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Cited by 59

The DOI matches its Crossref record; 43 of the 43 references it cites were found in a registry or an index.

MRI and proton MRSI in women heterozygous for X-linked adrenoleukodystrophy

Fatemi, A., Barker, P.B., Uluğ, A.M., Nagae-Poetscher, L.M., Beauchamp, N.J., Moser, A.B., Raymond, G.V., Moser, H.W., Naidu, S.·2003·Neurology
DOI 10.1212/01.wnl.0000059546.15529.cb
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Abstract

Objective To utilize neuroimaging procedures to assess the extent of cerebral involvement in female subjects heterozygous for X-linked adrenoleukodystrophy (X-ALD). Methods Brain MRI studies were performed in 76 female subjects heterozygous for X-ALD (mean age 43 years, range 8 to 75 years). Sixty-five had clinical evidence of spinal cord involvement resembling that in males with adrenomyeloneuropathy (AMN), two had clinical evidence of cerebral involvement, and nine showed no neurologic abnormality. Readers blinded to clinical findings further analyzed abnormal MRI studies. In eight women whose MRI results were normal, four-slice long echo time MRS imaging (MRSI) studies were performed and compared to those of eight age-matched controls. Results MRI results were normal in 65 subjects and abnormal in 11. In eight of the latter group, the MRI changes were judged to be due to causes other than X-ALD. Lesions were attributed to X-ALD in the remaining three. Two of these patients had lesions that resembled those in male patients with cerebral X-ALD. In one patient with a mild AMN-like syndrome, brain MRI abnormalities were confined to the corticospinal tract. When compared to those of controls, MRSI studies in eight female patients with normal results on brain MRI showed a significant reduction of N-acetylaspartate/creatine and N-acetylaspartate/choline ratios in the internal capsule and corticospinal projection fibers. The N-acetylaspartate/choline ratio was significantly reduce…

Overview

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Crossref · 10.1212/01.wnl.0000059546.15529.cb

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Citations

Cites

Show all 43 references

Research works printed but not linked: 4

  1. Moser HW SK Watkins PA Powers J Moser AB. X-linked adrenoleukodystrophy. In: Scriver CR Beaudet AL Valle D Sly WS eds. The metabolic basis of inherited disease. 8 ed. New York NY: McGraw-Hill 2001; 3257–3302.
  2. Menage P, Carreau V, Tourbah A, et al. [Symptomatic heterozygotic adrenoleukodystrophy in adults. 10 cases.] Rev Neurol . 1993; 149: 445–454.
  3. Naidu S, Washington C, Thirumala S, Watkins PA, Moser HW, Smith K. X-chromosome inactivation in symptomatic heterozygotes in X-linked adrenoleukodystrophy. Ann Neurol . 1997; 42: 498.
  4. Rogers LSN, Weiner MW. Gender effects on NAA in brain gray and white matter received by mixed effects statistical analysis. Proc Intl Soc Magn Reson Med . 2002; 10: 229.

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Metadata

Authors
Fatemi, A., Barker, P. B., Uluğ, A. M., Nagae-Poetscher, L. M., Beauchamp, N. J., Moser, A. B., Raymond, G. V., Moser, H. W., & Naidu, S.
Affiliations
From the Kennedy Krieger Institute (Drs. Fatemi, Barker, H.W. Moser, Raymond, Naidu, A.B. Moser and Nagae-Poetscher) and Department of Radiology and Radiological Science (Drs. Barker, Nagae-Poetscher, and Beauchamp), Johns Hopkins Medical Institutions, Baltimore, MD; and Department of Radiology (Dr. Uluğ), Weill Medical College of Cornell University–NY Presbyterian Hospital.
Venue
Neurology
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2003-04-22
Volume
60
Issue
8
Pages
1301-1307
ISSN
  • 0028-3878
  • 1526-632X
DOI
10.1212/01.wnl.0000059546.15529.cb
Cited by
59
References
43
Language
en

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CoChat. (2026). Citation check: MRI and proton MRSI in women heterozygous for X-linked adrenoleukodystrophy. https://app.cochat.ai/papers/10.1212/01.wnl.0000059546.15529.cb
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Fatemi, A., Barker, P. B., Uluğ, A. M., Nagae-Poetscher, L. M., Beauchamp, N. J., Moser, A. B., Raymond, G. V., Moser, H. W., & Naidu, S. (2003). MRI and proton MRSI in women heterozygous for X-linked adrenoleukodystrophy. Neurology. https://doi.org/10.1212/01.wnl.0000059546.15529.cb

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