Diffusion tensor-based imaging reveals occult abnormalities in adrenomyeloneuropathy.

[adrenomyeloneuropathy]

"Pure " adrenomyeloneuropathy (AMN ) is the noninflammatory myeloneuropathic variant of X-linked adrenoleukodystrophy , where the disease process appears to be restricted to spinal cord tracts and peripheral nerves . The absence of obvious brain involvement makes it distinct from the inflammatory cerebral phenotypes of X-linked adrenoleukodystrophy . However , some pure AMN patients later experience development of cerebral demyelination , but little is known about the extent of brain involvement in pure AMN patients who have normal brain magnetic resonance imaging . We used diffusion tensor imaging to investigate possible occult cerebral abnormalities in such pure AMN patients . Fractional anisotropy and trace were studied in three -dimensional reconstructions of white matter tracts commonly involved in cerebral phenotypes of X-linked adrenoleukodystrophy . Results demonstrated reduced fractional anisotropy and increased trace in bilateral corticospinal tracts and genu of corpus callosum (p < 0 .05 ). Diffusion tensor imaging-based three -dimensional fiber tracking showed occult tract-specific cerebral microstructural abnormalities in pure AMN patients who had a normal conventional brain magnetic resonance image . Corticospinal tract abnormalities could reflect a centripetal extension of spinal cord long -tract distal axonopathy . Accompanying abnormalities in genu of corpus callosum indicate that the disease pathology in pure AMN may not be limited to spinal cord long tracts alone , although the involvement of the latter is most prominent and severe .

Diseases
Validation

Diseases presenting "long-tract distal axonopathy" symptom

adrenomyeloneuropathy

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