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Related Experiment Videos

Diffusion tensor-based imaging reveals occult abnormalities in adrenomyeloneuropathy.

Prachi Dubey1, Ali Fatemi, Hao Huang

  • 1Department of Neurogenetics and Functional Magnetic Resonance Imaging Kirby Center, Kennedy Krieger Institute, Baltimore, MD 21205, USA.

Annals of Neurology
|October 22, 2005
PubMed
Summary
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Pure adrenomyeloneuropathy (AMN), a variant of X-linked adrenoleukodystrophy, shows hidden brain abnormalities in patients with normal MRI scans. Diffusion tensor imaging revealed microstructural changes in white matter tracts, suggesting wider disease involvement.

Area of Science:

  • Neuroimaging
  • Neurology
  • Genetics

Background:

  • "Pure" adrenomyeloneuropathy (AMN) is a variant of X-linked adrenoleukodystrophy (X-ALD) affecting spinal cord and peripheral nerves.
  • Distinct from cerebral X-ALD, pure AMN typically lacks apparent brain involvement on conventional MRI.
  • The extent of subclinical cerebral pathology in pure AMN remains poorly understood.

Purpose of the Study:

  • To investigate occult cerebral abnormalities in pure AMN patients with normal brain MRI.
  • To utilize diffusion tensor imaging (DTI) to assess white matter microstructure.
  • To identify tract-specific changes in affected brain regions.

Main Methods:

  • Diffusion tensor imaging (DTI) was employed in pure AMN patients with normal conventional brain MRI.

Related Experiment Videos

  • Fractional anisotropy (FA) and trace were analyzed in 3D white matter tract reconstructions.
  • Tracts commonly affected in cerebral X-ALD phenotypes were specifically examined.

    • Main Results:

    • Reduced FA and increased trace were observed in bilateral corticospinal tracts and the genu of the corpus callosum (p < 0.05).
    • DTI-based fiber tracking identified occult, tract-specific microstructural abnormalities.
    • These findings were present in patients with normal conventional brain MRI.

    Conclusions:

    • Pure AMN patients with normal MRI exhibit subclinical cerebral microstructural abnormalities.
    • Abnormalities in corticospinal tracts may indicate centripetal extension of spinal cord pathology.
    • Involvement of the corpus callosum suggests disease pathology may extend beyond spinal cord long tracts in pure AMN.