NR ABKH

AU Blisard,K.S.; Davis,L.E.; Harrington,M.G.; Lovell,J.K.; Kornfeld,M.; Berger,M.L.

TI Pre-mortem diagnosis of Creutzfeldt-Jakob disease by detection of abnormal cerebrospinal fluid proteins

QU Journal of the Neurological Sciences 1990 Oct; 99(1): 75-81

PT journal article

AB Creutzfeldt-Jakob disease (CJD) may be difficult to diagnose early or when it has an atypical presentation. We describe two patients with progressive dementia in whom the results of diagnostic brain biopsies were unhelpful. Spinal fluid from these patients, analyzed by two-dimensional electrophoresis, contained two abnormal proteins (Nos. 130 and 131, with relative molecular masses of 26,000 and 29,000 daltons and isoelectric points of 5.2 and 5.1). These findings suggested a provisional diagnosis of Creutzfeldt-Jakob disease, which was confirmed in both patients at autopsy. Detection of these abnormal cerebrospinal fluid proteins appears to be a valuable laboratory adjunct in evaluating patients with an unexplained progressive dementia.

IN Bei zwei Patienten mit progressiver Demenz brachten Gehirnbiopsien keinen Aufschluß. In der Rückenmarksflüssigkeit fanden die Autoren jedoch nach zweidimensionaler Gelelektrophorese zwei normalerweise nicht auftretende Proteine (Nr. 130 und Nr. 131) mit den relativen Molekularmassen 26 kD und 29 kD und isoelektrischen Punkten bei pH 5,2 bzw. 5,1. Später wurde in beiden Fällen die Creutzfeldt-Jakob-Krankheit durch Autopsien bestätigt.

MH Aged; Brain/pathology; Case Report; Cerebrospinal Fluid Proteins/*analysis; Creutzfeldt-Jakob Syndrome/*cerebrospinal fluid/pathology; Human; Male; Middle Age; Support, U.S. Gov't, Non-P.H.S.; Support, U.S. Gov't, P.H.S.

AD K. S. Blisard, Research Service, Veterans Administration Medical Center, Albuquerque, NM, U.S.A. (Present address: Department of Pathology and Laboratory Medicine, University of Cincinnati, Cincinnati, OH, U.S.A.); L. E. Davis, J. K. Lovell, Neurology Service, Veterans Administration Medical Center, Albuquerque, NM, U.S.A.; K. S. Blisard, M. Kornfeld, Department of Pathology, University of New Mexico School of Medicine, Albuquerque, NM, U.S.A.; L. E. Davis, J. K. Lovell, M. L. Berger, Department of Neurology, University of New Mexico School of Medicine, Albuquerque, NM, U.S.A.; M. G. Harrington, Biochemical Genetics Section, National Institute of Mental Health, National Institutes of Health, Bethesda, MD, U.S.A. (Present address: Department of Biology, California Institute of Technology, Pasadena, CA, U.S.A.)

SP englisch

PO Niederlande

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