Author(s): V. Theophilus Premkumar1, M. Sooriyakumar2, K. Muralidharan3, Vrinda V4, P. Kumar5
Prion diseases are a group of fatal neurodegenerative diseases caused by the trans-formation of an endogenous protein, PrP (prion-related protein), into an abnormal conformation, the most common of which in humans is Creutzfeldt-Jakob disease. We report the case of a 40 year old lady who presented with rapidly progressive dementia with pyramidal, extra-pyramidal, cerebellar symptoms, myoclonus and akinetic mutism. Her EEG showed typical periodic sharp wave complexes and MRI Brain revealed DWI>FLAIR intensity in bilateral caudate nuclei, putamen & bilateral subcortical frontal lobes. The clinico-radiological correlation was consistent with the diagnosis of Creutzfeldt-Jakob disease.