title: Brain metabolic impairment in non-cerebral and cerebral forms of X-linked adrenoleukodystrophy by proton MRS: identification of metabolic patterns by discriminant analysis.
abstract: 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.
authors.affiliation: Centre de Résonance Magnétique Biologique et Médicale, UMR CNRS 6612, Faculté de Médecine, Marseille, France.
authors.forename: A M
authors.initials: AM
authors.lastname: Salvan
authors.forename: S
authors.initials: S
authors.lastname: Confort-Gouny
authors.forename: B
authors.initials: B
authors.lastname: Chabrol
authors.forename: P J
authors.initials: PJ
authors.lastname: Cozzone
authors.forename: J
authors.initials: J
authors.lastname: Vion-Dury
mesh_terms: D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism*; D001921:Brain / Q000378:metabolism*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D016002:Discriminant Analysis; D008040:Genetic Linkage; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D014960:X Chromosome