Intracranial and spinal MR imaging findings associated with Krabbe's disease: Case report

Krabbe's disease is an autosomal recessive leukodystrophy with well-documen ted intracranial findings on both, CT scans and MR images. We herein presen t what is thought to be the second case of Krabbe's disease with spinal inv olvement shown on MR images as abnormal contrast enhancement of the lumbosa cral nerve roots. The typical intracranial findings of T2 hyperintensity wi thout contrast enhancement were present within the periventricular white ma tter, but there was no area of abnormal signal intensity or enhancement wit hin the substance of the spinal cord. We briefly review the pathophysiology , clinical presentation, and imaging findings of Krabbe's disease. Spinal a bnormalities may precede the onset of brain abnormalities, and MR imaging m ay be a useful diagnostic tool in cases of Krabbe's disease and other leuko dystrophies.