NR AWGS
AU Heinemann,U.; Krasnianski,A.; Meissner,B.; Kretzschmar,H.A.; Zerr,I.
TI Value of 14-3-3 in the differential diagnosis of CJD
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions DIA-25
PT Konferenz-Poster
AB The clinical diagnosis of Creutzfeldt-Jakob disease (CJD) is basing on a characteristic clinical pattern (rapidly progressive dementia, cerebellar syndrome, visual disturbances, extrapyramidal or pyramidal signs, myoclonus and akinetic mutism) and detection of 14-3-3 in cerebrospinal fluid or periodic sharp wave complexes in EEG. Different studies described 14-3-3 detection in acute diseases such as ischemia, tumours or inflammatory disorders of the brain (Siman 2004, Saiz 1999, Satoh 2003). Whereas some of them are easily to differentiate from CJD, the presentation might overlap. Therefore we investigated the value of 14-3-3 in differential diagnosis of CJD. In Germany, over 2100 patients were notified to the Surveillance Unit in Göttingen since 1993. In 106 of them, a neuropathologically defined other diagnosis was established. In 90 patients who were tested for 14-3-3 and had a neuropathologically verified diagnosis, 58 (64%) patients had a negative test result for 14-3-3, also during course (in 5 patients). In 32 patients (36%), 14-3-3 was positive. Of the 32 positive samples, three became negative during course. The diagnoses of these patients were vasculitis and two biopsy-excluded CJD with non-specific gliosis. Six stayed positive during course with the diagnoses of encephalitis (n=3), Alzheimer's disease (n=2) and vascular dementia. In the remaining 23 patients, six (26%) had a hypoxic event prior to the lumbar puncture, four an inflammatory process (17%), and each three (13%) epileptic seizures and cerebral ischemia. These acute processes explain the detection of 14-3-3 as false positive regarding CJD. This shows a high value of 14-3-3 in differential diagnosis of CJD, when acute processes influencing the test result are excluded.
AD U. Heinemann, A. Kransnianski, B. Meissner, I. Zerr: Department of Neurology, Georg-August University Göttingen, Germany; H.A. Kretzschmar: Department of Neuropathology, Ludwig-Maximilian University Munich, Germany. E-mail: uta.Heinemann@med.uni-goettingen.de
SP englisch
PO Italien