Brain metabolic impairment in non-cerebral and cerebral forms of x-linked adrenoleukodystrophy by proton MRS: Identification of metabolic patterns by discriminant analysis

Cerebral metabolism in six children with X‐linked adrenoleukodystrophy (X‐ALD) was studied using 1H magnetic resonance spectroscopy (MRS), and the status of the patients was monitored for evaluating disease progression. Spectra were abnormal even in patients with no cerebral impairment. Four differe...

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Veröffentlicht in:Magnetic resonance in medicine 1999-06, Vol.41 (6), p.1119-1126
Hauptverfasser: Salvan, Anne-Marie, Confort-Gouny, Sylviane, Chabrol, Brigitte, Cozzone, Patrick J., Vion-Dury, Jean
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container_end_page 1126
container_issue 6
container_start_page 1119
container_title Magnetic resonance in medicine
container_volume 41
creator Salvan, Anne-Marie
Confort-Gouny, Sylviane
Chabrol, Brigitte
Cozzone, Patrick J.
Vion-Dury, Jean
description Cerebral metabolism in six children with X‐linked adrenoleukodystrophy (X‐ALD) was studied using 1H magnetic resonance spectroscopy (MRS), and the status of the patients was monitored for evaluating disease progression. Spectra were abnormal even in patients with no cerebral impairment. Four different metabolic patterns were identified, and a metabolic classification of the disease was proposed, from grade 0 to grade III. The evolution of the disease toward grade II appears to be systematic, but many patients did not evolve from this grade to grade III, which is the metabolic mark of severe progressive forms. Metabolic data of X‐ALD were processed using discriminant analysis, which provides a classification accuracy of 95.2%. Proton cerebral MRS together with discriminant analysis may be useful during the follow‐up in X‐ALD for monitoring the evolution of the disease and the effects of therapy. Magn Reson Med 41:1119–1126, 1999. © 1999 Wiley‐Liss, Inc.
doi_str_mv 10.1002/(SICI)1522-2594(199906)41:6<1119::AID-MRM7>3.0.CO;2-F
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Reson. Med</addtitle><description>Cerebral metabolism in six children with X‐linked adrenoleukodystrophy (X‐ALD) was studied using 1H magnetic resonance spectroscopy (MRS), and the status of the patients was monitored for evaluating disease progression. Spectra were abnormal even in patients with no cerebral impairment. Four different metabolic patterns were identified, and a metabolic classification of the disease was proposed, from grade 0 to grade III. The evolution of the disease toward grade II appears to be systematic, but many patients did not evolve from this grade to grade III, which is the metabolic mark of severe progressive forms. Metabolic data of X‐ALD were processed using discriminant analysis, which provides a classification accuracy of 95.2%. Proton cerebral MRS together with discriminant analysis may be useful during the follow‐up in X‐ALD for monitoring the evolution of the disease and the effects of therapy. 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subjects Adolescent
Adrenoleukodystrophy - diagnosis
Adrenoleukodystrophy - genetics
Adrenoleukodystrophy - metabolism
Biological and medical sciences
Brain - metabolism
brain metabolic grading
Carbohydrates (enzymatic deficiencies). Glycogenosis
Case-Control Studies
Child
Child, Preschool
Discriminant Analysis
Errors of metabolism
Genetic Linkage
Humans
Magnetic Resonance Spectroscopy
Male
Medical sciences
Metabolic diseases
proton magnetic resonance spectroscopy
X Chromosome
X-ALD
title Brain metabolic impairment in non-cerebral and cerebral forms of x-linked adrenoleukodystrophy by proton MRS: Identification of metabolic patterns by discriminant analysis
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