IngentaConnect The earliest MR imaging and proton MR spectroscopy abnormalities ...

Abstract:

Wang C, Melberg A, Weis J, Månsson J-E, Raininko R. The earliest MR imaging and proton MR spectroscopy abnormalities in adult-onset Krabbe disease.

Acta Neurol Scand 2007: 116: 268-272.

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.

Keywords: brain; globoid cell leukodystrophy; magnetic resonance imaging; magnetic resonance spectroscopy; spinal cord

Document Type: Research article

DOI: 10.1111/j.1600-0404.2007.00867.x

Affiliations: 1: Department of Radiology, Uppsala University, Uppsala, Sweden 2: Department of Neuroscience, Neurology, Uppsala University, Uppsala, Sweden 3: Department of Medical Imaging, Uppsala University Hospital, Uppsala, Sweden 4: Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, Sahlgren Academy at Göteborg University, Gothenburg, Sweden

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