Abstract
Adrenomyeloneuropathy (AMN) is a phenotype variant of X-linked adrenoleukodystrophy. We present two patients with adult-onset AMN who were initially suspected to have demyelinating disorders radiologically and finally diagnosed on the basis of laboratory data. The brain magnetic resonance images showed abnormal signal intensity at pyramidal tracts and cerebellar hemisphere bilaterally with abnormal enhancement after contrast medium administration. Review of the literature shows that the brain magnetic resonance findings of adrenomyeloneuropathy may include normal brain, tract demyelination, white matter demyelination, or brain atrophy. Disease progression was demonstrated by follow-up imaging.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Reference
Aubourg P, Adamsbaum C, Lavallard-Rousseau MC, Lemaitre A, Boureau F, Mayer M, Kalifa G (1992) Brain MRI and electrophysiologic abnormalities in preclinical and clinical adrenomyeloneuropathy. Neurology 42:85–91
Mitsumoto H (2000) Disorders of upper and lower motor neurons. In: Bradley WG, Daroff RB, Fenichel GM, Marsden CD (eds) Neurology in clinical practice. Butterworth-Heinemann, Boston, pp1985–2017
Evans OB, Parker CC, Hass RH, Naidu S, Moser HW, Bock HGO (2000) Inborn errors of metabolism of the nervous system. In: Bradley WG, Daroff RB, Fenichel GM, Marsden CD (eds) Neurology in clinical practice. Butterworth-Heinemann, Boston, pp 1640–1641
Kumar AJ, Rosenbaum AE, Naidu S, Wener L, Citrin CM, Lindenberg R, Kim WS, Zinreich SJ, Molliver ME, Mayberg HS (1987) Adrenoleukodystrophy: correlating MR images with CT. Radiology 165:497–504
Aubourg P, Sellier N, Chaussain JK, Kalifa G (1989) MRI detects cerebral involvement in neurologically asymptomatic patients with adrenoleukodystrophy. Neurology 1511–1532
Kumar AJ, Köhler W, Kruse B, Naidu S, Bergin A, Edwin D, Moser HW (1995) MR findings in adult-onset adrenoleukodystrophy. AJNR Am J Neuroradiol 16:1227–1237
Powers JM, Moser HW (1998) Peroxisomal disorders: genotype, phenotype, major neuropathologic lesions, and pathogenesis. Brain Pathol 8:101–120
Barkovich AJ, Ferriero DM, Bass N, Boyer R (1997) Involvement of the pontomedullary corticospinal tracts: a useful finding in the diagnosis of X-linked adrenokeukodystrophy. AJNR Am J Neuroradiol 18:95–100
Melhem ER, Loes DJ, Georgiades CS, Raymond GV, Moser HW (2000) X-linked Adrenoleukodystrophy: the role of contrast-enhanced MR imaging in predicting disease progression. AJNR Am J Neuroradiol 21:839–844
Geel BM van, Bezman L, Loes DJ, Moser HW, Raymond GV (2001) Evolution of phenotypes in adult male patients with X-linked adrenoleukodystrophy. Ann Neurol 49:186–194
Geel BM van, Assie J, Wanders RJA, Barth PG (1997) X-linked adrenoleukodystrophy: clinical presentation, diagnosis and therapy. J Neurol Neurosurg Psychiatry 63:4–14
Geel BM van, Assies J, Weverling GJ, Barth PG (1994) Predominance of the leukodystrophy in the Netherlands: a survey of 30 kindreds. Neurology 44:2343–2346
Waragai M, Takaya Y, Hayashi M, Shibata N, Kobayashi M (1996) MRI of adrenoleukodystrophy involving predominantly the cerebellum and brain stem. Neuroradiology 38:788–791
Tzkika AA, Ball WS Jr, Vigueron DB, Dunn RS, Nelson SJ, Kirks DR (1993) Childhood adrenoleukodystrophy: Assessment with proton MRS. Radiology 189:467–480
Eichler FS, Barker PB, Cox C, Edwin D, Ulug AM, Moser HW, Raymond GV (2002) Proton MR spectroscopic imaging predicts lesion progression on MRI in X-linked adrenoleukodystrophy. Neurology 58:901–907
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Mo, YH., Chen, YF. & Liu, HM. Adrenomyeloneuropathy, a dynamic progressive disorder: brain magnetic resonance imaging of two cases. Neuroradiology 46, 296–300 (2004). https://doi.org/10.1007/s00234-003-1096-8
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00234-003-1096-8