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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.
Address correspondence and reprint requests to Dr. Gerald V. Raymond, Assoc. Professor of Neurology, Kennedy Krieger Institute, Johns Hopkins Medical Institutions, 707 N. Broadway, Room 500L, Baltimore, MD; e-mail: raymond{at}kennedykrieger.org
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.
Editorial, see page 1677
This study was supported by the General Clinical Research Center of the Johns Hopkins Hospital (M01 RR 00052), the National Institute for Biomedical Imaging and Bioengineering (EB00991-01), and the National Center for Research Resources (P41 RR15241-02).
Conflict of Interest Statement: Dr. van Zijl is a paid lecturer for the Phillips Medical Systems. This arrangement has been approved by Johns Hopkins University in accordance with its Conflict of Interest policies.
Received October 14, 2004. Accepted in final form March 22, 2005.
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  K. M. Zackowski, P. Dubey, G. V. Raymond, S. Mori, A. J. Bastian, and H. W. Moser Sensorimotor Function and Axonal Integrity in Adrenomyeloneuropathy Arch Neurol, January 1, 2006; 63(1): 74 - 80. [Abstract] [Full Text] [PDF]
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 H. W. Moser, G. V. Raymond, and P. Dubey Adrenoleukodystrophy: New Approaches to a Neurodegenerative Disease JAMA, December 28, 2005; 294(24): 3131 - 3134. [Abstract] [Full Text] [PDF]
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 F. Barkhof and R. C. McKinstry Quantifying spinal cord demyelination with magnetic transfer imaging Neurology, May 24, 2005; 64(10): 1677 - 1678. [Full Text] [PDF]
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