<p>In addition to clinical symptoms, the diagnosis of prion diseases is primarily based on the detection of several clinical biomarkers. The following methods are helpful in diagnosing Creutzfeldt–Jakob disease (CJD): electroencephalography reveals triphasic periodic blocks with a&#xa0;frequency of 0.5–2/s and a duration of 100–600 ms; cerebrospinal fluid analyses shows excessive elevation in the concentration of 14-3‑3 protein with a&#xa0;specificity of 93%, as well as an elevation of the tau protein complex; and finally, magnetic resonance imaging (MRI) shows signal accentuation in the cortex and basal ganglia. The use of diffusion-weighted magnetic resonance imaging (DWI) provides additional diagnostic evidence. In combination with fluid-attenuated inversion recovery (FLAIR) sequences, MRI achieves a&#xa0;specificity of 93%. In particular, the cortex and basal ganglia are found to be affected, whereby the exact distribution varies. MRI has thus become fundamental in the diagnosis of CJD.</p>

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MRT-Diagnostik der Creutzfeldt-Jacob-Erkrankung

  • Peter Kapeller

摘要

In addition to clinical symptoms, the diagnosis of prion diseases is primarily based on the detection of several clinical biomarkers. The following methods are helpful in diagnosing Creutzfeldt–Jakob disease (CJD): electroencephalography reveals triphasic periodic blocks with a frequency of 0.5–2/s and a duration of 100–600 ms; cerebrospinal fluid analyses shows excessive elevation in the concentration of 14-3‑3 protein with a specificity of 93%, as well as an elevation of the tau protein complex; and finally, magnetic resonance imaging (MRI) shows signal accentuation in the cortex and basal ganglia. The use of diffusion-weighted magnetic resonance imaging (DWI) provides additional diagnostic evidence. In combination with fluid-attenuated inversion recovery (FLAIR) sequences, MRI achieves a specificity of 93%. In particular, the cortex and basal ganglia are found to be affected, whereby the exact distribution varies. MRI has thus become fundamental in the diagnosis of CJD.