Improved tissue characterization in adrenoleukodystrophy using magnetization transfer imaging. 1996

E R Melhem, and S N Breiter, and A M Ulug, and G V Raymond, and H W Moser
Department of Radiology, Boston University Medical Center, MA 02118, USA.

OBJECTIVE We performed this study to evaluate the usefulness of magnetization transfer (MT) imaging techniques and magnetization transfer ratio (MTR) measurements in assessing patients with adrenoleukodystrophy (ALD). METHODS Twelve patients who encompassed the range of ALD phenotypes were evaluated prospectively with MR and MT imaging (including digital subtraction images). The MT images and spin-echo MR images were compared by two staff neuroradiologists and a staff neurologist for extent of disease, different zones of involvement, and the presence of enhancement. MTR measurements were obtained from affected and unaffected regions of the brain. RESULTS Eight of the 12 patients exhibited gadolinium enhancement. In seven of these patients, the MT subtraction images showed two zones of abnormal signal within the affected white matter. Their MTR measurements were classified as zone 1 (mean MTR, 35%; SD, 6%) and zone 2 (mean MTR, 20%; SD, 6%). Three of the other patients showed a single zone of involvement corresponding to the affected white matter on the standard spin-echo MR images. Their MTR measurements were similar to that of zone 1. The remaining two patients had no demonstrable signal abnormalities on any of the images. We noted no difference in any of the 12 subjects in the extent of white matter involvement on any of the imaging techniques. The MTR measurements in the unaffected white matter (mean MTR, 46%; SD, 5%) approximated established MTRs in white matter of normal volunteers. CONCLUSIONS MT imaging and MTR measurements are helpful clinical techniques that may improve the evaluation of patients with ALD, particularly in the proposed ability of these techniques to divide the affected white matter into different zones that correspond to specific pathologic areas. These techniques can help the understanding of pathogenetic mechanisms and the evaluation of therapies.

UI MeSH Term Description Entries
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D008297 Male Males
D011446 Prospective Studies Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group. Prospective Study,Studies, Prospective,Study, Prospective
D001921 Brain The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM. Encephalon
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000326 Adrenoleukodystrophy An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS). Adrenomyeloneuropathy,Schilder-Addison Complex,X-Linked Adrenoleukodystrophy,ALD (Adrenoleukodystrophy),Addison Disease and Cerebral Sclerosis,Bronze Schilder Disease,Melanodermic Leukodystrophy,Siemerling-Creutzfeldt Disease,X-ALD,X-ALD (X-Linked Adrenoleukodystrophy),Adrenoleukodystrophy, X-Linked,Leukodystrophies, Melanodermic,Leukodystrophy, Melanodermic,Schilder Addison Complex,Siemerling Creutzfeldt Disease,X ALD,X ALD (X Linked Adrenoleukodystrophy),X Linked Adrenoleukodystrophy
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D013382 Subtraction Technique Combination or superimposition of two images for demonstrating differences between them (e.g., radiograph with contrast vs. one without, radionuclide images using different radionuclides, radiograph vs. radionuclide image) and in the preparation of audiovisual materials (e.g., offsetting identical images, coloring of vessels in angiograms). Subtraction Technic,Subtraction Technics,Subtraction Techniques,Technic, Subtraction,Technics, Subtraction,Technique, Subtraction,Techniques, Subtraction

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