NR APAV
AU Zerr,I.; Bodemer,M.; Bartl,M.; Körtner,K.; Jastrow,U.; Westner,I.; Kretzschmar,H.A.; Poser,S.
TI Surveillance, diagnosis and differential diagnosis of CJD in Germany
QU International Conference - Prion diseases: from basic research to intervention concepts - TSE-Forum, 08.10.-10.10.2003, Gasteig, München - Poster session - DG-71
PT Konferenz-Poster
AB Since 1993, neurological and psychiatric hospitals throughout Germany are circulated three times a year and are asked to notify patients with rapid progressive dementia to the clinical part of the German CJD Surveillance Unit. After notification of a suspected case, an standardized examination of the patient is conducted on site and all relevant clinical data are taken to the center in Göttingen. Blood, cerebrospinal fluid and urine samples are taken and stored at -80 C. An interview and a questionnaire on possible risk factors is done with the relatives. Patients are then classified according standardized criteria as probable, possible or other case and a report is sent to the hospital. After a follow up, the diagnosis is either confirmed by autopsy in the refence center for spongiform encephalopathies in Munich or a reclassification is done according to the evolution of the clinical syndrome. In the years 1993-2003, 1580 patients were seen prospectively in the clinical study. In 962, the diagnosis was confirmed by neuropathology or was classified as clinically probable CJD. In 425 patients, the classification of other dementia was done. The most frequent differential diagnosis was Alzheimer's disease, followed by dementia with Lewy bodies. In young patients, an encephalitis associated with Hashimoto's thyreoiditis, was the most common and treatable disease course. In the framework of the study, an evaluation of new diagnostic parameters was carried our. The most sensitive marker of the disease is the detection of the proteins 14-3-3 in the CSF (in 95%), followed by the magnetic resonance imaging (hyperintense basal ganglia) in 66%. Of interest, the introduction of new diagnostic techniques such as 14-3-3 has direct consequences for the epidemiological and broadens the phenotypic range of the disease: since the introduction of the biochemical markers in the diagnostic process in our laboratory, more atypical variants of sporadic CJD are seen in the study.
AD Inga Zerr, Monika Bodemer, Mario Bartl, Katrin Körtner, Ute Jastrow, Sigrid Poser, Dept. of Neurology, Georg-August University, Göttingen, Germany; Ingo Westner, Hans Kretzschmar, Insitute of Neuropathology, Ludwig-Maximilian University, Munich
SP englisch
PO Deutschland