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

From the Department of Neurological and Behavioral Sciences (S.M., M.D.L., M.T.D., M.L.S., P.F., A.F., N.D.S.), University of Siena; IRCCS Centro Neurolesi "Bonino-Pulejo" (S.M., P.B.), Messina; Neuroradiology Unit (P.G.), Azienda Ospedaliera Senese, Siena; and Azienda Sanitaria Locale (M.M.), Siena, Italy.

Address correspondence and reprint requests to Dr. Nicola De Stefano, Department of Neurological and Behavioral Sciences, Viale Bracci 2, 53100, Siena, Italy

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.

GLOSSARY: ¹H-MRSI = proton MR spectroscopic imaging; AC = anterior commissure; AMN = adrenomyeloneuropathy; BOLD = blood oxygenation level dependent; BR = brisk reflexes; Cho/Cr = choline to creatine ratio; Cr = creatine; DTI = diffusion tensor imaging; EA = endocrine abnormalities; FLAIR = fluid-attenuated inversion recovery; fMRI = functional MRI; FMRIB = Functional Magnetic Resonance Imaging of the Brain; FILM = FMRIB’s improved linear model; Fr-WM = frontal WM; HM = healthy men; Lac = lactate; MD = motor deficits; NAA = N-acetylaspartate; Naa/Cr = N-acetylaspartate to creatine ratio; PC = posterior commissure; PD = proton density; Post-WM = deep posterior WM; Pv-WM = periventricular WM; SA = sensory abnormalities; SCI = spinal cord injury; SP = spastic paraparesis; Sph Dis = sphincteric disturbances; VLCFA = very-long-chain fatty acids; VOI = volume of interest; WM = white matter.

destefano{at}unisi.it

N.D.S. was supported in part by a MIUR grant. A.F. was supported in part by a grant from Regione Toscana.

Disclosure: The authors report no conflicts of interest.

Received January 15, 2007. Accepted in final form April 16, 2007.

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