Prominent brain axonal damage and functional reorganization in "pure" adrenomyeloneuropathy

S. Marino, M. De Luca, M. T. Dotti, M. L. Stromillo, P. Formichi, P. Galluzzi, M. Mondelli, P. Bramanti, A. Federico, N. De Stefano

Research output: Contribution to journalArticle

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 (H-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 H-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.

Original languageEnglish
Pages (from-to)1261-1269
Number of pages9
JournalNeurology
Volume69
Issue number12
DOIs
Publication statusPublished - Sep 2007

Fingerprint

Adrenoleukodystrophy
Brain
Creatine
Spinal Cord Injuries
Spinal Cord
Hand
Magnetic Resonance Imaging
Parietal Lobe
Pathologic Processes
Choline
Protons

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Marino, S., De Luca, M., Dotti, M. T., Stromillo, M. L., Formichi, P., Galluzzi, P., ... De Stefano, N. (2007). Prominent brain axonal damage and functional reorganization in "pure" adrenomyeloneuropathy. Neurology, 69(12), 1261-1269. https://doi.org/10.1212/01.wnl.0000276945.92950.69

Prominent brain axonal damage and functional reorganization in "pure" adrenomyeloneuropathy. / Marino, S.; De Luca, M.; Dotti, M. T.; Stromillo, M. L.; Formichi, P.; Galluzzi, P.; Mondelli, M.; Bramanti, P.; Federico, A.; De Stefano, N.

In: Neurology, Vol. 69, No. 12, 09.2007, p. 1261-1269.

Research output: Contribution to journalArticle

Marino, S, De Luca, M, Dotti, MT, Stromillo, ML, Formichi, P, Galluzzi, P, Mondelli, M, Bramanti, P, Federico, A & De Stefano, N 2007, 'Prominent brain axonal damage and functional reorganization in "pure" adrenomyeloneuropathy', Neurology, vol. 69, no. 12, pp. 1261-1269. https://doi.org/10.1212/01.wnl.0000276945.92950.69
Marino, S. ; De Luca, M. ; Dotti, M. T. ; Stromillo, M. L. ; Formichi, P. ; Galluzzi, P. ; Mondelli, M. ; Bramanti, P. ; Federico, A. ; De Stefano, N. / Prominent brain axonal damage and functional reorganization in "pure" adrenomyeloneuropathy. In: Neurology. 2007 ; Vol. 69, No. 12. pp. 1261-1269.
@article{bca25ba1b486485f88348a13aeab540c,
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 (H-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 H-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.",
author = "S. Marino and {De Luca}, M. and Dotti, {M. T.} and Stromillo, {M. L.} and P. Formichi and P. Galluzzi and M. Mondelli and P. Bramanti and A. Federico and {De Stefano}, N.",
year = "2007",
month = "9",
doi = "10.1212/01.wnl.0000276945.92950.69",
language = "English",
volume = "69",
pages = "1261--1269",
journal = "Neurology",
issn = "0028-3878",
publisher = "Lippincott Williams and Wilkins",
number = "12",

}

TY - JOUR

T1 - Prominent brain axonal damage and functional reorganization in "pure" adrenomyeloneuropathy

AU - Marino, S.

AU - De Luca, M.

AU - Dotti, M. T.

AU - Stromillo, M. L.

AU - Formichi, P.

AU - Galluzzi, P.

AU - Mondelli, M.

AU - Bramanti, P.

AU - Federico, A.

AU - De Stefano, N.

PY - 2007/9

Y1 - 2007/9

N2 - 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 (H-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 H-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.

AB - 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 (H-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 H-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.

UR - http://www.scopus.com/inward/record.url?scp=34548780360&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=34548780360&partnerID=8YFLogxK

U2 - 10.1212/01.wnl.0000276945.92950.69

DO - 10.1212/01.wnl.0000276945.92950.69

M3 - Article

C2 - 17875914

AN - SCOPUS:34548780360

VL - 69

SP - 1261

EP - 1269

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 12

ER -