NR AOSO
AU Fiorini,M.; Zanusso,G.; Farinazzo,A.; Gelati,M.; Ferrari,S.; Rizzuto,N.; Monaco,S.
TI Normal TAU Protein Levels in Cerebrospinal Fluid of 14-3-3 Positive Subjects: a Retroperspective Study
QU International Conference - Prion diseases: from basic research to intervention concepts - TSE-Forum, 08.10.-10.10.2003, Gasteig, München - Poster session - DG-47
PT Konferenz-Poster
AB
The clinical diagnosis of Creutzfeldt-Jakob Disease (CJD) is currently based upon the combination of a less than two years progressive dementia, myoclonus and multifocal neurological dysfunction, associated with characteristic periodic sharp-wave complexes (PSWCs) on electroencephalography (EEG). Advances in CJD diagnostics have occurred, in particular in the assay of 14-3-3 protein in cerebrospinal fluid (CSF), which appears to have a high sensitivity and specificity for sporadic CJD diagnosis.
In this work we present 15 subjects who presented with a rapidly evolving dementia over 3-4 weeks, accompanied with extrapyramidal and/or pyramidal signs, ataxia and other neurological signs. EEGs were characterized by a diffuse slowing without PSWCs and the magnetic resonance imaging of the brain excluded ischemic stroke, haemorrhages, or space-occupying lesions as cause of the illness. Serum analysis excluded toxic or metabolic disturbances and the presence of anti-thyroid antibodies. Routine CSF findings were normal, without blood-brain-barrier impairment or pleocytosis, except for 14-3-3 protein positivity determined by western blot. A diagnosis of probable CJD was proposed according with the diagnostic criteria. In all subjects we dosed the levels of Tau protein in the CSF, known to be consistently increased in CJD, and they ranged between 80 to 920 pg/ml with a mean level of 280 pg/ml. These Tau levels were consistently below 1300 pg/ml, a cut off considered diagnostic for sCJD (Otto et al., Neurology 2002). Over a period of 4 to 8 weeks after the lumbar puncture all patients had an almost complete recovery of symptoms. Retrospectively, we did not find other causes for the 14-3-3 positivity other than a treatment with normal dosage of neuroleptic drugs for a period of one-two months before our observation. We propose to dose Tau protein in all 14-3-3 positive patients
AD Michele Fiorini, Gianluigi Zanusso, Alessia Farinazzo, Matteo Gelati, Sergio Ferrari, Nicola Rizzuto, Salvatore Monaco, Department of Neurological and Visual Sciences, Section of Neurology University of Verona, Verona, Italy
SP englisch
PO Deutschland