NR ATLP
AU Heinemann,U.; Bartl,M.; Krasnianski,A.; Meissner,B.; Varges,D.; Kretzschmar,H.A.; Zerr,I.
TI Differential diagnosis of Creutzfeldt-Jakb disease (CJD) with similar clinical appearance
QU International Conference - Prion 2005: Between fundamentals and society's needs - 19.10.-21.10.2005, Congress Center Düsseldorf - Poster Session: Diagnosis DIA-53
PT Konferenz-Poster
AB
Creutzfeldt-Jakob disease is a neurodegenerative disorder. In 1998 clinical diagnostic criteria were established. They include dementia under 2 years duration combined with two typical neurological signs and positive 14-3-3 in cerebrospinal fluid (CSF) or EEG signs. Clinical practice showed very good applicability of these criteria with reliable results.
Since 1993 the Goettinger CJD surveillance examines all suspected cases of CJD in Germany and classifies the patients according to the mentioned criteria. In this manner we examined since 1998 816 probable cases, 89 possible cases of CJD and 259 other cases. Altogether 535 patients got a neuropathological examination. This survey investigates all patients since 1998, where clinical and neuropathological diagnosis are divergent.
20 patients with definite CJD were clinically classified as other cases. Most of these cases did not show any or enough neurological symptoms which are necessary for possible or probable diagnosis. In another case dementia could not be verified because of aphasia. Further problems existed because of inflammatory CSF signs like oligoclonal bands an therefor classification as other case.
24 patients with neuropathological exclusion of prion disease were classified as probable or possible CJD. Most of these patients showed histological signs of Alzheimer's disease as Braak stadium 1-5. Another often differential diagnosis with similar clinical appearance is vascular dementia. Also in this group patients with inflammatory disease are difficult to differentiate.
Problems are uprised by atypical cases of AD with rapid course and many neurological symptoms. Vascular dementia is difficult to differentiate as expected. The relevance of inflammatory CSF signs in differential diagnosis should be further evaluated because this survey demonstrates both false classification as other or as probable case. Summarizing there is a need for complete diagnostic procederes to reliably diagnose CJD.
AD U.Heinemann, M.Bartl, A.Krasnianski, B.Meissner, D.Varges, H.Kretzschmar, I.Zerr, Department of Neurology, University of Göttingen, Germany
SP englisch
PO Deutschland