title: Classification of childhood white matter disorders using proton MR spectroscopic imaging.
abstract: OBJECTIVE
  Childhood white matter disorders often show similar MR imaging signal-intensity changes, despite different underlying pathophysiologies. The purpose of this study was to determine if proton MR spectroscopic imaging ((1)H-MRSI) may help identify tissue pathophysiology in patients with leukoencephalopathies.
  
  
  METHODS
  Seventy patients (mean age, 6; range, 0.66-17 years) were prospectively examined by (1)H-MRSI; a diagnosis of leukoencephalopathy due to known genetic defects leading to lack of formation, breakdown of myelin, or loss of white matter tissue attenuation (rarefaction) was made in 47 patients. The diagnosis remained undefined (UL) in 23 patients. Patients with definite diagnoses were assigned (on the basis of known pathophysiology) to 3 groups corresponding to hypomyelination, white matter rarefaction, and demyelination. Choline (Cho), creatine (Cr), and N-acetylaspartate (NAA) signals from 6 white matter regions and their intra- and intervoxel (relative to gray matter) ratios were measured. Analysis of variance was performed by diagnosis and by pathophysiology group. Stepwise linear discriminant analysis was performed to construct a model to predict pathophysiology on the basis of (1)H-MRSI, and was applied to the UL group.
  
  
  RESULTS
  Analysis of variance by diagnosis showed 3 main metabolic patterns. Analysis of variance by pathophysiology showed significant differences for Cho/NAA (P < .001), Cho/Cr (P < .004), and NAA/Cr (P < .002). Accuracy of the linear discriminant analysis model was 75%, with Cho/Cr and NAA/Cr being the best parameters for classification. On the basis of the linear discriminant analysis model, 61% of the subjects in the UL group were classified as hypomyelinating.
  
  
  CONCLUSIONS
  (1)H-MRSI provides information on tissue pathophysiology and may, therefore, be a valuable tool in the evaluation of patients with leukoencephalopathies.
authors.affiliation: Department of Neuroradiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. alberto_bizzi@fastwebnet.it
authors.forename: A
authors.initials: A
authors.lastname: Bizzi
authors.forename: G
authors.initials: G
authors.lastname: Castelli
authors.forename: M
authors.initials: M
authors.lastname: Bugiani
authors.forename: P B
authors.initials: PB
authors.lastname: Barker
authors.forename: E H
authors.initials: EH
authors.lastname: Herskovits
authors.forename: U
authors.initials: U
authors.lastname: Danesi
authors.forename: A
authors.initials: A
authors.lastname: Erbetta
authors.forename: I
authors.initials: I
authors.lastname: Moroni
authors.forename: L
authors.initials: L
authors.lastname: Farina
authors.forename: G
authors.initials: G
authors.lastname: Uziel
mesh_terms: D000326:Adrenoleukodystrophy / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D000328:Adult; D038261:Alexander Disease / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D001224:Aspartic Acid / Q000031:analogs & derivatives / Q000378:metabolism; D001921:Brain / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D002794:Choline / Q000378:metabolism; D003401:Creatine / Q000378:metabolism; D004252:DNA Mutational Analysis; D003937:Diagnosis, Differential; D004292:Dominance, Cerebral / Q000502:physiology; D005260:Female; D020279:Hereditary Central Nervous System Demyelinating Diseases / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D007091:Image Processing, Computer-Assisted / Q000379:methods*; D007223:Infant; D047908:Intracellular Signaling Peptides and Proteins / Q000172:deficiency; D019344:Lactic Acid / Q000378:metabolism; D016014:Linear Models; D008279:Magnetic Resonance Imaging / Q000379:methods*; D009682:Magnetic Resonance Spectroscopy / Q000379:methods*; D008297:Male; D008565:Membrane Proteins / Q000172:deficiency; D028361:Mitochondrial Diseases / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D009136:Muscular Dystrophies / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D020371:Pelizaeus-Merzbacher Disease / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D011446:Prospective Studies
chemical_list: D001224:Aspartic Acid; D002794:Choline; D003401:Creatine; D047908:Intracellular Signaling Peptides and Proteins; D019344:Lactic Acid; D008565:Membrane Proteins; C412048:HYCC1 protein, human; C000179:N-acetylaspartate