| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
From the F.M. Kirby Research Center for Functional Brain Imaging (Drs. Eichler, Barker, Cox, Edwin, Moser, and Raymond), Kennedy Krieger Institute, Johns Hopkins University, and Department of Radiology (Dr. Barker), Johns Hopkins University, Baltimore, MD; and Department of Radiology (Dr. Ulug), Weill Medical College of Cornell University, New York, NY.
Address correspondence and reprint requests to Dr. G.V. Raymond, Kennedy Krieger Institute, 707 North Broadway, Baltimore, MD 21205; e-mail: raymond{at}kennedykrieger.org
Background: The phenotypic expression of X-linked adrenoleukodystrophy (X-ALD) ranges from the rapidly progressive childhood cerebral form to the milder adrenomyeloneuropathy in adults. It is not possible to predict phenotype by mutation analysis or biochemical assays. Multislice proton MRS imaging (MRSI) has previously detected more extensive brain abnormalities in X-ALD than conventional MRI, which has been suggested to predict impending demyelination. However, the significance of these changes is unclear.
Objective: The purpose of this study was to determine the long-term sensitivity and specificity of MRSI for disease progression in X-ALD.
Methods: Twenty-five patients with X-ALD were investigated (average age, 15 years; range, 2–43 years) with MRI and proton MRSI at baseline and follow-up MRI over a mean period of 3.5 years. Eight patients had normal MRI findings at baseline and on follow-up (noncerebral group), 11 had abnormal MRI at baseline and no change on follow-up (cerebral nonprogressive group), and 6 had progressive MRI abnormalities (cerebral progressive group). On MRSI, voxels were analyzed in the normal MRI–appearing perilesional white matter, or in the corresponding area in the noncerebral group.
Results: The concentration ratio of N-acetylaspartate (NAA) to choline was the most sensitive indicator of disease progression. The average NAA/choline ratio was 5.99 for the noncerebral group, 5.75 for the cerebral nonprogressive group, and 3.74 for the cerebral progressive group (p = 0.002). At a cut-off point of 5.0, the NAA/choline ratio predicted disease progression in all patients with six cerebral progressive disease (sensitivity 100%). The specificity was 83%, the positive predictive value was 66%, and the negative predictive value was 100%.
Conclusions: Multislice proton MRS imaging is able to identify impending or beginning degeneration in white matter that still appears normal on conventional MRI. Multislice proton MRSI may be a suitable technique for the prediction of lesion progression on MRI in X-linked adrenoleukodystrophy.
This article has been cited by other articles:
|
|
 |
|
  S. Marino, M. De Luca, M. T. Dotti, M. L. Stromillo, P. Formichi, P. Galluzzi, M. Mondelli, P. Bramanti, A. Federico, and N. De Stefano Prominent brain axonal damage and functional reorganization in "pure" adrenomyeloneuropathy Neurology, September 18, 2007; 69(12): 1261 - 1269. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 F. Eichler, A. Mahmood, D. Loes, L. Bezman, D. Lin, H. W. Moser, and G. V. Raymond Magnetic Resonance Imaging Detection of Lesion Progression in Adult Patients With X-linked Adrenoleukodystrophy Arch Neurol, May 1, 2007; 64(5): 659 - 664. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. Goebell, J. Fiehler, X.-Q. Ding, S. Paustenbach, S. Nietz, O. Heese, T. Kucinski, C. Hagel, M. Westphal, and H. Zeumer Disarrangement of Fiber Tracts and Decline of Neuronal Density Correlate in Glioma Patients--A Combined Diffusion Tensor Imaging and 1H-MR Spectroscopy Study AJNR Am. J. Neuroradiol., August 1, 2006; 27(7): 1426 - 1431. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Fatemi, S. A. Smith, P. Dubey, K. M. Zackowski, A. J. Bastian, P. C. van Zijl, H. W. Moser, G. V. Raymond, and X. Golay Magnetization transfer MRI demonstrates spinal cord abnormalities in adrenomyeloneuropathy Neurology, May 24, 2005; 64(10): 1739 - 1745. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. W. Moser and P. B. Barker Magnetic resonance spectroscopy: A new guide for the therapy of adrenoleukodystrophy Neurology, February 8, 2005; 64(3): 406 - 407. [Full Text] [PDF]
|
|
|
|
|
|
G. Oz, I. Tkac, L. R. Charnas, I. Y. Choi, K. J. Bjoraker, E. G. Shapiro, and R. Gruetter Assessment of adrenoleukodystrophy lesions by high field MRS in non-sedated pediatric patients Neurology, February 8, 2005; 64(3): 434 - 441. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. J. Loes, A. Fatemi, E. R. Melhem, N. Gupte, L. Bezman, H. W. Moser, and G. V. Raymond Analysis of MRI patterns aids prediction of progression in X-linked adrenoleukodystrophy Neurology, August 12, 2003; 61(3): 369 - 374. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. F. L. Schneider, K. A. Il'yasov, E. Boltshauser, J. Hennig, and E. Martin Diffusion Tensor Imaging in Cases of Adrenoleukodystrophy: Preliminary Experience as a Marker for Early Demyelination? AJNR Am. J. Neuroradiol., May 1, 2003; 24(5): 819 - 824. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Fatemi, P.B. Barker, A.M. Ulug, L.M. Nagae-Poetscher, N.J. Beauchamp, A.B. Moser, G.V. Raymond, H.W. Moser, and S. Naidu MRI and proton MRSI in women heterozygous for X-linked adrenoleukodystrophy Neurology, April 22, 2003; 60(8): 1301 - 1307. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
