Abstract: Being considered among the most fatal neurological conditions, Creutzfeldt-Jakob disease is a transmissible spongiform encephalopathy characterized by its unknown etiology and rapidly progressive neurodegenerative symptoms that often lead to a mean survival of 6 to 12 months. The accumulation of the prion protein causes brain matter degeneration, which leads to a set of clinical findings that include rapidly progressive dementia, myoclonus, tremors, cerebellar ataxia, and extrapyramidal signs. This clinical presentation is non-specific, which makes CJD a very difficult condition to diagnose, due to the low level of clinical suspicion. We present the case of a 48-year-old male presenting to the Neurology department with complaint of limbs clumsiness and cognitive decline. The patient's physical exam was significant for Severely declining in advanced cortical function, muscular hypertonia of extremities, bilateral positive Babinski's sign. Magnetic resonance imaging of his brain showed symmetric bilateral diffusion signal abnormality involving the cortex, bilateral caudate heads and putamina. Electroencephalogram revealed periodic sharp-slow compound wave emission in bilateral cerebral hemispheres. Lumbar puncture was significant for presence of 14-3-3 protein in cerebrospinal fluid. The multiple examinations performed in conjunction with the previous findings supported the diagnosis of sporadic CJD.

Key words: Sporadic Creutzfeldt-Jakob, Prion protein, Rapidly progressive dementia, Myoclonus, Diagnosis