PT  - JOURNAL ARTICLE
AU  - Given II, Curtis A.
AU  - Santos, Cesar C.
AU  - Durden, David D.
TI  - Intracranial and Spinal MR Imaging Findings Associated with Krabbe's Disease: Case Report
DP  - 2001 Oct 01
TA  - American Journal of Neuroradiology
PG  - 1782--1785
VI  - 22
IP  - 9
4099  - http://www.ajnr.org/content/22/9/1782.short
4100  - http://www.ajnr.org/content/22/9/1782.full
SO  - Am. J. Neuroradiol.2001 Oct 01; 22
AB  - Summary: Krabbe's disease is an autosomal recessive leukodystrophy with well-documented intracranial findings on both CT scans and MR images. We herein present what is thought to be the second case of Krabbe's disease with spinal involvement shown on MR images as abnormal contrast enhancement of the lumbosacral nerve roots. The typical intracranial findings of T2 hyperintensity without contrast enhancement were present within the periventricular white matter, but there was no area of abnormal signal intensity or enhancement within the substance of the spinal cord. We briefly review the pathophysiology, clinical presentation, and imaging findings of Krabbe's disease. Spinal abnormalities may precede the onset of brain abnormalities, and MR imaging may be a useful diagnostic tool in cases of Krabbe's disease and other leukodystrophies.