title: Prominent brain axonal damage and functional reorganization in "pure" adrenomyeloneuropathy.
abstract: BACKGROUND
  Cerebral involvement is usually absent in pure adrenomyeloneuropathy (AMN). Recently, nonconventional MR studies have reported brain abnormalities in patients with pure AMN, providing evidence that occult cerebral involvement may occur in this disease. It remains unclear, however, whether these brain abnormalities reflect centripetal extension of spinal cord long-tract axonopathy or can be the expression of a pathologic process largely involving the brain.
  
  
  METHODS
  Conventional MRI and proton MR spectroscopic imaging (1H-MRSI) data of four patients with pure AMN were compared to those of four men with spinal cord injury (SCI) and 10 age-matched healthy men (HM). Resonance intensity areas of N-acetylaspartate (NAA) and choline were calculated as ratios to creatine (Cr) in voxels located in white matter (WM) regions. Functional MRI (fMRI) data during simple motor task were obtained in a separate session in three patients with AMN and three age-matched HM.
  
  
  RESULTS
  Conventional MRI examinations were normal in all patients. On 1H-MRSI, NAA/Cr values were lower in all WM regions of patients with AMN than in those of patients with SCI (p < 0.05) and HM (p < 0.01). In contrast, patients with SCI showed NAA/Cr values lower than HM only in the periventricular WM (p = 0.04). At fMRI, patients with AMN showed a more pronounced activation than HM in all movement-associated cortical regions contralateral to the hand moved and an exclusive voxel activation of the primary motor, somatosensory, and posterior parietal cortices ipsilateral to the hand moved.
  
  
  CONCLUSIONS
  CNS damage in pure adrenomyeloneuropathy is not confined exclusively to spinal cord and seems to primarily involve the brain.
authors: Department of Neurological and Behavioral Sciences, University of Siena, Siena, Italy.
authors: S
authors: S
authors: Marino
authors: M
authors: M
authors: De Luca
authors: M T
authors: MT
authors: Dotti
authors: M L
authors: ML
authors: Stromillo
authors: P
authors: P
authors: Formichi
authors: P
authors: P
authors: Galluzzi
authors: M
authors: M
authors: Mondelli
authors: P
authors: P
authors: Bramanti
authors: A
authors: A
authors: Federico
authors: N
authors: N
authors: De Stefano
mesh_terms: D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D000328:Adult; D000368:Aged; D001224:Aspartic Acid / Q000031:analogs & derivatives / Q000032:analysis / Q000378:metabolism; D001369:Axons / Q000473:pathology*; D001921:Brain / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D002540:Cerebral Cortex / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D002794:Choline / Q000032:analysis / Q000378:metabolism; D006801:Humans; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D009068:Movement / Q000502:physiology; D009434:Neural Pathways / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D009473:Neuronal Plasticity / Q000502:physiology; D020127:Recovery of Function / Q000502:physiology; D012016:Reference Values; D013119:Spinal Cord Injuries / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D014855:Wallerian Degeneration / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology
chemical_list: D001224:Aspartic Acid; C000179:N-acetylaspartate; D002794:Choline