NR AXWG
AU Shiga,Y.
TI Spongiform Change Causes High Intensity Lesion in Diffusion-weighted MRI
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Pathology and Pathogenesis P03.113
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB High intemsity lesions in diffusion-weighted MRI (DWI) are important diagnostic marker of Creutzfeldt-Jakob disease (CJD). The pathogenesis that causes the CJD-related high intensity lesion has been discussed, but the conclusions are controversial. Time lag between DWI examination and postmortem pathological examination is the major reason. To describe the pathogenesis, we compared the DWI findings and pathological findings of special types of CJD, genetic CJD with V180I and sporadic CJD with MM2thalamic. In CJD with V180I DWI demonstrates prominent high intensity lesions in the wide range of cerebral cortex incongruous with slight clinical symptoms. On the other hand, in MM2-thalamic DWI demonstrates no high intensity lesions in the brain. Pathological findings of CJD with V180I are severe spongiform changes in whole layers of cerebral cortex, mild neuronal loss and astrogliosis, and weak synaptic deposition of PrP and those of MM2-T are severe neuronal loss and astrogliosis but no spongiform changes in the thalamus and inferior olive. Immunoreactivity of PrP are absent in the thalamus. In the cerebral cortex, spongiform changes are absent or limited to isolated foci, and immunoreactivity of PrP is absent or weak. Spongiform changes, neuronal loss, astrogliosis, and PrPsc deposition are major candidates. High intensity lesions in DWI are best fit to spongiform chnages based on our observation. Another characteristic of DWI findings of CJD is that the high intensity lesions with sequential DWI do not always progress with the advance of the disease, and the signal intensity sometimes decreases with the disease progression in some lesions. vacuoles of spongiform changes with a diameter of 5 to 20 µm would best provide T2 prolongation and restricted diffusion, i.e. best depicted as high intensity lesions by DWI. Vacuoles conglutinate each other with the progression of disease and vacuoles become larger in diameter. The larger in diamiter, the weaker the signal intensity. Spogifom-change hypothesis can easily explain this kind of signal changes with the disease progression. We conclude again that spongiform change is the cause of CJDrelated high intensity lesion in DWI.
AD Y. Shiga, Miyagi National Hospital, Neurology, Japan
SP englisch
PO Schottland