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

Cerebral metabolism in six children with X-linked adrenoleukodystrophy (X-A LD) was studied using H-1 magnetic resonance spectroscopy (MRS), and the st atus of the patients was monitored for evaluating disease progression. Spec tra were abnormal even in patients with no cerebral impairment. Four differ ent metabolic patterns were identified, and a metabolic classification of t he disease was proposed, from grade 0 to grade ill, The evolution of the di sease toward grade II appears to be systematic, but many patients did not e volve from this grade to grade ill, which is the metabolic mark of severe p rogressive forms. Metabolic data of X-ALD were processed using discriminant analysis, which provides a classification accuracy of 95.2%, Proton cerebr al 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. (C) 1999 Wiley-Liss, Inc.