A 40-year-old male presented with rapidly progressive dementia for 3 weeks along with rigidity, mask-like facies and mild choreoathetoid movements along with fatigue, sleep disturbance, weight loss, headache, anxiety, veigo, malaise, and ill-defined pain. There is no history of fever. O/E, Visual disturbances Cerebellar disturbances Myoclonus CSF revealed nothing except that there was increase in 14-14-3 protein. CSF revealed nothing except that there was increase in 14-14-3 protein. MRI EEG STUDY
Correct Answer: Creutzfeldt-Jacob disease
Description: This is a case of Creutzfeldt Jacob disease as there is rapid and progressive sho-term memory loss along with classical MRI signs and CSF finding of stress protein 14-14-3. The other conditions can be easily ruled as MRI will show specific abnormalities in these conditions. EEG shows slowing and periodic lateralized discharges over the right hemisphere with triphasic morphology. MRI IMAGE shows the classical Pulvinar sign whichrefers to bilateral flair hyperintensities involving thepulvinar thalamic nuclei DWI images show the classical coical ribbon sign. Creutzfeldt-Jacob disease Rapidly progressive Fatal neurodegenerative disorder Caused by an abnormal isoform of a cellular glycoprotein known as the prion protein Presents with acute dementia, visual and cerebellar disturbances Classical Triphasic morphology on EEG waves Positive 14-3-3 CSF assay High signal in caudate/putamen on MRI.
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