The earliest MR imaging and proton MR spectroscopy abnormalities in adult-onset Krabbe disease
2007 (English)In: Acta Neurologica Scandinavica, ISSN 0001-6314, E-ISSN 1600-0404, Vol. 116, no 4, 268-272 p.Article in journal (Refereed) Published
BACKGROUND: Adult-onset Krabbe disease is an uncommon form of leukodystrophy. Its magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) manifestations are not well documented. AIM OF THE STUDY: To describe early MR findings in adult-onset Krabbe disease. MATERIALS AND METHODS: A 28-year-old woman who had spastic paraparesis and a 5-year history of gait problems underwent MRI of the brain and cervical spine. Proton MRS was performed at 1.5 T using a short echo time. Metabolites were analyzed in the areas of MR signal abnormalities and normal-appearing brain. Six healthy volunteers were examined as controls. RESULTS: MRI revealed changes in the upper corticospinal tracts, splenium and, minimally, adjacent to the atria of the lateral ventricles. MRS showed decreased creatine, choline, N-acetylaspartate and glutamate and probably elevated lactate in the upper corticospinal tract but not in the normal-appearing frontal lobe. The spinal cord was thin. Laboratory tests verified Krabbe disease. CONCLUSIONS: These results indicate early involvement of the upper corticospinal tract in adult-onset Krabbe disease. The cases reported earlier had imaging changes indicating a more advanced disease or no MR findings. Thinning of the spinal cord is a new finding in Krabbe disease.
Place, publisher, year, edition, pages
2007. Vol. 116, no 4, 268-272 p.
brain, globoid cell leukodystrophy, magnetic resonance imaging, magnetic resonance spectroscopy, spinal cord
Medical and Health Sciences
IdentifiersURN: urn:nbn:se:uu:diva-11435DOI: 10.1111/j.1600-0404.2007.00867.xISI: 000249220700015PubMedID: 17824908OAI: oai:DiVA.org:uu-11435DiVA: diva2:39204