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Pediatr Radiol. 1999 Sep;29(9):669-72.

MRI appearances of metachromatic leukodystrophy.

Author information

1
Department of Radiology, St. Christopher's Hospital for Children, MCP. Hahnemann School of Medicine, Erie Avenue and Front Street, Philadelphia, PA 19134-1095, USA.

Abstract

BACKGROUND:

The leukodystrophies constitute a wide spectrum of cerebral disorders of varying etiology. The imaging appearances on CT and MRI are recognizable as abnormalities of white matter; however, it may be impossible to arrive at the correct diagnosis based on imaging studies alone.

PATIENTS AND METHODS:

Three patients of varying age and clinical symptomatology diagnosed with metachromatic leukodystrophy (MLD) had remarkably similar MRI appearances. A "tigroid" or "leopard-skin" appearance was demonstrated within deep white matter in each case.

RESULTS:

All of the patients had biochemical confirmation of MLD.

CONCLUSION:

Although the "tigroid" pattern previously was considered to be pathognomonic of Pelizaeus-Merzbacher disease, the diagnosis of MLD must now be considered when these MRI appearances are encountered.

PMID:
10460327
DOI:
10.1007/s002470050672
[Indexed for MEDLINE]

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