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  • 1
    ISSN: 1432-1459
    Keywords: Key words Protein S-100 ; CSF ; Creutzfeldt-Jakob disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We evaluated S-100 levels in paired cerebrospinal fluid (CSF) and serum samples in a group of 135 patients referred to the German Creutzfeldt-Jakob disease (CJD) surveillance unit from June 1993 to May 1995. The patients were seen in a prospective case control study. The diagnosis of probable CJD during life was made in any patient presenting with rapidly progressive dementia of less than 2 years’ duration, typical periodic sharp wave complexes (PSWCs) in the EEG and at least two of the following findings: myoclonus, visual/or cerebellar symptoms, pyramidal and/or extrapyramidal signs and/or akinetic mutism. Patients presenting with the above clinical signs and symptoms but without PSWCs were classified as possible, while those with a dementia of a duration exceeding 2 years and without PSWCs were classified as other. S-100 was determined in paired CSF and serum samples by a commercially available enzyme-linked immunosorbent assay. In a group of 76 patients with definite and probable CJD, S-100 concentration (median 25 ng/ml, range 2–117) in CSF was significantly higher (P 〈 0.0001) than in 32 patients diagnosed as other (median 4 ng/ml, range 1–19). Serum levels of S-100 were below 0.5 ng/ml in all groups. At a cut-off of 8 ng/ml an optimum sensitivity of 84.2% with a specificity of 90.6% for the diagnosis of CJD by the determination of S-100 in CSF is obtained. S-100 levels exceeding 8 ng/ml in CSF support the diagnosis of CJD in any patient presenting with rapidly progressive dementia.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Der Nervenarzt 71 (2000), S. 91-95 
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Creutzfeldt-Jakob-Krankheit ; Diagnostik ; Diffussionswichtung ; MRT ; Bildgebung ; Übertragbare spongiforme Enzephalopathien ; Key words Creutzfeldt-Jakob disease ; Diagnosis ; Diffusion-weighted image ; MRI ; Brain images ; Transmissible spongiform encephalopathies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Today the diagnosis of Creutzfeldt-Jakob disease (CJD) is proven only postmortem or by evidence of neuropathology. During the patient's lifetime EEG recordings or cerebrospinal fluid analysis may support the diagnosis. In most cases, T2-MRI scans show hyperintensities of the basal ganglia. A new imaging technique called diffusion-weighted MRI (DWI) has recently been established. The sensitivity of DWI was evaluated in five patients suspected of CJD. All five cases showed hyperintense signal changes in the basal ganglia on DWI sequences. These findings were more pronounced in DWI than in T2, FLAIR, or PD-weighted images. Thus, DWI seems to be the most sensitive sequence for detecting changes in patients with suspected CJD. Moreover, its short scanning time ensures that fewer artifacts occur, especially in the case of myoclonus.
    Notes: Zusammenfassung Die sichere Diagnose der Creutzfeldt-Jakob-Krankheit kann nur bioptisch oder autoptisch durch Untersuchung von Hirngewebe gestellt werden. Die klinische Verdachtsdiagnose erhärten können technische und laborchemische Untersuchungen; hierzu gehört neben dem EEG die Surrogatmarkerbestimmung im Liquor. Nachdem sich bei der Mehrzahl der Patienten Veränderungen der Basalganglien gezeigt haben, gehört das MRT zu den bildgebenden Verfahren, die bei Verdacht auf CJD eingesetzt werden können. Inzwischen ist die Diffusionswichtung (DWI) in die neuroradiologische Diagnostik eingeführt worden. DWI-gewichtete MRTs bei 5 CJD-Patienten wurden von uns untersucht. Alle 5 Fälle zeigten im DWI-MRT signalintense Veränderungen der Stammganglien. Diese Veränderungen waren jeweils deutlicher als in der T2, Flair- oder Protonenwichtung. Das DWI-MRT halten wir für sensitiver zum Nachweis kortikaler und basaler Veränderungen bei CJD-Patienten im Vergleich zu Standardsequenzen. Durch die kurze Untersuchungszeit können besonders bei den häufig vorhandenen Myoklonien andere Wichtungen aufgrund von Bewegungsartefakten oftmals nicht in der gewünschten Qualität durchgeführt werden.
    Type of Medium: Electronic Resource
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