421 · Cited by 663 · see citing works
Self-citationX-linked adrenoleukodystrophy: Clinical, metabolic, genetic and pathophysiological aspects
Kemp, S., Berger, J., & Aubourg, P. (2012) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2012.03.012 · Cited by 283 · see citing works
Adrenoleukodystrophy—Neuroendocrine pathogenesis and redefinition of natural history
Kemp, S., Huffnagel, I. C., Linthorst, G. E., Wanders, R. J., & Engelen, M. (2016) · Nature Reviews Endocrinology · doi:10.1038/nrendo.2016.90 · Cited by 275 · see citing works
Pathophysiology of X-linked adrenoleukodystrophy
Berger, J., Forss-Petter, S., & Eichler, F. S. (2014) · Biochimie · doi:10.1016/j.biochi.2013.11.023 · Cited by 238 · see citing works
Adrenoleukodystrophy: New Approaches to a Neurodegenerative Disease
Moser, H. W., Raymond, G. V., & Dubey, P. (2005) · JAMA · doi:10.1001/jama.294.24.3131 · Cited by 142 · see citing works
Self-citationDiffusion tensor–based imaging reveals occult abnormalities in adrenomyeloneuropathy
Dubey, P., Fatemi, A., Huang, H., Nagae‐Poetscher, L., Wakana, S., Barker, P. B., van Zijl, P., Moser, H. W., Mori, S., & Raymond, G. V. (2005) · Annals of Neurology · doi:10.1002/ana.20643 · Cited by 40 · see citing works
Self-citationRevisiting the Pathogenesis of X-Linked Adrenoleukodystrophy
Bougnères, P., & Le Stunff, C. (2025) · Genes · doi:10.3390/genes16050590 · Cited by 11 · see citing works
- Authors
- Fatemi, A., Smith, S. A., Dubey, P., Zackowski, K. M., Bastian, A. J., van Zijl, P. C., Moser, H. W., Raymond, G. V., & Golay, X.
- Affiliations
- Kennedy Krieger Institute (US)
- From the Neurogenetics Research Center (Drs. Fatemi, Dubey, Moser, and Raymond), the Motion Analysis Laboratory (Drs. Zackowski and Bastian), and the F.M. Kirby Research Center for functional Brain Imaging (Drs. Fatemi, Smith, Dubey, van Zijl, and Golay), the Kennedy Krieger Institute, Departments of Neurology, Radiology and Biophysics and Biophysical Chemistry, Johns Hopkins University, Baltimore, MD.
- Venue
- Neurology
- Published
- 2005-05-24
- Volume
- 64
- Issue
- 10
- Pages
- 1739-1745
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- OpenAlex
- W2100485002
- Cited by
- 64
- References
- 5
- Citations by year
- 2026: 2 · 2025: 4 · 2024: 3 · 2023: 1 · 2022: 1 · 2021: 5 · 2020: 1 · 2019: 1 · 2018: 1 · 2017: 2 · 2016: 4 · 2015: 3 · 2014: 2 · 2013: 3 · 2012: 7
- FWCI
- 4.34
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Neurology (publishedVersion)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Funders
- National Institute of Biomedical Imaging and Bioengineering (R21EB000991, EB00991-01, P41EB015909)
- National Center for Research Resources (M01 RR-00052, P41RR015241, M01RR000052, P41 RR15241-02)
- Topics
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Advanced MRI Techniques and Applications
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
- Keywords
- adrenomyeloneuropathy, cervical spinal cord, Expanded Disability Status Scale, postural sway, spinal cord atrophy
- MeSH terms
- Adrenoleukodystrophy, Adult, Atrophy, Cervical Vertebrae, Female, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Nerve Fibers, Myelinated, Neural Pathways, Predictive Value of Tests, Spinal Cord, Spinal Cord Diseases, Somatosensory Disorders
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