Creutzfeldt-Jakob Disease: Correlation of Clinical Neuroradiological Aspects and Detection of 14.3.3. Protein in Cerebrospinal Fluid

Creutzfeldt-Jakob disease (CJD) is a rare, transmissible illness that usually affects older adults and is characterised by rapidly progressive dementia, ataxia, myoclonus and various other neurological defects. This is now thought to be a chronic infections disease caused by an agent called a prion. The prions differ from viruses because contain little or no nucleic acid and evoke no immune response. CJD is a progressive, inevitably fatal disease with a mean survival time of 6 months to 1 year.Diagnosis is usually made clinically and electroencephalographically but even now, the diagnosis can only be confirmed by brain biopsy. CT scans usually show either no abnormalities (80% of cases) or non specific atrophy. Recently there have been reports of characteristic findings on MR images. Bilateral, symmetrically increased signal intensity was demonstrated in the basal ganglia but in various patients this finding was absent. Recently, diffusion weighted MR images proved to be useful in the evaluation of CJD bu...