NR AUQL
AU Zerr,I.
TI Differential diagnosis of molecular disease subtypes in CJD
QU TSE-Forum, 6. Kongress - Nationale TSE-Forschungsplattform, Greifswald 26.6.-28.6.2006, Vortrag V-04
PT Konferenz-Vortrag
AB The clinical diagnosis in sporadic CJD is supported by the detection of brain-derived proteins in the cerebrospinal fluid, PSWCs in the EEG and hyperintense signals in basal ganglia on magnetic resonance imaging (MRI). The detection of abnormal high levels of neuronal proteins such as 14-3-3 in the CSF is now established as a sensitive marker of neuronal loss and is helpful in the differential diagnosis of dementia. In a recent EC-based study an analysis of biochemical parameters was carried out in more than 3000 patients with various TSE forms. We demonstrated that sensitivity and predictive values of biochemical parameters depend on patient's characteristics and on molecular disease subtype. In a further analysis on brain imaging techniques such as positron emission tomography (PET) and magnetic resonance imaging, we attempted to define characteristic lesion pattern for CJD. In the first step we defined a standard MRI protocol. We demonstrated that the most sensitive detection techniques are FLAIR-and diffusion weighted images. In the second step we analysed cortical and subcortical MRI lesion pattern in patients with sporadic, genetic, iatrogenic and variant CJD in an EC based collaborative study. Characteristic lesion pattern in the MRI are strongly associated with molecular disease subtypes. In patients with MV2 subtype, hyperintense signal in the pulvinar thalamus is seen, and hyperintensities in the hippocampal areas was a characteristic feature of the rare VV1 subtype. An algorithm for the clinical definition of molecular disease subtypes was developed and will be presented. Our results support the introduction of MRI in the clinical diagnostic criteria since subtype specific lesion pattern using high sensitive techniques will allow a premortem clinical classification of particular disease subtype in CJD.
AD Inga Zerr, Prionforschungsgruppe, Neurologische Klinik, Universitätsklinikum Göttingen, Robert-Koch-Str. 40, 37075 Göttingen
SP englisch
PO Deutschland
OR Tagungsband