Send to:

Choose Destination
See comment in PubMed Commons below
Brain. 1999 Aug;122 ( Pt 8):1589-95.

Spinal xanthomatosis: a variant of cerebrotendinous xanthomatosis.

Author information

  • 1Departments of Neurology, Pathology and Laboratory of Pediatrics and Neurology, University Hospital Nijmegen, Utrecht, The Netherlands.


We describe seven Dutch patients from six families with a slowly progressive, mainly spinal cord syndrome that remained for many years the sole expression of cerebrotendinous xanthomatosis (CTX). MRI demonstrated white matter abnormalities in the lateral and dorsal columns of the spinal cord. Post-mortem examination of one of the patients showed extensive myelin loss in these columns. An array of genotypes was found in these patients. We conclude that 'spinal xanthomatosis' is a clinical and radiological separate entity of CTX that should be included in the differential diagnosis of 'chronic myelopathy'.

[PubMed - indexed for MEDLINE]
Free full text
PubMed Commons home

PubMed Commons

How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for HighWire
    Loading ...
    Write to the Help Desk